Rhumatology

1.Which one of the following is least associated with Behcet's syndrome?
ia
A.A Mouth ulcersia

B.A Genital ulcersia
x C.A Conjunctivitisia
D.A Deep vein thrombosisia
E.A Aseptic meningitisia
Next question
Oral ulcers + genital ulcers + anterior uveitis = Behcet's
Ocular involvement is the most feared complication of Behcet's disease. Conjunctivitis is
seen rarely and is much less common than anterior uveitis. Other ocular problems seen
include retinal vasculitis, iridocyclitis and chorioretinitis
Behcet's syndrome
sqweqwesf erwrewfsdfs adasd dhe
Behcet's syndrome is a complex multisystem disorder associated with presumed
autoimmune mediated inflammation of the arteries and veins. The precise aetiology has
yet to be elucidated however. The classic triad of symptoms are oral ulcers, genital ulcers
and anterior uveitis
he earaer aeraer asdsadas eerw dssdfsselleds
Epidemiology
• more common in the eastern Mediterranean (e.g. Turkey)he
• more common in men (complicated gender distribution which varies according to
country. Overall, Behcet's is considered to be more common and more severe in
men)he
• tends to affect young adults (e.g. 20 - 40 years old)he
• associated with HLA B5 and MICA6 allelehe
• c. 30% of patients have a positive family historyhe
Features
• classically: 1) oral ulcers 2) genital ulcers 3) anterior uveitishe
• thrombophlebitishe
• arthritishe
• neurological involvement (e.g. aseptic meningitis)he

• GI: abdo pain, diarrhoea, colitishe
• erythema nodosum, DVThe
Diagnosis
• no definitive testhe
• diagnosis based on clinical findingshe
• positive pathergytest is suggestive (puncture site following needle prick becomes
inflamed with small pustule forming)
2.Cytoplasmic antineutrophil cytoplasmic antibodies (cANCA) are most strongly
associated with which condition?ia
x A.A Wegener's granulomatosis

B.A Churg-Strauss syndromeia
C.A Polyarteritis nodosaia
D.A Goodpasture's syndromeia
E.A Autoimmune hepatitisia
Next question
cANCA = Wegener's; pANCA = Churg-Strauss + others
ANCA
There are two main types of anti-neutrophil cytoplasmic antibodies (ANCA) -
cytoplasmic (cANCA) and perinuclear (pANCA)
For the exam, remember:
• cANCA - Wegener's granulomatosishe
• pANCA - Churg-Strauss syndrome + others (see below)he
cANCA
• target = serine proteinase 3 (PR3)he
• level of cANCA may be used to monitor disease activityhe
• Wegener's granulomatosis, positive in > 90%he
• microscopic polyangiitis, positive in 40%he
pANCA
• target = myeloperoxidase (MPO)he
• cannot use level of pANCA to monitor disease activityhe

• associated with immune crescentic glomerulonephritis (positive in c. 80% of
patients)he
• microscopic polyangiitis, positive in 50-75%he
• Churg-Strauss syndrome, positive in 60%he
• Wegener's granulomatosis, positive in 25%he
Other causes of positive ANCA (usually pANCA)
• inflammatory bowel disease (UC > Crohn's)he
• connective tissue disorders: RA, SLE, Sjogren'she
• autoimmune hepatitishe
3.Which of the following features are not typically seen in a patient with adult onset
Still's disease?ia
A.A Maculopapular rashia

x B.A Rheumatoid factor
C.A Pyrexiaia
D.A High ferritin levelia
E.A Lymphadenopathyia
Adult onset Still's disease is typically rheumatoid factor negative

Still's disease in adults
Adult Still's disease
• typically affects 16-35 year oldshe
Features
• arthralgiahe
• elevated serum ferritinhe

• rash: salmon-pink, maculopapular, pruritiche
• pyrexiahe
• lymphadenopathyhe
• rheumatoid factor (RF) and anti-nuclear antibody (ANA) negativehe

4.A 34-year-old intravenous drug user is admitted with a purpuric rash affecting her legs.
Blood tests reveal the following:
Hb 11.4g/dl
Platelets 489 * 109/l
WCC 12.3 * 109/l
HCV PCR positive

HBsAg negative
Rheumatoid factor positive

C3/C4 reduced

What is the most likely diagnosis?ia
A.A Polyarteritis nodosaia
B.A Henoch-Schonlein purpuraia
C.A Wegener's granulomatosisia
x D.A Cryoglobulinaemiaia
E.A Systemic lupus erythematousia
Next question
Hepatitis C infection is associated with type II (mixed) cryoglobulinaemia, suggested by
the purpuric rash, positive rheumatoid factor and reduced complement levels
Cryoglobulinaemia
Immunoglobulins which undergo reversible precipitation at 4 deg C, dissolve when
warmed to 37 deg C. One third of cases are idiopathic
Three types
• type I (25%): monoclonalhe
• type II (25%): mixed monoclonal and polyclonal: usually with RFhe

• type III (50%): polyclonal: usually with RFhe
Type I
• monoclonal - IgG or IgMhe
• associations: multiple myeloma, Waldenstrِm macroglobulinaemiahe
Type II
• mixed monoclonal and polyclonal: usually with RF he
• associations: hepatitis C, RA, Sjogren's, lymphomahe
Type III
• polyclonal: usually with RFhe
• associations: RA, Sjogren'she
Symptoms (if present in high concentrations)
• Raynaud's only seen in type Ihe
• cutaneous: vascular purpura, distal ulceration, ulcerationhe
• arthralgiahe
• renal involvement (diffuse glomerulonephritis)he

Tests
• low complement (esp. C4)he
• high ESRhe
Treatment
• immunosuppressionhe
• plasmapheresishe
5.A 45-year-old female with a history of rheumatoid arthritis presents to the Emergency
Department with a two day history of a hot, painful, swollen right elbow joint. What is
the most appropriate management?ia
x A.A Joint aspiration
B.A Start infliximabia
C.A Oral high-dose prednisoloneia
D.A Short course of methotrexateia
E.A Depomederone injectionia
Next question
Joint aspiration is mandatory in all patients with a hot, swollen joint to rule out septic
arthritis. If this was excluded in the above patient then intra-articular or system steroid
therapy may be considered.
Septic arthritis
Overview
• most common organism overall is Staphylococcus aureushe
• however
in young adults who are sexually active Neisseria gonorrhoea is the most
common cause (up to 75% of cases)he
6.A 34-year-old woman with a history of antiphospholipid syndrome presents with a
swollen and painful leg. Doppler ultrasound confirms a deep vein thrombosis (DVT). She
had a previous DVT 4 months ago and is taking warfarin with a target INR of 2-3. How
should her anticoagulation be managed?ia
x A.A Life-long warfarin, increase target INR to 3 - 4
B.A Add in life-long low-dose aspirinia
C.A A further 6 months warfarin, target INR 2 - 3ia
D.A A further 6 months warfarin, target INR 3 - 4ia
E.A Life-long warfarin, target INR 2 - 3ia
Next question
The evidence base is scanty here but most clinicians would increase the target INR to 3-4
if a patient has had a further thrombosis with an INR of 2-3. Please see the BCSH
guidelines
Antiphospholipid syndrome
Antiphospholipid syndrome is an acquired disorder characterised by a predisposition to
both venous and arterial thromboses, recurrent fetal loss and thrombocytopenia. It may
occur as primary disorder or secondary to other conditions, most commonly systemic
lupus erythematous (SLE)
A key point for the exam is to appreciate that antiphospholipid syndrome causes a
paradoxical rise in the APTT. This is due to an ex-vivo reaction of the lupus
anticoagulant autoantibodies with phospholipids involved in the coagulation cascade
Features
• venous/arterial thrombosishe
• recurrent fetal losshe
• livedo reticularishe
• thrombocytopeniahe
• prolonged APTThe
• other features: pre-eclampsia, pulmonary hypertensionhe

Associations other than SLE
• other autoimmune disordershe
• lymphoproliferative disordershe
• phenothiazines (rare)he
Management - based on BCSH guidelines
• initial venousthromboembolic events: evidence currently supports use of warfarin
with a target INR of 2-3 for 6 monthshe
• recurrent venous thromboembolic events: lifelong warfarin; if occurred whilst
taking warfarin then increase target INR to 3-4he
• arterial thrombosis should be treated with lifelong warfarin with target INR 2-3he
7.Which one of the following would not suggest an underlying connective tissue disorder
in a patient with Raynaud's?ia
A.A Unilateral symptomsia
B.A Digital ulcersia
C.A Presence of autoantibodiesia
x D.A Onset at 25 years old
E.A Calcinosisia
Next question
Raynaud's disease (i.e. primary) presents in young women with bilateral symptoms
Raynaud's
Raynaud's phenomena may be primary (Raynaud's disease) or secondary (Raynaud's
phenomenon)
Raynaud's disease typically presents in young women (e.g. 30 years old) with
symmetrical attacks
Factors suggesting underlying connective tissue disease
• onset after 40 yearshe
• unilateral symptomshe
• rasheshe
• presence of autoantibodieshe
• digital ulcers, calcinosishe
• very rarely: chilblainshe

Secondary causes
• connective tissue disorders: scleroderma (most common), rheumatoid arthritis,
SLEhe
• leukaemiahe
• type I cryoglobulinaemia, cold agglutininshe

• use of vibrating toolshe
• drugs: oral contraceptive pill, ergothe
• cervical ribhe

Management
• calcium channel blockershe
• IV prostacyclin infusionshe
8.A 31-year-old female intolerant of methotrexate is started on azathioprine for
rheumatoid arthritis. Routine blood monitoring shows a Hb of 6.9, platelets of 72 and
WBC of 2.5. Which of the following factors will predispose her to azathioprine toxicity?
ia
A.A Cimetidineia
B.A Rifampicinia
C.A Fast acetylator statusia
x D.A Thiopurine methyltransferase deficiency
E.A Alcohol excessia
Next question
Thiopurine methyltransferase (TPMT) deficiency is present in c. 1 in 300 people and
predisposes to azathioprine related pancytopaenia
Azathioprine
Azathioprine is metabolised to the active compound mercaptopurine, a purine analogue
that inhibits DNA synthesis. A thiopurine methyltransferase (TPMT) test may be needed
to look for individuals prone to azathioprine toxicity
Adverse effects include
• bone marrow depressionhe
• N/Vhe
• pancreatitishe
A significant interaction may occur with allopurinol and lower doses of azathioprine should be used
9.A health trust in the United Kingdom which serves a population of 100,000 is planning
services for patients with rheumatoid arthritis. How many of the population would be
expected to have the disease?ia
A.A 100ia
B.A 300ia
x C.A 1,000
D.A 2,000ia
E.A 10,000ia
Next question
The prevalence of rheumatoid arthritis in the UK population is approximately 1%
Rheumatoid arthritis: epidemiology
Epidemiology
• peak onset = 30-50 years, although occurs in all age groupshe
• F:M ratio = 3:1 he
• prevalence = 1%he
• some ethnic differences e.g. high in Native Americanshe

• associated with HLA-DR4 (especially Felty's syndrome)
10.A 47-year-old man with a history of chronic sinusitis presents with haemoptysis and
shortness of breath to the Emergency Department. Initial investigations reveal:
Hb 10.4g/dl
Platelets 477 * 109/l
WCC 14.3 * 109/l
ESR 92 mm/h
Urea 20 mmol/l
Creatinine 198 µmol/l
Urine dipstick blood +++

A.A Mixed cryoglobulinaemiaia
B.A Takayasu's arteritisia
x C.A Wegener's granulomatosis
D.A Haemolytic uraemic syndromeia
E.A Henoch-Schonlein purpuraia
Next question
The combination of pulmonary and renal involvement combined with a history of chronic
sinusitis points towards a diagnosis of Wegener's granulomatosis
Wegener's granulomatosis
Wegener's granulomatosis is an autoimmune condition associated with a necrotizing
granulomatous vasculitis, affecting both the upper and lower respiratory tract as well as
the kidneys
Features
• upper respiratory tract: epistaxis, sinusitis, nasal crustinghe
• lower respiratory tract: dyspnoea, haemoptysishe
• glomerulonephritis ('pauci-immune', 80% of patients)he
• saddle-shape nose deformityhe
• also: vasculitic rash, eye involvement (e.g. proptosis), cranial nerve lesionshe
Investigations
• cANCA positive in > 90%, pANCA positive in 25%he
• chest x-ray: wide variety of presentations, including cavitating lesionshe
Management
• steroidshe
• cyclophosphamide (90% response)he

• plasma exchangehe
• median survival = 8-9 yearshe

11.A 27-year-old man presents to the Emergency Department with a hot swollen knee
joint. He is pyrexial with a temperature of 38.4 C and a white cell count of 17.8. Which
of the following organisms is most likely to be cultured from his knee aspirate?ia
A.A E. coliia
B.A Candidaia
x C.A Neisseria gonorrhoea
D.A Staphylococcus epidermidisia
E.A Streptococcus pneumoniaeia
Next question
Young people with septic arthritis - gonococcus; older people - Staph aureus
Neisseria gonorrhoea is the most common cause of septic arthritis in young sexually
active adults. The most common organism seen in the general population however is
Staphylococcus aureus
Gonorrhoea
Gonorrhoea is caused by the Gram negative diplococcus Neisseria gonorrhoea. Acute
infection can occur on any mucous membrane surface, typically genitourinary but also
rectum and pharynx. The incubation period of gonorrhoea is 2-5 days
Local complications that may develop include urethral strictures, epididymitis and
salpingitis (hence may lead to infertility).
Management
• ciprofloxacin 500mg PO use to be the treatment of choicehe
• however, there is
increased resistance to ciprofloxacin and therefore cephalosporins
are now usedhe
• options include cefixime 400mg PO (single dose) or ceftriaxone 250mg IM he
Disseminated gonococcal infection (DGI) and gonococcal arthritis may also occur, with
gonococcal infection being the most common cause of septic arthritis in young adults.
The pathophysiology of DGI is not fully understood but is thought to be due to
haematogenous spread from mucosal infection (e.g. asymptomatic genital infection).
Initially there may be a classic triad of symptoms: tenosynovitis, migratory polyarthritis
and dermatitis. Later complications include septic arthritis, endocarditis and perihepatitis
(Fitz-Hugh-Curtis syndrome)
Key features of disseminated gonococcal infection
• tenosynovitishe
• migratory polyarthritishe
• dermatitis (lesions can be maculopapular or vesicular)

12.An autoantibody screen reveals that a patient is positive for anti-Jo 1 antibodies. What
is the most likely underlying diagnosis?ia
A.A Limited cutaneous systemic sclerosisia
B.A Mixed connective tissue diseaseia
C.A Dermatomyositisia
x D.A Polymyositisia
E.A Diffuse cutaneous systemic sclerosisia
Next question
Anti-Jo 1 antibodies are more commonly seen in polymyositis than dermatomyositis
Extractable nuclear antigens
Overview
• specific nuclear antigenshe
• usually associated with being ANA positivehe
Examples
• anti-Ro: Sjogren's syndrome, SLE, congenital heart blockhe
• anti-La: Sjogren's syndromehe
• anti-Jo 1: polymyositishe
• anti-scl-70: diffuse cutaneous systemic sclerosishe
• anti-centromere: limited cutaneous systemic sclerosishe
13.A 54-year-old woman with a long history of rheumatoid arthritis is reviewed in clinic
complaining of shortness of breath. Oxygen saturations are 92% on room air with
spirometry showing a restrictive pattern associated with a reduced transfer factor. Which
one of the following drugs is most likely to be responsible?ia
A.A Depomederoneia
B.A Hydroxychloroquineia
x C.A Methotrexateia
D.A Ciclosporinia
E.A Celecoxibia
Next question
Amiodarone & methotrexate are common causes of lung fibrosis
This has patient has pulmonary fibrosis which may be caused by methotrexate. Other
anti-rheumatoid drugs such as sulfasalazine and gold may also cause pulmonary fibrosis
Drugs causing lung fibrosis
Causes
• amiodaronehe
• cytotoxic agents: busulphan, bleomycinhe

• anti-rheumatoid drugs: methotrexate, sulfasalazine, goldhe
• nitrofurantoinhe
14.Which of the following antibodies is most specific for diffuse cutaneous systemic
sclerosis?ia
A.A Anti-nuclear factoria
B.A Anti-centromere antibodiesia
x C.A Anti-Scl-70 antibodies
D.A Rheumatoid factoria
E.A Anti-Jo 1antiobodiesia
Next question
Although ANA is positive in 90% of patients with systemic sclerosis, anti-Scl-70
antibodies are the most specific test for diffuse cutaneous systemic sclerosis
Systemic sclerosis
A group of conditions of unknown aetiology characterised by hardened, sclerotic skin and
other connective tissue
Overview
• increase in type I collagen in tissueshe
• female:male = 4:1he
Three patterns of disease:
Limited cutaneous systemic sclerosis - 'CREST syndrome'
• CREST: Calcinosis, Raynaud's phenomenon, oEsophageal dysmotility,
Sclerodactyly, Telangiectasiahe
• Raynaud's may be first signhe
• scleroderma affects face and distal limbs predominatelyhe
• associated with anti-centromere antibodieshe
Diffuse cutaneous systemic sclerosis
• scleroderma affects trunk and proximal limbs predominatelyhe
• associated with scl-70 antibodieshe
• hypertension, lung fibrosis and renal involvement seenhe
• poor prognosishe
Scleroderma (without internal organ involvement)
• tightening and fibrosis of skinhe
• may be manifest as plaques (morphoea) or linearhe
Antibodies
• ANA positive in 90%he
• RF positive in 30%he
• anti-scl-70 antibodies associated with diffuse cutaneous systemic sclerosishe
• anti-centromere antibodies associated with limited cutaneous systemic sclerosish
15.Which one of the following is least associated with systemic lupus erythematous?ia
A.A Anti-nuclear antibodiesia
B.A Anti-Sm antibodiesia
C.A Elevated ESRia
x D.A Elevated C3 and C4 levels
E.A Elevated anti-dsDNA titres in active diseaseia
Next question
SLE: ANA is 99% sensitive - anti-Sm & anti-dsDNA are 99% specific
SLE: investigations
Immunology
• 99% are ANA positivehe
• 20% are rheumatoid factor positivehe
• anti-dsDNA: highly specific (> 99%), but less sensitive (70%)he
• anti-Sm: most specific (> 99%), sensitivity (30%)he
Monitoring
• ESR: during active disease the CRP is characteristically normal - a raised CRP may
indicate underlying infection he
• complement levels (C3, C4) are low during active disease (formation of complexes
leads to consumption of complement)he
• anti-dsDNA titres can be used for disease monitoring (but note not present in all
patients)he
16.A 62-year-old female presents to her GP with a 4 week history of intermittent
headaches and lethargy. Blood tests reveal the following:
ESR 67 mm/hr
A.A Polymyalgia rheumaticaia
B.A Cluster headachesia
D.A Migraineia
x E.A Temporal arteritis
Next question
This is a classic history of temporal arteritis. Treatment should be started immediately
with high dose steroids (e.g. prednisolone 1mg/kg/day) to reduce the chance of visual
loss
Temporal arteritis
Temporal arteritis is large vessel vasculitis which overlaps with polymyalgia rheumatica
(PMR). Histology shows changes which characteristically 'skips' certain sections of
affected artery whilst damaging others
Features
• typically patient > 60 years oldhe
• usually rapid onset (e.g. < 1 month)he
• headache (found in 85%)he
• jaw claudication (65%)he
• tender, palpable temporal arteryhe
• features
of PMR: aching, morning stiffness in proximal limb muscles (not
weakness)he
• also lethargy, depression, low-grade fever, anorexia, night sweatshe
Investigations
• ESR > 50 mm/hr (note ESR < 30 in 10% of patients)he
• temporal artery biopsy: skip lesions may be presenthe
• note CK and EMG normalhe
• reduced CD8+ T cellshe
Treatment
• high-dose prednisolone (dramatic response)
17.A 79-year-old man presents to his GP with a history of lower back pain and right hip
pain. Blood tests reveal the following:
Calcium 2.20 mmol/l
Phosphate 0.8 mmol/l
ALP 890 u/L

A.A Primary hyperparathyroidismia
B.A Chronic renal failureia
C.A Osteomalaciaia
D.A Osteoporosisia
x E.A Paget's disease
The normal calcium and phosphate combined with a raised alkaline phosphate points to a
diagnosis of Paget's
Paget's disease
Pathogenesis
• increased bone turnoverhe
• thought to be primarily a disorder of osteoclasts, with excessive osteoclastic

resorption followed by increased osteoblastic activityhe
Complications
• deafnesshe
• bone sarcoma (1% if affected for > 10 years)he

• fractureshe
• skull thickeninghe
• high-output cardiac failurehe

Investigations
• calcium and phosphate typically normalhe
• ALP highhe
• skull x-ray: thickened vault, osteoporosis circumscriptahe

Management
• bisphosphonate if severe, calcitonin sometimes usedhe
18.Which one of the following cytokines is the most important in the pathogenesis of
Rheumatoid diseaseia
A.A IFN-betaia
B.A IFN-alphaia
C.A IL-4ia
x D.A TNFia
E.A IL-2ia
Next question
Tumour necrosis factor
Tumour necrosis factor (TNF) is a pro-inflammatory cytokine with multiple roles in the
immune system
TNF is secreted mainly by macrophages and has a number of effects on the immune
system, acting mainly in a paracrine fashion:
• activates macrophages and neutrophilshe
• acts as costimulator for T cell activationhe
• key mediator of bodies response to Gram negative septicaemiahe
• similar properties to IL-1he
• anti-tumour effect (e.g. phospholipase activation)he
TNF-alpha binds to both the p55 and p75 receptor. These receptors can induce apoptosis.
It also cause activation of NFkB
Endothelial effects include increase expression of selectins and increased production of
platelet activating factor, IL-1 and prostaglandins
TNF promotes the proliferation of fibroblasts and their production of protease and
collagenase. It is thought fragments of receptors act as binding points in serum
Systemic effects include pyrexia, increased acute phase proteins and disordered
metabolism leading to cachexia
TNF is important in the pathogenesis of rheumatoid arthritis - antibodies to TNF (e.g.
infliximab, etanercept) are now licensed for treatment of severe rheumatoid
TNF blockers
• etanercept: s/c administration, can cause demyelinationhe
• infliximab: IV administration, risks include reactivation of TBhe
Infliximab is also used in active Crohn's disease unresponsive to steroids
19.A 25-year-old man presents to his GP complaining of dysuria and pain in his left knee.
Three weeks previously he had suffered a severe bout of diarrhoea. What is the most
likely diagnosis?ia
x A.A Reactive arthritis
B.A Disseminated gonococcal infectionia
C.A Behcet's syndromeia
D.A Ulcerative colitisia
E.A Rheumatoid arthritisia
Next question
Urethritis + arthritis + conjunctivitis = reactive arthritis
Two of the classic three features of reactive arthritis (urethritis, arthritis and
conjunctivitis) are present in this patient
Reactive arthritis
Reactive arthritis is one of the HLA-B27 associated seronegative spondyloarthropathies.
It encompasses Reiter's syndrome, a term which described a classic triad of urethritis,
conjunctivitis and arthritis following a dysenteric illness during the Second World War.
Later studies identified patients who developed symptoms following a sexually
transmitted infection.
The American College of Rheumatology now define reactive arthritis as an episode of
peripheral arthritis lasting for greater than 1 month associated with urethritis/cervicitis or
diarrhoea
Epidemiology
• post-STI form much more common in men (e.g. 10:1)he
• post-dysenteric form equal sex incidencehe
Organisms often responsible for post-dysenteric form
• Shigella flexnerihe
• Salmonella typhimuriumhe
• Salmonella enteritidishe
• Yersinia enterocoliticahe
• Campylobacterhe

Organisms often responsible for post-STI form
• Chlamydia trachomatishe
20.Perinuclear antineutrophil cytoplasmic antibodies (pANCA) are most strongly
associated with which condition?ia
A.A Goodpasture's syndromeia
x B.A Churg-Strauss syndrome
D.A Wegener's granulomatosisia
E.A Autoimmune hepatitisia
Next question
ANCA
There are two main types of anti-neutrophil cytoplasmic antibodies (ANCA) -
cytoplasmic (cANCA) and perinuclear (pANCA)
For the exam, remember:
• cANCA - Wegener's granulomatosishe
• pANCA - Churg-Strauss syndrome + others (see below)he
cANCA
• target = serine proteinase 3 (PR3)he
• level of cANCA may be used to monitor disease activityhe
• Wegener's granulomatosis, positive in > 90%he
• microscopic polyangiitis, positive in 40%he
pANCA
• target = myeloperoxidase (MPO)he
• cannot use level of pANCA to monitor disease activityhe

• associated with immune crescentic glomerulonephritis (positive in c. 80% of
patients)he
• microscopic polyangiitis, positive in 50-75%he
• Churg-Strauss syndrome, positive in 60%he
• Wegener's granulomatosis, positive in 25%he
Other causes of positive ANCA (usually pANCA)
• inflammatory bowel disease (UC > Crohn's)he
• connective tissue disorders: RA, SLE, Sjogren'she
• autoimmune hepatitishe
21.Which one of the following statements regarding systemic lupus erythematous is most
true?ia
A.A It is linked with HLA A5ia
x B.A Onset is typically between 20-40 years old
C.A It is more common in Caucasiansia
D.A The female:male ratio is 3:1ia
E.A The incidence has decreased in the past 30 yearsia
Next question
Systemic lupus erythematous
Epidemiology
• much more common in females (F:M = 9:1)he
• more common in Afro-Caribbeans* and Asian communitieshe
• onset is usually 20-40 yearshe
• incidence hasrisen substantially during the past 50 years (3 fold using American
College of Rheumatology criteria)he
Pathophysiology
• autoimmune disease he
• associated with HLA B8, DR2, DR3he
• thought tobe caused by immune system dysregulation leading to immune complex
formation he
• immune complex deposition can affect any organ; skin, joints, kidneys brain most
commonly affectedhe
*It is said the incidence in black Africans is much lower than in black Americans - the reasons for
this are unclear
22.Sjogren's syndrome is associated with each one of the following, except:ia
A.A Renal tubular acidosisia
B.A Xerostomiaia
C.A Dyspareuniaia
x D.A Dilated cardiomyopathy
E.A Raynaud's phenomenonia
Next question
Sjogren's syndrome
Sjogren's syndrome is an autoimmune disorder affecting exocrine glands. It may be
primary or associated with rheumatoid arthritis or other connective tissue disorder.
Sjogren's syndrome is much more common in females (ratio 9:1). There is a marked
increased risk of lymphoid malignancy (40-60 fold)
Features
• dry eyes: keratoconjunctivitis siccahe
• dry mouthhe
• vaginal dryness --> dyspareunia, Raynaud's, renal tubular acidosishe
• arthralgiahe
Investigation
• rheumatoid factor (RF) positive in 100%he
• anti-Ro (SSA) and anti-La (SSB) antibodieshe
• hypergammaglobulinaemiahe
• Schirmer's test: filter paper near conjunctival sac to measure tear formationhe
• histology: lymphocytic infiltrationhe
23.Which of the following is associated with a good prognosis in rheumatoid arthritis?ia
x A.A Rheumatoid factor negative
B.A HLA DR4ia
C.A Female sexia
D.A Rheumatoid nodulesia
E.A Insidious onsetia
Next question
Rheumatoid arthritis: prognostic features
A number of features have been shown to predict a poor prognosis in patients with
rheumatoid arthritis, as listed below
Poor prognostic features
• rheumatoid factor positivehe
• poor functional status at presentationhe
• HLA DR4he
• X-ray: early erosions (e.g. after < 2 years)he
• extra articular features e.g. noduleshe
• female sexhe
• insidious onsethe
24.Which one of the following features is least commonly seen in drug-induced lupus?ia
x A.A Glomerulonephritisia
B.A Arthralgiaia
C.A Myalgiaia
D.A Malar rashia
E.A Pleurisyia
Next question
Glomerulonephritis is unusual in drug-induced lupus
Drug-induced lupus
In drug-induced lupus not all the typical features of systemic lupus erythematous are
seen, with renal and nervous system involvement being unusual. It usually resolves on
stopping the drug
Features
• arthralgiahe
• myalgiahe
• skin (e.g. malar rash) and pulmonary involvement (e.g. pleurisy) are commonhe
• ANA positive in 100%, dsDNA negativehe
Causes
• procainamidehe
• isoniazidhe
• minocyclinehe
• hydralazinehe
• chlorpromazinehe
• anti-epileptics: carbamazepine, phenytoinhe
25.Rheumatoid arthritis is associated with which one of the following HLA haplotypes?ia
A.A HLA-B5ia
B.A HLA-B27ia
C.A HLA-DR2ia
D.A HLA-DR3ia
x E.A HLA-DR4
Next question
Around 70% of patients with rheumatoid arthritis (RA) are HLA-DR4. Patients with
Felty's syndrome (a triad of RA, splenomegaly and neutropaenia) are even more strongly
associated with 90% being HLA-DR4
Rheumatoid arthritis: epidemiology
Epidemiology
• peak onset = 30-50 years, although occurs in all age groupshe
• F:M ratio = 3:1 he
• prevalence = 1%he
• some ethnic differences e.g. high in Native Americanshe

• associated with HLA-DR4 (especially Felty's syndrome)
26.Which of the following statements is true regarding psoriatic arthropathy?ia
A.A Skin disease always precedes joint diseaseia
B.A Approximately one-third of patients with psoriasis eventually develop
arthropathyia
C.A The mainstay of management is analgesia, physiotherapy and joint
replacementia
x D.A Males and females are equally affected
E.A Arthritis mutilans is the most common subtypeia
Next question
Males and females are affected equally by psoriatic arthritis
Psoriatic arthropathy
Psoriatic arthropathy correlates poorly with cutaneous psoriasis and often precedes the
development of skin lesions. Around 10% percent of patients with skin lesions develop
an arthropathy with males and females being equally affected
Types*
• rheumatoid-like polyarthritis: (30-40%, most common type)he
• asymmetrical oligoarthritis: typically affects hands and feet (20-30%)he
• sacroilitishe
• DIP joint disease (10%)he
• arthritis mutilans (severe deformity fingers/hand, 'telescoping fingers')he

Management
• treat as rheumatoid arthritishe
• but better prognosishe
*Until recently it was thought asymmetrical oligoarthritis was the most common type, based on data
from the original 1973 Moll and Wright paper. Please see the link for a comparison of more recent
studies
27.Which one of the following is least recognised as a risk factor for developing
osteoporosis?ia
A.A Cushing's syndromeia
B.A Turner's syndromeia
C.A Hyperparathyroidismia
x D.A Hypothyroidismia
E.A Diabetes mellitusia
Next question
Hyperthyroidism is associated with an increased risk of osteoporosis
Osteoporosis: causes
Risk factors
• family historyhe
• female sexhe
• increasing agehe
• deficient diethe
• sedentary lifestylehe
• smokinghe
• premature menopausehe
• low body weight he
• Asians and Orientalshe
Diseases which predispose
• endocrine: glucocorticoid
excess (e.g. Cushing's, steroid therapy), hyperthyroidism,
hypogonadism (e.g. Turner's), growth hormone deficiency, hyperparathyroidism,
diabetes mellitushe
• multiple myeloma, lymphomahe
• GI problems: malabsorption (e.g. coeliacs), gastrectomy, liver diseasehe
• rheumatoid arthritishe
• long term heparin therapyhe
• chronic renal failurehe
• osteogenesis imperfecta, homocystinuriahe
28.Which one of the following statements concerning discoid lupus is correct?ia
A.A Commonly progresses to SLEia
B.A Causes non-scarring alopeciaia
x C.A Characterised by follicular keratin plugs
D.A Is rarely photosensitiveia
E.A Typically presents in older malesia
Next question
Discoid lupus erythematous is characterised by follicular keratin plugs
Discoid lupus erythematous
Basics
• more common in young womenhe
• characterised by follicular keratin plugshe
• very rarely progresses to SLEhe
Features
• erythematous, raised rash, sometimes mild-moderate scalehe
• may be photosensitivehe
• more common on face, neck, ears and scalphe
• scarring alopeciahe
29.A 63-year-old man presents to the Emergency Department with a 2 day history of a
painful and swollen left knee joint. Aspiration reveals positively birefringent crystals and
no organisms are seen. Which of the following conditions are not recognised causes of
the underlying condition?ia
A.A Haemochromatosisia
B.A Low magnesiumia
x C.A High phosphate
D.A Acromegalyia
E.A Hyperparathyroidismia
Next question
A low phosphate predisposes to pseudogout
Pseudogout
Basics
• due to calcium pyrophosphate dihydratehe
• more common in elderly femaleshe
• weakly-positively birefringent brick shaped crystalshe
Features
• knee, wrist and shoulders most commonly affectedhe
• x-ray: chondrocalcinosishe

Risk factors
• hyperparathyroidismhe
• hypothyroidismhe
• haemochromatosishe
• acromegalyhe
• low magnesium, low phosphatehe

Management
• aspiration of joint fluidhe
30.Which of the following is not a recognised cause of Raynaud's phenomenon?ia
A.A Oral contraceptive pillia
B.A Cervical ribia
C.A Type I cryoglobulinaemiaia
x D.A Pizotifenia
E.A Sclerodermaia
Next question
Ergotamine rather than pizotifen is associated with Raynaud's phenomenon
Raynaud's
phenomenon)
symmetrical attacks
• rasheshe

Secondary causes
SLEhe
• leukaemiahe

• cervical ribhe

Management
31.A 45-year-old woman is referred to rheumatology outpatients with a 4 month history
of joint pains, myalgia and generalised lethargy. An autoantibody screen reveals she is
ANA positive and ribonuclear protein positive. The creatine kinase is elevated at 525.
A.A Systemic lupus erythematousia
x B.A Mixed connective tissue disease
C.A Polymyositisia
D.A Dermatomyositisia
E.A CREST syndromeia
Next question
Anti-ribonuclear protein (anti-RNP) = mixed connective tissue disease
Mixed connective tissue disease
Features of SLE, systemic sclerosis and polymyositis
Anti-RNP positive
32.Which one of the following X-ray changes is not associated with osteoarthritis?ia
A.A Decreased joint spaceia
B.A Subchondral sclerosisia
C.A Subchondral cystsia
D.A Osteophytes at the joint marginia
x E.A Periarticular erosions
Next question
Periarticular erosions are seen in rheumatoid arthritis. The other four changes are
classically seen in osteoarthritis
Osteoarthritis
X-ray changes
• decrease of joint spacehe
• subchondral sclerosishe
• subchondral cysts he
• osteophytes forming at joint marginshe
Management
• analgesia, physio/occupational therapy, joint replacementhe
• glucosamine: see below - may disease modifying propertieshe
Glucosamine
• normal constituent of glycosaminoglycans in cartilage and synovial fluidhe
• a recent systematic review of several double blind RCTs of glucosamine in knee
osteoarthritis reported significant short-term symptomatic benefits including
significantly reduced joint space narrowing and improved pain scores
33.Which of the following findings is not typical in a patient with antiphospholipid
syndrome?ia
A.A Prolonged APTTia
x B.A Thrombocytosisia
C.A Recurrent venous thrombosisia
D.A Recurrent arterial thrombosisia
E.A Livedo reticularisia
Next question
Antiphospholipid syndrome: arterial/venous thrombosis, miscarriage, livedo reticularis
Thrombocytopenia is associated with antiphospholipid syndrome
Features


34.Which one of the following is least recognised as a risk factor for developing
osteoporosis?ia
A.A Multiple myelomaia
B.A Rheumatoid arthritisia
x C.A Long-term warfarin therapy
D.A Chronic renal failureia
E.A Diabetes mellitusia
Next question
Risk factors
• female sexhe
• smokinghe

diabetes mellitushe
35.A 64-year-old man presents to the Emergency Department with a 2 day history of an
swollen, painful left knee. Aspirated joint fluid shows calcium pyrophosphate crystals.
Which of the following blood tests is most useful in revealing an underlying cause?ia
x A.A Transferrin saturation
B.A ACTHia
C.A ANAia
D.A Serum ferritinia
E.A LDHia
Next question
This is a typical presentation of pseudogout. An elevated transferrin saturation may
indicate haemochromatosis, a recognised cause of pseudogout
A high ferritin level is also seen in haemochromatosis but can be raised in a variety of
infective and inflammatory processes, including pseudogout, as part of an acute phase
response
Pseudogout
Basics
• due to calcium pyrophosphate dihydratehe
• more common in elderly femaleshe

• weakly-positively birefringent brick shaped crystalshe
Features
• knee, wrist and shoulders most commonly affectedhe
• x-ray: chondrocalcinosishe
Risk factors
• hyperparathyroidismhe
• hypothyroidismhe
• haemochromatosishe
• acromegalyhe
• low magnesium, low phosphatehe
Management
• aspiration of joint fluidhe
36.A 24-year-old Asian female presents to her GP with lethargy and dizzy spells. On
examination she is noted to have an absent left radial pulse. Blood tests show an ESR of
84 mm/hr. What is the likely diagnosis?ia
A.A Turner's syndromeia
x B.A Takayasu's arteritis
C.A Kawasaki diseaseia
D.A Systemic lupus erythematousia
E.A Lyme diseaseia
Next question
Takayasu's arteritis
Takayasu's arteritis is a large vessel vasculitis. It typically causes occlusion of the aorta
and questions commonly refer to an absent limb pulse. It is more common in females and
Asian people
Associations
• renal artery stenosishe

Management
• steroidshe
37.Which one of the following drugs is least associated with drug-induced lupus?ia
x A.A Trimethoprimia
B.A Isoniazidia
C.A Hydralazineia
D.A Procainamideia
E.A Chlorpromazineia
Next question
Drug-induced lupus
In drug-induced lupus not all the typical features of systemic lupus erythematous are
seen, with renal and nervous system involvement being unusual. It usually resolves on
stopping the drug
Features
• arthralgiahe
• myalgiahe
• skin (e.g. malar rash) and pulmonary involvement (e.g. pleurisy) are commonhe
• ANA positive in 100%, dsDNA negativehe
Causes
• procainamidehe
• isoniazidhe
• minocyclinehe
• hydralazinehe
• chlorpromazinehe
• anti-epileptics: carbamazepine, phenytoinhe
38.Which one of the following drugs is most likely to cause gout?ia
x A.A Low-dose aspirin
B.A Lansoprazoleia
C.A Phenytoinia
D.A Gentamicinia
E.A Penicillinia
Next question
Gout: drug causes
Drug causes
• thiazides, frusemidehe
• low-dose aspirinhe
• alcohol he
• cytotoxic agentshe
• pyrazinamide, ethambutolhe
39.A 54-year-old man with a history of type 2 diabetes mellitus presents with a history of
right shoulder pain. On examination there is limited movement of the right shoulder in all
directions. What is the most likely diagnosis?ia
x A.A Adhesive capsulitis
B.A Dermatomyositisia
C.A Avascular necrosisia
D.A Lhermitte's syndromeia
E.A Diabetic amyotrophyia
Next question
Diabetic amyotrophy affects the lower limbs
Adhesive capsulitis
Adhesive capsulitis has a known association with diabetes. Patients typically have a
painful freezing phase, an adhesive phase and a recovery phase. The episode typically
lasts 2-3 years.
40.Each one of the following is seen in reactive arthritis, except:ia
A.A Urethritisia
B.A Keratoderma blenorrhagicaia
C.A Conjunctivitisia
x D.A Aseptic meningoencephalitis
E.A Circinate balanitisia
Reactive arthritis: features

Hans Reiter described a classic triad is of urethritis, conjunctivitis and arthritis following
a dysenteric illness during the Second World War. Later studies identified patients who
developed symptoms following a sexually transmitted infection. The American College
of Rheumatology now define reactive arthritis as an episode of peripheral arthritis lasting
for greater than 1 month associated with urethritis/cervicitis or diarrhoea
Features
• typically develops
within 4 weeks of initial infection - symptoms generally last
around 4-6 monthshe
• arthritis is typically an asymmetrical oligoarthritis of lower limbshe
• may present as monoarthritis e.g. kneehe
• symptoms of urethritishe
• eye: conjunctivitis (seen in 50%), anterior uveitishe
• skin: circinate balanitis
(painless vesicles on the coronal margin of the prepuce),
keratoderma blenorrhagica (waxy yellow papules on palms and soles)he
Around 25% of patients have recurrent episodes whilst 10% of patients develop chronic disease
41.A 54-year-old male presents with proximal myopathy and a macular rash over his
back and upper arms. He is a heavy smoker and his sodium is 121. What is the likely
underlying diagnosis?ia
A.A Addison's diseaseia
B.A Polymyositisia
C.A Overlap syndromeia
x D.A Dermatomyositisia
E.A Hypothyroidismia
Next question
This man may have an underlying small cell lung cancer causing SIADH
Dermatomyositis
Overview
• inflammatory disorder causing symmetrical, proximal muscle weakness and
characteristic skin lesionshe
• may be idiopathic or associated with connective tissue disorders or underlying
malignancy (found in 20-25% - more if patient older)he
• polymyositisis a variant of the disease where skin manifestations are not
prominenthe
Skin features
• photosensitivehe
• macular rash over back and shoulderhe

• heliotrope rash over cheekhe
• Gottron's papules - roughened red papules over extensor surfaces of fingershe
• nail fold capillary dilatationhe
Other features
• proximal muscle weakness +/- tendernesshe
• Raynaud'she
• respiratory muscle weaknesshe

• interstitial lung disease: e.g. fibrosing alveolitis or organising pneumoniahe
• dysphagia, dysphoniahe
42.Which of the following is least likely to be associated with ankylosing spondylitis?ia
A.A Apical fibrosisia
B.A Achilles tendonitisia
C.A Amyloidosisia
x D.A Achalasiaia
E.A Heart blockia
Next question
Ankylosing spondylitis features - the 'A's
• Apical fibrosis
• Anterior uveitis
• Aortic regurgitation
• Achilles tendonitis
• AV node block
• Amyloidosis
Achalasia is not a recognised association of ankylosing spondylitis

Ankylosing spondylitis: features
Features
• typically a young man who presents with lower back pain and stiffnesshe
• stiffness is usually worse in morning and improves with activityhe
• peripheral arthritis (25%, more common if female)he
Other features - the 'A's
• Apical fibrosishe
• Anterior uveitishe
• Aortic regurgitationhe
• Achilles tendonitishe
• AV node blockhe
• Amyloidosishe
• and cauda equina syndromehe

X-rays are often normal early in disease, later changes include:
• sacroilitis: subchondral erosions, sclerosishe
• squaring of lumbar vertebraehe
• 'bamboo spine' (late & uncommon)he
- chest x-ray: apical fibrosis
Spirometry may show a restrictive defect due to a combination of pulmonary fibrosis,
kyphosis and ankylosis of the costovertebral joints
43.Which of the following have not been shown to be a marker of poor prognosis in
rheumatoid arthritis?ia
A.A Insidious onsetia
B.A Early erosions on x-rayia
x C.A Male sex
D.A Rheumatoid nodulesia
E.A Rheumatoid factor positiveia
Next question
Rheumatoid arthritis: prognostic features
A number of features have been shown to predict a poor prognosis in patients with
rheumatoid arthritis, as listed below
Poor prognostic features
• rheumatoid factor positivehe
• poor functional status at presentationhe
• HLA DR4he
• X-ray: early erosions (e.g. after < 2 years)he

• extra articular features e.g. noduleshe
• female sexhe
• insidious onsethe
44.Which one of the following is an example of a large vessel vasculitis?ia
A.A Polyarteritis nodosaia
B.A Churg-Strauss syndromeia
x C.A Takayasu's arteritis
D.A Henoch-Schonlein purpuraia
E.A Kawasaki diseaseia
Next question
Vasculitides
Large vessel
• temporal arteritishe
• Takayasu's arteritishe
Medium vessel
• polyarteritis nodosahe
• Kawasaki diseasehe
Small vessel
• ANCA-associated vasculitides (Wegener's*, Churg-Strauss*, microscopic
polyangiitis)he
• Henoch-Schonlein purpurahe
• cryoglobulinaemic vasculitishe

*may also affect medium-sized vessels
45.A 31-year-old female with a history of SLE gives birth following a 39 week
pregnancy. The newborn is noted to be bradycardic. Which one of the following
autoantibodies are associated with congenital heart block?ia
x A.A Anti-Ro
B.A Anti-Laia
C.A Anti-RNPia
D.A AMAia
E.A Anti-Jo 1ia
Next question
SLE: pregnancy
Overview
• risk of maternal autoantibodies crossing placentahe
• leads to condition termed neonatal lupus erythematoushe
• neonatal complications include congenital heart blockhe
• strongly associated with anti-Ro (SSA) antibodieshe
46.Each one of the following is part of the American College of Rheumatology criteria
for diagnosing rheumatoid arthritis, except:ia
x A.A Raised ESR or CRP
B.A Morning stiffness > 1 hria
C.A Subcutaneous nodulesia
D.A Symmetrical arthritisia
E.A Rheumatoid factor positiveia
Next question
Rheumatoid arthritis: ACR criteria
American College of Rheumatology criteria
• requires 4 of the following 7 criteriahe
• sensitivity = 92%, specificity = 89%he
Morning stiffness > 1 hr (for at least 6 weeks)
Soft-tissue swelling of 3 or more joints (for at least 6 weeks)
Swelling of PIP, MCP or wrist joints (for at least 6 weeks)
Symmetrical arthritis
Subcutaneous nodules
Radiographic evidence of erosions or periarticular osteopenia
47.A 61-year-old female is noted to have multiple sites of muscle tenderness in the
rheumatology clinic. A diagnosis of fibromyalgia is suspected. What is the most
appropriate management?ia
A.A Naproxenia
x B.A Amitriptylineia
C.A Cognitive behavioural therapyia
D.A Propranololia
E.A Prednisoloneia
Next question
Fibromyalgia
The management of fibromyalgia consists of the following:
• explanationhe
• increase in aerobic exercisehe
• low-dose amitriptylinehe
48.A 27-year-old female is referred to the medical outpatient clinic due to a long history
of fatigue and joint pains. An autoimmune screen is done which is positive for smooth
muscle antibodies. What is the most appropriate next investigation?ia
x A.A Liver function tests
B.A Thyroid function testsia
C.A Creatinine kinaseia
D.A Serum glucoseia
E.A Electrocardiogramia
Next question
Smooth muscle antibodies are associated with autoimmune hepatitis. Presentation is
usually insidious and extrahepatic clinical features are common
Autoimmune hepatitis
Autoimmune hepatitis is condition of unknown aetiology which is most commonly seen
in young females. Recognised associations include other autoimmune disorders,
hypergammaglobulinaemia and HLA B8, DR3.Two types of autoimmune hepatitis have
been characterised according to the types of circulating antibodies present
Type I Type II
Anti-nuclear antibodies (ANA) and/or anti- Anti-liver/kidney microsomal
smooth muscle antibodies (SMA) type 1 antibodies (LKM1)
he earaer aeraer asdsadas eerw he earaer aeraer asdsadas eerw
dssdfsselleds dssdfsselleds
Affects both adults and children Affects children only

Features
• may present with signs of chronic liver diseasehe
• acute hepatitis: fever, jaundice etc (only 25% present in this way)he
• amenorrhoea (common)he
• ANA/SMA/LKM1 antibodies, raised IgG levelshe

• liverbiopsy: inflammation extending beyond limiting plate 'piecemeal necrosis',
bridging necrosishe
Management
• steroids, other immunosuppressants e.g. azathioprinehe
• liver transplantationhe
49.Deficiency of which one of the following types of complement are associated with the
development of systemic lupus erythematous?ia
x A.A C4ia
B.A C5ia
C.A C6ia
D.A C7ia
E.A C8ia
Next question
Complement deficiencies of C4a and C4b have been shown to be associated with an
increased risk of developing systemic lupus erythematous
SLE: investigations
Immunology
Monitoring
patients)he
50.Which one of the following drugs is least likely to cause gout?ia
x A.A Lithiumia
B.A Bendrofluazideia
C.A Alcoholia
D.A Low-dose aspirinia
E.A Frusemideia
Next question
Gout: drug causes
Drug causes
• thiazides, frusemidehe
• low-dose aspirinhe
• alcohol he
• cytotoxic agentshe
• pyrazinamide, ethambutolhe
51.Osteopetrosis is due to a defect in:ia
x A.A Osteoclast function
B.A PTH receptorsia
C.A Osteoblast functionia
D.A Calcium resorption in proximal tubuleia
E.A Calcium absorptionia
Next question
Osteopetrosis
Overview
• aka marble bone diseasehe
• rare disorder of defective osteoclast function resulting in failure of normal bone

resorptionhe
52.A 51-year-old male presents with an acute onset of swelling and pain in his right knee.
Aspiration shows negatively birefringent crystals with no organisms seen. His pain fails
to settle with NSAIDs. What is the most appropriate next step in his management?ia
x A.A Repeat joint aspiration and intra-articular depomederone
B.A Allopurinolia
C.A IV flucloxacillinia
D.A Diureticsia
E.A Low dose methotrexateia
Next question
Gout: management
Acute management
• NSAIDshe
• intra-articular steroid injectionhe

Indications for allopurinol*
• recurrent attackshe
• tophihe
• renal diseasehe
• renal stoneshe
• prophylaxis if on cytotoxicshe
*patients with Lesch-Nyhan syndrome often take allopurinol for life
53.Which one of the following antibodies is most specific for limited cutaneous systemic
sclerosis?ia
A.A Anti-Jo 1antiobodiesia
B.A Rheumatoid factoria
C.A Anti-Scl-70 antibodiesia
x D.A Anti-centromere antibodies
E.A Anti-nuclear factoria
Next question
Although ANA is positive in 90% of patients with systemic sclerosis, anti-centromere
antibodies are the most specific test for limited cutaneous systemic sclerosis
Systemic sclerosis
A group of conditions of unknown aetiology characterised by hardened, sclerotic skin and
other connective tissue
Overview
• increase in type I collagen in tissueshe
• female:male = 4:1he
Three patterns of disease:
Limited cutaneous systemic sclerosis - 'CREST syndrome'
• CREST: Calcinosis, Raynaud's phenomenon, oEsophageal dysmotility,
Sclerodactyly, Telangiectasiahe
• Raynaud's may be first signhe
• scleroderma affects face and distal limbs predominatelyhe
• associated with anti-centromere antibodieshe
Diffuse cutaneous systemic sclerosis
• scleroderma affects trunk and proximal limbs predominatelyhe
• associated with scl-70 antibodieshe
• hypertension, lung fibrosis and renal involvement seenhe
• poor prognosishe
Scleroderma (without internal organ involvement)
• tightening and fibrosis of skinhe
• may be manifest as plaques (morphoea) or linearhe
Antibodies
• RF positive in 30%he
• anti-scl-70 antibodies associated with diffuse cutaneous systemic sclerosishe
• anti-centromere antibodies associated with limited cutaneous systemic sclerosishe
54.Which one of the following is most useful in the management of Familial
Mediterranean Fever?ia
A.A Prednisoloneia
B.A Erythromycinia
C.A Cyclophosphamideia
x D.A Colchicineia
E.A Benzylpenicillinia
Next question
Familial Mediterranean Fever
Familial Mediterranean Fever (FMF, also known as recurrent polyserositis) is an
autosomal recessive disorder which typically presents by the second decade. It is more
common in people of Turkish, Armenian and Arabic descent
Features - attacks typically last 1-3 days
• pyrexiahe
• abdominal pain (due to peritonitis)he
• pleurisyhe
• pericarditishe
• arthritishe
• erysipeloid rash on lower limbshe

Management
• colchicine may helphe
55.Which one of the following is the most common ocular manifestation of rheumatoid
arthritis?ia
A.A Scleritisia
B.A Episcleritisia
x C.A Keratoconjunctivitis sicca
D.A Corneal ulcerationia
E.A Keratitisia
Next question
Rheumatoid arthritis: ocular manifestations
Ocular manifestations of rheumatoid arthritis are common, with 25% of patients having
eye problems
Ocular manifestations
• keratoconjunctivitis sicca (most common)he
• episcleritis (erythema)he
• scleritis (erythema and pain)he
• corneal ulcerationhe
• keratitishe
Iatrogenic
• steroid-induced cataractshe
• chloroquine retinopathyhe
56.The presence of anti-cyclic citrullinated peptide antibody is suggestive of which one

of the following conditions?ia
x B.A Rheumatoid arthritis
C.A Type 1 diabetes mellitusia
D.A Addison's diseaseia
E.A Dermatomyositisia
Next question
Anti-cyclic citrullinated peptide antibodies are associated with rheumatoid arthritis
Anti-cyclic citrullinated peptide antibody may be detectable up to 10 years before the
development of rheumatoid arthritis. It may therefore play a key role in the future of
rheumatoid arthritis, allowing early detection of patients suitable for aggressive anti-TNF
therapy. It has a sensitivity similar to rheumatoid factor (70-80%, see below) with a much
higher specificity of 90-95%.
Rheumatoid factor
Rheumatoid factor (RF) is a circulating antibody (usually IgM) which reacts with
antigenic sites on the Fc portion of the patients own IgG
RF can be detected by either
• Rose-Waaler test: sheep red cell agglutinationhe
• Latex agglutination test (less specific)he
RF is positive in 70-80% of patients with rheumatoid arthritis, high titre levels are
associated with severe progressive disease (but NOT a marker of disease activity)
Other conditions associated with a positive RF include:
• Sjogren's syndrome (<= 100%)he
• Felty's syndrome (<= 100%)he
• infective endocarditis (= 50%)he
• SLE (= 20-30%)he
• systemic sclerosis (= 30%)he
• general population (= 5%)he
• rarely: TB, HBV, EBV, leprosyhe
57.An autoantibody screen reveals that a patient is positive for anti-RNP antibodies.
What is the most likely underlying diagnosis?ia
A.A Limited cutaneous systemic sclerosisia
B.A Diffuse cutaneous systemic sclerosisia
C.A Dermatomyositisia
D.A Polymyositisia
x E.A Mixed connective tissue disease
Next question
Mixed connective tissue disease
Features of SLE, systemic sclerosis and polymyositis
Anti-RNP positive
58.A 64-year-old female is referred to rheumatology out-patients by her GP with a history
of arthritis in both hands. Which one of the following x-ray findings would most favour a
diagnosis of rheumatoid arthritis over other possible causes?ia
A.A Loss of joint spaceia
x B.A Juxta-articular osteoporosis
C.A Subchondral sclerosisia
D.A Osteophytesia
E.A Subchondral cystsia
Next question
Juxta-articular osteoporosis would point towards a diagnosis of rheumatoid arthritis
(RA). Loss of joint space is common in both RA and osteoarthritis
Rheumatoid arthritis: x-ray changes
Early x-ray findings
• loss of joint spacehe
• juxta-articular osteoporosishe
• soft-tissue swellinghe

Late x-ray findings
• periarticular erosionshe
• subluxationhe
59.Which one of the following has been shown in clinical trials to significantly slow joint
space narrowing in patients with osteoarthritis?ia
A.A Cod-liver oilia
B.A Physiotherapyia
C.A Methotrexateia
x D.A Glucosamineia
E.A Vitamin B6ia
Next question
Osteoarthritis
X-ray changes
Management
Glucosamine
significantly reduced joint space narrowing and improved pain scoreshe
60.A 33-year-old man who is suspected of having ankylosing spondylitis has a lumbar
spine x-ray. Which one of the following features is most likely to be present?ia
A.A Wedge shaped discsia
x B.A Sclerosisia
C.A 'Rugger-Jersey' spineia
D.A Osteophytesia
E.A Subchondral cystsia
Features
• peripheral arthritis(25%, more common if female)heaeraer asdsadas eerw
dssdfsselled
• AV node blockhe
• Amyloidosishe

61.Which one of the following is true regarding the investigation of a patient with
dermatomyositis?ia
A.A Creatine kinase is characteristically normalia
B.A Muscle biopsy is contraindicatedia
x C.A Anti-Jo-1 antibodies are usually negative
D.A Antinuclear antibodies are always negativeia
E.A EMG is normalia
Dermatomyositis: investigations and management

Investigations
• elevated creatine kinasehe
• EMGhe
• muscle biopsyhe
• anti-Jo-1 antibodies
are not commonly seen in dermatomyositis - they are more
common in polymyositis where they are seen in a pattern of disease associated
with lung involvement, Raynaud's and feverhe
Management
• prednisolonehe
62.A 47-year-old man who is known to have dermatomyositis secondary to small cell
lung cancer is noted to have roughened red papules over the extensor surfaces of the
fingers. What are these lesions called?ia
A.A Heberden's nodeia
B.A Aschoff nodulesia
x C.A Gottron's papules
D.A Bouchard's nodesia
E.A Muehrcke's linesia
Gottron's papules are roughened red papules over the extensor surfaces and are seen in
dermatomyositis
Heberden's and Bouchard's nodes are seen in osteoarthritis. Aschoff nodules are
pathognomonic of rheumatic fever whilst Muehrcke's lines are white, transverse lines of
the fingernail seen in hypoalbuminaemia
Dermatomyositis
Overview
• inflammatory disorder causing symmetrical, proximal muscle weakness and
characteristic skin lesionshe
• may be idiopathic or
associated with connective tissue disorders or underlying
malignancy (found in 20-25% - more if patient older)he
• polymyositisis a variant of the disease where skin manifestations are not
prominenthe
Skin features
• photosensitivehe
• macular rash over back and shoulderhe
• heliotrope rash over cheekhe
• Gottron's papules - roughened red papules over extensor surfaces of fingershe
• nail fold capillary dilatationhe

Other features
• proximal muscle weakness +/- tendernesshe
• Raynaud'she
• respiratory muscle weaknesshe
• interstitial lung disease: e.g. fibrosing alveolitis or organising pneumoniahe

• dysphagia, dysphoniahe
63.Which of the following is not a recognised feature of temporal arteritis?ia
A.A Rapid response to high dose prednisoloneia
B.A Pyrexiaia
C.A Skip lesions in temporal arteryia
D.A Visual disturbanceia
x E.A Elevated creatinine kinase
Next question
Creatinine kinase levels are not elevated in temporal arteritis
Temporal arteritis
Temporal arteritis is large vessel vasculitis which overlaps with polymyalgia rheumatica
(PMR). Histology shows changes which characteristically 'skips' certain sections of
affected artery whilst damaging others
Features
• headache (found in 85%)he
• jaw claudication (65%)he
• tender, palpable temporal arteryhe
• features
of PMR: aching, morning stiffness in proximal limb muscles (not
weakness)he
• also lethargy, depression, low-grade fever, anorexia, night sweatshe
Investigations
• ESR > 50 mm/hr (note ESR < 30 in 10% of patients)he
• temporal artery biopsy: skip lesions may be presenthe
Treatment
• high-dose prednisolone (dramatic response)
64.Which one of the following conditions has polygenic inheritance?ia

A.A Bartter's syndromeia
B.A Huntington diseaseia
x C.A Ankylosing spondylitis
D.A Fragile X syndromeia
E.A Von Willebrand's diseaseia
Ankylosing spondylitis
Ankylosing spondylitis is a HLA-B27 associated spondyloarthropathies. It typically
presents in males (sex ratio 5:1) aged 20-30 years old
HLA-B27 is of little use in making the diagnosis as it is positive in:
• 90% of patients with ankylosing spondylitishe
• 10% of normal patientshe
Management
• analgesiahe
• physiotherapyhe
• sulphasalazine may be useful if peripheral joint involvement - doesn't improve
spinal mobilityhe
65.A 69-year-old man presents to his GP with an acute episode of gout on his left first
metatarsal-phalangeal joint. What is the most likely underlying mechanism?ia
A.A Sedentary lifestyleia
x B.A Decreased renal excretion of uric acid
C.A Increased endogenous production of uric acidia
D.A Starvationia
E.A Too much protein in dietia
Next question
The vast majority of gout is due to decreased renal excretion of uric acid
Decreased renal excretion of uric acid is thought to account for 90% of cases of primary
gout. Secondary risk factors such as alcohol intake and medications should also be
investigated
Gout: predisposing factors
Impaired excretion
• drugs: diuretics, low-dose aspirinhe
• renal failurehe
• lead toxicityhe

Increased production
• myeloproliferative/lymphoproliferative disorderhe
• cytotoxic drugshe
• severe psoriasishe
Lesch-Nyhan syndrome
• hypoxanthine-guanine phosphoribosyl transferase deficiencyhe
• inheritance = x-linked recessivehe

• features: gout, renal failure, learning difficulties, head-banginghe
66.Which one of the following features is least typical of polymyalgia rheumaticaia
x A.A Elevated creatinine kinase
B.A Low-grade feveria
C.A Morning stiffness in proximal limb musclesia
D.A Polyarthralgiaia
E.A Anorexiaia
Next question
Polymyalgia rheumatica
Pathophysiology
• overlaps with temporal arteritishe
• histology shows vasculitis with giant cells, characteristically 'skips' certain sections
of affected artery whilst damaging othershe
• muscle bed arteries affected most in polymyalgia rheumaticahe
Features
• aching, morning stiffness in proximal limb muscles (not weakness)he
• also mildpolyarthralgia, lethargy, depression, low-grade fever, anorexia, night
sweatshe
Investigations
• ESR > 40 mm/hrhe
Treatment
• prednisolone e.g. 15mg/od - dramatic responsehe
67.A 47-year-old female is referred to the rheumatology clinic due to cold fingers. Which
connective tissue disease is most strongly associated with Raynaud's phenomenon?ia
B.A Rheumatoid arthritisia
x C.A Systemic sclerosis
D.A Sjogren's syndromeia
E.A Polyarteritis nodosaia
Next question
Raynaud's phenomenon is associated with all the above conditions but is most strongly
linked to systemic sclerosis. Around 2% of women and 6% of men with Raynaud's
phenomenon develop systemic sclerosis
Raynaud's
phenomenon)
symmetrical attacks
• rasheshe


Secondary causes
SLEhe
• leukaemiahe

• cervical ribhe
Management
68.Which one of the following antibodies is most specific for systemic lupus
erythematous?ia
A.A Anti-neutrophil cytoplasmic antibodiesia
B.A Anti-nuclear antibodiesia
x C.A Anti-Sm antibodies
D.A Anti-dsDNA antibodiesia
E.A Anti-cardiolipin antibodiesia
Next question
This is tough as both anti-Sm and anti-dsDNA antibodies are highly specific for SLE.
However, anti-Sm antibodies are slightly more specific
SLE: investigations
Immunology
Monitoring
patients)he
69.Which one of the following is the most common type of psoriatic arthritis?ia
A.A Monoarthritisia
B.A Sacroilitisia
C.A DIP joint diseaseia
x D.A Rheumatoid-like polyarthritis
E.A Arthritis mutilansia
Psoriatic arthropathy
Psoriatic arthropathy correlates poorly with cutaneous psoriasis and often precedes the
development of skin lesions. Around 10% percent of patients with skin lesions develop
an arthropathy with males and females being equally affected
Types*
• rheumatoid-like polyarthritis: (30-40%, most common type)he
• asymmetrical oligoarthritis: typically affects hands and feet (20-30%)he
• sacroilitishe
• DIP joint disease (10%)he
• arthritis mutilans (severe deformity fingers/hand, 'telescoping fingers')he

r asdsadas eerw dssdfsselleds
Management
• treat as rheumatoid arthritishe
• but better prognosishe
*Until recently it was thought asymmetrical oligoarthritis was the most common type, based on data
from the original 1973 Moll and Wright paper. Please see the link for a comparison of more recent
studies
70.A 50-year-old man with no past medical history is investigated for ongoing back pain.
He is found to have a vertebral collapse secondary to osteoporosis. What is the most
appropriate test to determine the cause of his osteoporosis?ia
A.A Thyroid function testsia
B.A Prostate specific antigenia
C.A Oestrogen levelia
D.A Prolactin levelia
x E.A Testosterone level
Next question
Whilst thyrotoxicosis is a known cause of osteoporosis, testosterone deficiency is much
more likely in a middle-aged male
Risk factors
• female sexhe
• smokinghe

diabetes mellitushe
• osteogenesis imperfecta, homocystinuria

71.A 33-year-old female is admitted to the Emergency Department due to right-sided
weakness. She has a past history of deep vein thrombosis following the birth of her
daughter. The only other past history of note is two miscarriages. A CT head confirms an
ischaemic stroke in the left middle cerebral artery territory. What is the likely finding on
echocardiography?ia
x A.A Normalia
B.A Dilated cardiomyopathyia
C.A Bicuspid aortic valveia
D.A Atrial septal defectia
E.A Ventricular septal defectia
Next question
This is a typical MRCP question. On first sight this question appears to be pointing
towards a paradoxical embolus. However, given the history of miscarriages a diagnosis of
antiphospholipid syndrome is more likely
Features

72.A 66-year-old female presents to her GP due to pain at the base of her left thumb. She
has no past medical history of note. On examination there is diffuse tenderness and
swelling of her left first carpometacarpal joint. What is the most likely diagnosis?ia
x A.A Osteoarthritisia
B.A De Quervain's tenosynovitisia
C.A Goutia
D.A Rheumatoid arthritisia
E.A Primary hyperparathyroidismia
Next question
The trapeziometacarpal joint (base of thumb) is the most common site of hand
osteoarthritis
Osteoarthritis
X-ray changes
Management
Glucosamine
significantly reduced joint space narrowing and improved pain scoreshe
73.A 66-year-old female is on long-term prednisolone therapy for polymyalgia
rheumatica. What is the most appropriate protection against osteoporosis?ia
A.A Hormone replacement therapyia
B.A Calcitoninia
x C.A Oral bisphosphonate
D.A Calcium and vitamin Dia
E.A Hip-protectorsia
Next question
Oral bisphosphonate therapy is recommended for patients older than 65 years who have
taken, or who are likely to remain on oral corticosteroids for more than 3 months
Bisphosphonates
Bisphosphonates are analogues of pyrophosphate, a molecule which decreases
mineralisation in bone. They inhibit osteoclasts by reducing recruitment and promoting
apoptosis
Clinical uses
• prevention and treatment of osteoporosishe
• hypercalcaemia of malignancyhe
• Paget's diseasehe
Adverse effects
• gastrointestinal side effects are commonhe
• oesophagitis, oesophageal ulcers - especially alendronatehe
• osteonecrosis of the jawhe
74.Which one of the following is most likely to indicate an underlying connective tissue
disorder in a patient with Raynaud's phenomenon?ia
A.A Chilblainsia
B.A Bilateral symptomsia
C.A Female patientia
D.A Onset at 18 years oldia
x E.A Recurrent miscarriages
Next question
A history of recurrent miscarriages could indicate systemic lupus erythematous or anti-
phospholipid syndrome. Chilblains (pernio) are itchy, painful purple swellings which
occur on the fingers and toes after exposure to the cold. They are occasionally associated
with underlying connective tissue disease but this is rare
Raynaud's
phenomenon)
symmetrical attacks
• rasheshe

Secondary causes
SLEhe
• leukaemiahe
• cervical ribhe

Management
75.Which one of the following conditions is least associated with polyarthritis?ia
A.A Sarcoidosisia
x B.A Amyloidosisia
C.A Tuberculosisia
D.A Henoch-Schonlein purpuraia
E.A Rubellaia
Next question
Polyarthritis
Differential diagnosis
• SLEhe
• seronegative spondyloarthropathieshe
• Henoch-Schonlein purpurahe
• sarcoidosishe
• tuberculosishe
• pseudogouthe
• viral infection: EBV, HIV, hepatitis, mumpshe

76.Which one of the following is most recognised as a risk factor for developing
osteoporosis?ia
x A.A Osteogenesis imperfecta
B.A Marfan's syndromeia
C.A Myotonic dystrophyia
D.A Duchenne muscular dystrophyia
E.A Ehler-Danlos syndromeia
Next question
Risk factors
• female sexhe
• smokinghe

diabetes mellitushe
77.Each one of the following is part of the American College of Rheumatology criteria
for diagnosing rheumatoid arthritis, except:ia
A.A Swelling of PIP, MCP or wrist jointsia
B.A Rheumatoid factor positiveia
C.A Subcutaneous nodulesia
D.A Radiographic evidence of erosions or periarticular osteopeniaia
x E.A Arthralgia not responding to NSAIDs
Next question
Rheumatoid arthritis: ACR criteria
American College of Rheumatology criteria
• requires 4 of the following 7 criteriahe
• sensitivity = 92%, specificity = 89%he
Morning stiffness > 1 hr (for at least 6 weeks)
Soft-tissue swelling of 3 or more joints (for at least 6 weeks)
Swelling of PIP, MCP or wrist joints (for at least 6 weeks)
Symmetrical arthritis
Subcutaneous nodules
Radiographic evidence of erosions or periarticular osteopenia
78.A 33-year-old female presents to her GP 6 weeks after the birth of her first child with a
two-week history of polyarthralgia, fever and a skin rash. First-line investigations show:
ESR 45 mm/hour
he earaer aeraer asdsas eerw dssdfsselleds
A.A Polymorphic eruption of pregnancyia
x B.A Systemic lupus erythematous
C.A Rheumatoid arthritisia
D.A Reactive arthritisia
E.A Pseudogoutia
Unlike many autoimmune diseases systemic lupus erythematous (SLE) often becomes
worse during pregnancy and the puerperium
SLE: pregnancy
Overview
• risk of maternal autoantibodies crossing placentahe
• leads to condition termed neonatal lupus erythematoushe
• neonatal complications include congenital heart blockhe
• strongly associated with anti-Ro (SSA) antibodieshe
79.Which one of the following is least associated with the development of gout?ia
A.A Psoriasisia
B.A Lesch-Nyhan syndromeia
C.A Lymphomaia
x D.A Lithium toxicity
E.A Renal failureia
Next question
Gout: predisposing factors
Impaired excretion
• drugs: diuretics, low-dose aspirinhe
• renal failurehe
• lead toxicityhe
Increased production
• myeloproliferative/lymphoproliferative disorderhe
• cytotoxic drugshe
• severe psoriasishe
Lesch-Nyhan syndrome
• hypoxanthine-guanine phosphoribosyl transferase deficiencyhe
• inheritance = x-linked recessivehe

• features: gout, renal failure, learning difficulties, head-banginghe
80.Which one of the following is most recognised as a potential complication in a patient
with ankylosing spondylitis?ia
x A.A Heart block
B.A Aortic stenosisia
C.A Achalasiaia
D.A Diabetes mellitusia
E.A Bronchiectasisia
Next question
• Apical fibrosis
• Anterior uveitis
• Aortic regurgitation
• Achilles tendonitis
• AV node block
• Amyloidosis

Features
• peripheral arthritis (25%, more common if female)he
• AV node blockhe
• Amyloidosishe

81.A 50-year-old female with a history of rheumatoid presents with a suspected septic
knee joint. Which of the following organisms is most likely to be responsible?ia
x A.A Staphylococcus aureus
B.A Staphylococcus epidermidisia
C.A Escherichia coliia
D.A Neisseria gonorrhoeaia
E.A Streptococcus pneumoniaia
Next question
Septic arthritis
Overview
• most common organism overall is Staphylococcus aureushe
• however
in young adults who are sexually active Neisseria gonorrhoea is the most
common cause (up to 75% of cases)he
82.A 30-year-old female who is known to have antiphospholipid syndrome is diagnosed
as having a deep vein thrombosis. This is her first thrombotic event. How should her
anticoagulation be managed?ia
A.A Life-long low-dose aspirinia
x B.A 6 months warfarin, target INR 2 - 3
C.A Life-long warfarin, target INR 3 - 4ia
D.A Life-long warfarin, target INR 2 - 3ia
E.A 6 months warfarin, target INR 2 - 3 followed by life-long low-dose aspirin
and clopidogrelia
Next question
This is a tough question and some textbooks may contradict this answer, suggesting
either lifelong warfarin or a target INR of 3-4. Please see the link to the BCSH
guidelines. There is also a recent review in JAMA 2006; 295(9): 1050-7
Features

83.Which one of the following is most recognised as a risk factor for developing
osteoporosis?ia
A.A Cystinuriaia
B.A Psoriasisia
C.A Cystinosisia
x D.A Homocystinuria
E.A Lichen planusia
End session
Risk factors
• female sexhe
• smokinghe
diabetes mellitushe
84.Reactive arthritis is associated with which one of the following HLA antigens?ia
x A.A HLA-B27
B.A HLA-A3ia
C.A HLA-DR4ia
D.A HLA-B5ia
E.A HLA-DR3ia
Next question
Reactive arthritis
It encompasses Reiter's syndrome, a term which described a classic triad of urethritis,
conjunctivitis and arthritis following a dysenteric illness during the Second World War.
Later studies identified patients who developed symptoms following a sexually
transmitted infection.
The American College of Rheumatology now define reactive arthritis as an episode of
peripheral arthritis lasting for greater than 1 month associated with urethritis/cervicitis or
diarrhoea
Epidemiology
• post-STI form much more common in men (e.g. 10:1)he
• post-dysenteric form equal sex incidencehe
Organisms often responsible for post-dysenteric form
• Shigella flexnerihe
• Salmonella typhimuriumhe
• Salmonella enteritidishe
• Yersinia enterocoliticahe
• Campylobacterhe
Organisms often responsible for post-STI form
• Chlamydia trachomatishe
85.Which one of the following is least associated with acute sarcoidosis?ia
A.A Erythema nodosumia
x B.A Photosensitivityia
C.A Pyrexiaia
D.A Bilateral hilar lymphadenopathyia
E.A Polyarthralgiaia
Next question
Sarcoidosis
Sarcoidosis is a multisystem disorder of unknown aetiology characterised by non-
caseating granulomas. It is more common in young adults and in people of African
descent
Features
• acute: erythema nodosum, bilateral hilar lymphadenopathy, swinging fever,
polyarthralgiahe
• insidious: dyspnoea, non-productive cough, malaise, weight losshe
• skin: lupus perniohe
Syndromes associated with sarcoidosis
Lofgren's syndrome is an acute form of the disease characterised by bilateral hilar lymphadenopathy
(BHL), erythema nodosum, fever and polyarthralgia. It usually carries an excellent prognosis
In Mikulicz's syndrome there is enlargement of the parotid and lacrimal glands due to sarcoidosis,
whilst in Heerfordt's syndrome (uveoparotid fever) there is parotid enlargement, fever and uveitis
secondary to sarcoidosis
86.Key points from session
Amiodarone & methotrexate are common causes of lung fibrosis
• AV node blockhe
• Amyloidosishe
Anti-cyclic citrullinated peptide antibodies are associated with rheumatoid arthritis

Antiphospholipid syndrome: arterial/venous thrombosis, miscarriage, livedo reticularis
Oral ulcers + genital ulcers + anterior uveitis = Behcet's
The vast majority of gout is due to decreased renal excretion of uric acid
Urethritis + arthritis + conjunctivitis = reactive arthritis
60

Rhumatology

Uploaded by

Copyright:

Available Formats

You might also like

Rhumatology

Uploaded by

Document Information

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

Rhumatology

Uploaded by

Copyright:

Available Formats

1.Which one of the following is least associated with Behcet's syndrome?

A.A Mouth ulcersia

• neurological involvement (e.g. aseptic meningitis)he

x A.A Wegener's granulomatosis

• cannot use level of pANCA to monitor disease activityhe

A.A Maculopapular rashia

Adult onset Still's disease is typically rheumatoid factor negative

• elevated serum ferritinhe

• rheumatoid factor (RF) and anti-nuclear antibody (ANA) negativehe

HCV PCR positive

Rheumatoid factor positive

he earaer aeraer asdsadas eerw dssdfsselleds

• type II (25%): mixed monoclonal and polyclonal: usually with RFhe

• renal involvement (diffuse glomerulonephritis)he

he earaer aeraer asdsadas eerw dssdfsselleds

• recurrent fetal losshe

• other features: pre-eclampsia, pulmonary hypertensionhe

• very rarely: chilblainshe

• type I cryoglobulinaemia, cold agglutininshe

he earaer aeraer asdsadas eerw dssdfsselleds

• some ethnic differences e.g. high in Native Americanshe

Urine dipstick blood +++

he earaer aeraer asdsadas eerw dssdfsselleds

• cyclophosphamide (90% response)he

• median survival = 8-9 yearshe

• dermatitis (lesions can be maculopapular or vesicular)

• cytotoxic agents: busulphan, bleomycinhe

What is the most likely diagnosis?ia

• thought to be primarily a disorder of osteoclasts, with excessive osteoclastic

• bone sarcoma (1% if affected for > 10 years)he

• high-output cardiac failurehe

he earaer aeraer asdsadas eerw dssdfsselleds

• skull x-ray: thickened vault, osteoporosis circumscriptahe

he earaer aeraer asdsadas eerw dssdfsselleds

• cannot use level of pANCA to monitor disease activityhe

• some ethnic differences e.g. high in Native Americanshe

• arthritis mutilans (severe deformity fingers/hand, 'telescoping fingers')he

he earaer aeraer asdsadas eerw dssdfsselleds

• low magnesium, low phosphatehe

• very rarely: chilblainshe

• type I cryoglobulinaemia, cold agglutininshe

he earaer aeraer asdsadas eerw dssdfsselleds

• recurrent fetal losshe

• other features: pre-eclampsia, pulmonary hypertensionhe

• low body weight he

• more common in elderly femaleshe

he earaer aeraer asdsadas eerw dssdfsselleds

Reactive arthritis: features

• macular rash over back and shoulderhe

• respiratory muscle weaknesshe

Achalasia is not a recognised association of ankylosing spondylitis

he earaer aeraer asdsadas eerw dssdfsselleds

• X-ray: early erosions (e.g. after < 2 years)he

he earaer aeraer asdsadas eerw dssdfsselleds

he earaer aeraer asdsadas eerw dssdfsselleds

• ANA/SMA/LKM1 antibodies, raised IgG levelshe

• rare disorder of defective osteoclast function resulting in failure of normal bone

• intra-articular steroid injectionhe

• erysipeloid rash on lower limbshe