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Pathology

Structure of a joint

A joint is a point where 2 bones meet to allow communication. Enthesis is where the tendons/ligaments insert on the bone. Arthritis is a condition where the
joint is inflamed. Typically, manifest as joint pain, monoarthritic/polyarthritis or acute/chronic. The normal hyaline cartilage covering the bones acts as shock
absorbers and wear resistant surface. It has no blood supply, no lymphatic and no innervation. Mostly composed of water. The collagen fibres resist tensile
strength and transmit vertical load.

Groups of Arthritis
- Seronegative spondyloarthropathies → pathologic changes in the ligamentous attachment rather than synovium, involvement of sacroiliac joints/spine with or
without other joints, absence of rheumatoid factor, genetic predisposition of HLA – B27. E.g. Ankylosing spondylitis, reactive arthritis, psoriatic
arthritis, enterohepatic arthritis, juvenile – onset spondyloarthropathies and undifferentiated SpA. T cell response directed against and undefined antigen
(likely infections) which cross react with native molecules of MSK.
- Autoimmune arthritis → E.g. RA, SLE
- Degenerative – OA
- Crystal – Gout; pseudogout
- Infection - Septic

Arthritis OA RA Septic arthritis Gouty arthritis Ankylosing Reactive arthritis Psoriatic arthritis Enterohepatic Lupus arthritis
spondyloarthropathies arthritis
Definition Degeneration of the Nonsuppurative Inflammation of Transient attacks of Inflammatory disorder Post infection arthritis Chronic Arthritis Chronic, remitting and
articular joint and its proliferative and the synovial acute arthritis initiated of unknown cause that with a classic triad of inflammatory associated with relapsing, often febrile
disordered repair inflammatory synovitis membrane with by crystallization of affects the axial arthritis, arthropathy chronic IBD → illness characterized by
→ destruction of purulent effusion monosodium urate skeleton primarily, nongonococcal / associated with UC or CD injury to the skin,
articular cartilage → in joint space (MSU) crystals AKA rheumatoid infectious urethritis / psoriasis joints, kidneys and
ankylosis of the joints within and around spondylitis and Marie cervicitis / diarrhoea, serosal membranes
joints + hyperuricemia – Strumpell disease conjunctivitis →
Reiter's triad
Joints Weight bearing Small joint of the hand Knee joint in 1st Sacroiliac joint and Ankle, knee, feet Peripheral Knees and ankles PIP, MCP, hands and
involved joints → knee, hip, → MCP and PIP; adults, hip joint metatarsophalangeal zygapophyseal joint / polyarthritis → common, wrist
lower lumbar and Polyarticular in children joint (podagra), hip and shoulder DIP of hands and sometimes wrist,
commonly used instep; knee joint; involvement feet fingers, toes
joint → DIP, PIP; ankle joint; wrist joint; *usually at the Axial joints →
Monoarticular elbow joint; finger enthuses, fibrous and sacroiliac joint and
joints; olecranon bursa fibrocartilaginous enthuses
and Achilles tendon connections of
ligaments and tendons
to bone
Aetiology Idiopathic/primary Breakdown of self- Bacterial Hyperuricemia Post infection from Gi infections by Breakdown in the self
due to ageing. tolerance → antibody infection → (plasma urate level > Chlamydia / Yersinia, tolerance leading to
against self-antigen and S.aureus, 0.36 mmol/dL) ureaplasma urealiticum Salmonella, autoantibodies,
Secondary due to cytokine mediated H.influenza, (genitourinary), Shigella and particularly
trauma to joint, inflammation N.gonorrhoeae Increased synthesis Shigella, Salmonella, Campylobacter antinuclear
congenital defects, predominantly secreted via direct Reduced excretion Yersinia, antibodies that is
systemic ds – by the CD4+ T cells trauma, (more common) Campylobacter amplified by the action
diabetes, obesity, hematogenous of of nucleic acid on
hemochromatosis spread, adjacent Primary gout → dendritic cells and the
focus unknown enzyme B cell
defects
Secondary gout → ● Genetic - HLA-
increased uric acid B8;DR3;A1
turnover, CRF, ● Hormonal
congenital (Lesch – ● Environmental -
Nyhan syndrome, EBV infection; UV
HGPRT deficiency) light
● Immunological
Risk factors Increasing age, Genetic predisposition Existing joint Obesity, heavy Male, HLA-B27 Male, HLA-B27 HLA – B27, HLA- Female, child bearing
obesity, genetic → HLA – DRB1 problems (OA, alcohol consumption, CW6 age about 20 - 40 years
factors, female sex, Environmental factors → gout, RA), metabolic syndrome; old,
trauma, infection, infection and smoking dermatological thiazide; duration of
repetitive trauma, problem – hyperuricemia; renal
history of Female, middle age psoriasis insufficiency
inflammatory infected,
arthritis immunocompro
mised, joint
trauma, DM
Pathogenesis Genetic and The genetic Precipitation of MSU Genetic predisposition Genetic predisposition Lipopolysaccharid Susceptible genes +
environmental susceptibility and the crystals on the joint to HLA – B27 → to HLA – B27 + cross es of bacteria cell environmental triggers
factors related to environmental factor triggering complement Release of MHC reaction due to wall stimulate (UV radiation) →
aging and (viral infection) → APC activation and complex containing bacterial DNA found in immune response increased cell
biomechanical stress to T Helper and B cell -> phagocytosis by arthrogenic peptide → inflamed synovium apoptosis but in a
→ degradation of responses to the antigen monocyte. The release of IL – 23 → process → Release of defective manner +
chondroid matrix > in joint tissue. B cell complement activation → Activation of MHC complex inefficient
synthesis in OA. secretes IgM Ab against → neutrophil resident CD 8+ T cell containing arthrogenic phagocytosis →
Chondrocyte is joint antigen → immune chemotaxis → → Release of TNF, IL- peptide → release of IL increased burden of
injured due to the complex formation and phagocytosis → lysis 22, IL 17 → – 23 → → Activation nucleic acid →
aging and deposition on the joint. T of neutrophil → Inflammation of of resident CD 8+ T production of B and T
biomechanical stress cell secretes cytokines to release of lysosomal tendino ligamentous cell → Release of TNF, cells specific to nuclear
factor → attract various enzyme. Release of insertion CAUSING IL- 22, IL 17 → antigen → production
hyperactive state of inflammatory cells. IFN inflammatory ENTHESITIS → Inflammation of of ANA → antigen-
chondrocyte where – G activates cytokines IL-1, TNF, squaring and fusion of tendino ligamentous antibody complexes →
there is active macrophages and IL-6 and IL-8 → the vertebral bodies + insertion CAUSING endocytosis of the
proliferation and resident synovial cells. release of proteases. bony outgrowths → ENTHESITIS complex by B cell via
secretion of IL – 17 recruit Release of lysozyme + severe spinal interaction with nucleic
inflammatory neutrophils and proteases + immobility HLA - B27 increases acid specific TCR +
mediators, collagen, monocytes. TNF & IL-1 prostaglandins, free susceptibility by T cell MHC presentation via
proteoglycans and stimulates resident radicals and LTB4 → Usually affects the receptor repertoire TLR of dendritic cells
proteins → synovial cells to secrete tissue injury and axial skeleton through selection, molecular → stimulation of B and
remodelling of the proteases to destroy inflammation forming mimicry causing T cells by IFN – G →
cartilage occurs → hyaline cartilage. Repeated attacks of syndesmophytes -> autoimmunity against persistently high anti
thickened cartilage RANKL receptors of T acute arthritis leads axial skeleton and HLA - B27 and/or self nuclear IgG
for better shock cells stimulate osteoclast to chronic arthritis sacroiliac joints antigens and mode of
absorption. But this activity. Together all the presentation of bacteria
leads to increased inflammatory cells and - derived peptides to T
articular fibrillation antigen - antibody - lymphocytes.
+ entry of synovial complexes lead to
fluid -> subchondral synovial hyperplasia and
cyst which signals pannus formation.
the long bone Wherever the pannus
thinking not enough sticks cause destruction
bone causing of the bone → fibrosis
increased bone and ankylosis
formation ->
osteophytes

Secondary
inflammatory
changes in
synovium and
subchondral bone →
burn out state where
there is
chondrocyte drop –
out, loss of
cartilage,
subchondral bone
damage
Morphology  Early OA Synovium oedematous, Acute arthritis → Joint aspiration fluid is
(hyperactive) → thickened, hyperplastic dense neutrophil sterile
fissure on articular and with bulbous villi. infiltrate, MSU
surface and Dense inflammatory crystals, synovium
fibrillation infiltrate. Pannus edematous
 Late OA (drop- formation. Juxta articular Chronic tophaceous
out) → joint mice, erosion. Fibrous arthritis → tophi
bone eburnation, ankylosis and bony (MSU crystals +
subchondral cyst, ankylosis. Blood vessels macrophage,
subchondral shows acute necrotizing lymphocyte, giant
sclerosis, vasculitis, obliterating cell), synovium is
osteophytes, endarteritis and fibrotic, hyperplastic
narrowed joint leukocytoclastic and thickened, pannus
space vasculitis + ocular formation that
changes. destroys juxta –
articular bone
erosions, fibrous/bony
ankylosis ensues
Crystals are needle
shaped negatively
birefringent
Clinical Late 50s Rheumatoid nodules, Sudden onset of Asymptomatic Insidious onset of Preceded by Arthritis mutilans → Abrupt onset of Malar rash. Discoid
features Deep, aching pain Joints are swollen + painful, swollen, hyperuricemia → intermittent hip, urethritis/cervicitis/ deforming and arthritis, last for rash, Photosensitivity.
worsens with use warm + painful + reduced ROM acute gouty arthritis buttock or back pain, diarrhoea → acute destructive arthritis, years Pericarditis, Pleuritis,
Morning stiffness - morning stiffness> 1 hr, joint, fever with → intercritical gout → pain worse in morning, onset of arthritis + pencil in cup Oral ulcers, Arthritis,
short duration Boutonniere’s deformity chills, malaise chronic tophaceous rest and in the middle asymmetrical + lower deformity → Renal disorder
Crepitus (PIP in flexion, DIP in arthritis / gouty of the night, stiffness limb and hip joint, joint sausage digits (persistent proteinuria
Limited movement extension), Ulnar nephropathy improves with stiffness, back pain, > 500 mg + cellular
Can affect deviation of MCP, Swan Acute gouty arthritis movement and synovitis of digital Asymmetrical cast), deposition of
bursa/tendon/ neck deformity (DIP → sudden onset of exercise, enthesitis due tendon sheath leading Spondyloarthritis antigen - antibody
muscle - muscle flexion PIP extension), excruciating joint pain inflammation of the to sausage finger/toe, with sacroiliitis and complexes may lead to
wasting of the ROM reduced, + localized warmth, ligaments and tendon, calcaneal spur/bony spondylitis lupus nephritis
quadriceps due to symmetrical, Radial hyperaemia and dactylitis due to outgrowths CNS → seizures and
knee joint deviation of the wrist exquisite tenderness tendinitis, peripheral Asymmetrical / psychosis
involvement arthritis EXTRA MSK S/S bilateral symmetrical Hemato → anemia,
XTRA MSK Chronic tophi – Circinate balanitis, Rheumatoid like leucopenia,
Weight - bearing Fatigue, fever, anorexia, Deposition of the MSK S/S conjunctivitis/acute lymphopenia,
joints weight loss MSU crystals in Low back pain/neck uveitis, keratoderma Nail dystrophy - thrombocytopenia
knee/hip/shoulder/ CVS – pericarditis, subcutaneous layer pain improves with blennorrhagica, oral pitting, onycholysis,
Heberden nodes – myocarditis and is painless; serum exercise, impaired lesions, cutaneous ridging and Arthritis
DIP - hereditary RESPI – bronchiolitis, urea more than 0.54 spinal mobility, eruptions/skin hyperkeratosis Non – erosive,
nodal OA pleuritis w effusion, mmol/dl; Occurs on hyperkyphosis, loss of changes reversible, arthralgia,
Bouchard nodes – interstitial fibrosis, the digits of the hands; lumbar lordosis, Rash first psoriasis myalgia, frank arthritis,
PIP - hereditary cryptogenic fibrosing pinna; elbow; knees; thoracic kyphosis, first → joint pain myopathy, myositis,
nodal OA alveolitis - got cyanosis, Achilles tendon flexion deformities of tenderness, swelling,
Cannot open the jar clubbing, basal crepts the hips effusion of joint,
if the 1st MCP joint relieved by moving recurrent synovitis →
forward XTRA MSK S/S swan – neck
Can cause cervical suspect TB if taking Acute anterior uveitis, deformities,
spondylosis - can biological DMARDS IBD, psoriasis, CVS osteonecrosis,
cause spine GI – hepatitis (ACS, aortitis causing avascular necrosis of
involvement HEMATO – anemia of aortic regurgitation, femoral head
chronic ds causing stroke, conduction
normochromic abnormalities), Respi
Lhermitte's sign normocytic anemia -> (restrictive pulmonary
- flex the neck cause hypochromic microcytic ds, upper lobe
shooting pain down due to UGIB with fibrosis),
spine chronic NSAID use,
vasculitis (nail infarct, Stiffness can be
Lumbar spondylosis skin necrosis and checked via Schober‘s
- pain while flexing mononeuritis multiplex) test where the
and extending the ORAL – xerostomia, extension is less than 5
back periodontitis cm
EYE- scleritis,
Monoarthritic, episcleritis (one painful Neck stiffness can be
asymmetric the other not), tested by asking the
scleromalacia perforans patient to stand against
where some part of the a wall and try to touch
sclera is so thin that it the head to the wall
can perforate they cant
SKIN – subcutaneous
nodules > extensor Lateral flexion and
surfaces + more on rotation (fix hip joint)
pressure points such as is limited
elbow, Achilles tendon
Bursitis Wide inter mallelus
Tenosynovitis - more on distance is big cause
the flexor parts, if occurs protruding belly and
in the finger can cause cannot see feet +
trigger finger balance with flexed
CNS – mononeuritis knee
multiplex and affects
any nerve, Carpal tunnel
syndrome, peripheral
neuropathy due to
vasculitis of vasa
nervorum, cervical
myelopathy →
separation of atlanto
odontoid process
Felty syndrome (RA,
splenomegaly,
neutropenia)
Ix X - ray FBC CBC→ Clinical manifestation X – ray → BAMBOO 1. ANA (screening),
RF which is an Antibody leukocytosis, and SPINE, Anti – dsDNA, Anti
against Fc portion of IgG ESR elevated, HLA – B27 testing - – Sm,(diagnostic)
(screening purpose), anti blood cultures, Joint aspiration (gold 90% 2. CBC
– CCP (diagnostic) urethral/ cervical standard) ESP/CRP 3. Serum creatinine
Xray / pharyngeal / synovial fluid MRI of SI joints 4. Urinalysis
To detect early features rectal cultures, microscopy -> 5. CRP, ESR
do MRI or MSK synovial fluid NEGATIVELY 6. Complement levels-
ultrasound doppler analysis → birefringent needle C3 C4 (both low)
which can show colour - Gram stain shaped crystals 7. LFT
bony erosions and bone 8. CT - infarcts and
edema which is early serum urea and hemorrhage with
erosions creatinine and renal cerebral atrophy
Joint aspiration profile 9. MRI
CXR for CFA and
pulmonary fibrosis Ultrasound KUB to A combo of high ESR
Baseline RP and LFT to look for renal stone + high anti Smith +
monitor treatment SE cause urate stones are low C3 shows acute
radiolucent in X ray flare of SLE
KUB
Types/stages 1. Chronic 1. Acute critical 1. Asymmetric
persistent RA - gout Oligoarthritis -
usually in 2. Intercritical can affect less
seropositive RA, gout than 5 small and
prolonged joint 3. Chronic large joints
pain with polyarticular 2. Symmetrical
intermittent relief tophaceous joints similar
2. Palindromic - gout presentation to
monoarticular, 4. Uric acid rheumatoid
attacks last 24 - nephropathy arthritis
48 hours 3. Distal arthritis
3. Transient - involving DIP
subsides within joints (marked
12 months nail changes)
4. Remitting - many 4. Arthritis mutilans
years with pain - deforming and
and deformity destructive
and one day arthritis
wake up no pain 5. Spondyloarthritis
but still got -> sacroiliitis and
deformity cause spondylitis
of subluxation
5. Rapidly
progressive -
sudden onset
with severe joint
damage

There can be
seronegative RA and
also false positive RF
esp in elderly population
Xray Seagull sign Joint effusion Not useful Punched out bone
Subchondral cyst Juxta articular lesions with an
Subchondral osteopenia overhanging edge
sclerosis Narrowing of joint space
Osteophytes Loss of articular Takes years to
Bony eburnation cartilage develop
Narrowing of joint Subluxation
pain
Better to do yearly X
Knee OA - standing Ray sometimes no more
pain but ongoing
Kellgren Lawrence inflammation Bamboo spine,
Grading System Romanus lesion on
MRI
Dx Revised eular ACR
criteria
>6 score for the RA:
(joint involvement,
serology for RF or
ACPA, acute – phase
reactants CRP or ESR,
duration of symptoms < /
> than 6 weeks)
Tx 1. Reduce weight 1. Patient education Antibiotic Diclofenac sodium, 1. Patient education Antibiotics to treat 1. NSAIDs Treatment of IBD 1. Cutaneous
2. Pain management and counselling therapy, joint indometacin, (sleep on foam infections, NSAIDS, 2. Synthetic + ulcerative colitis symptoms -
activity 2. NSAIDs (anti - immobilization allopurinol >2 attacks mattress, exercise, immunosuppressive, DMARDs - has colectomy sunblock and topical
modification, inflammatory) and physical per year; xanthine avoid smoking) corticosteroid, physical (methotrexate, (surgical options) steroids
3. Arthroplasty 3. Synthetic therapy, synovial oxidase inhibitor; do 2. Biological therapy, eye drops sulfasalazine, 2. Maintenance/
4. Knee joint DMARDs - fluid drainage not start during acute DMARDs - cyclosporine, symptomatic -
replacement methotrexate (folate attack, colchicine, entercept, gomali leflunomide) NSAIDS
5. intra articular antagonist) -> intra articular mumab 3. Hydroxychloroqu 3. If not
corticosteroids pulmonary fibrosis corticosteroids 3. Do not use ine flares up the controlled by
injection takes x ray tro CFA, injection methotrexate - psoriasis NSAIDS -> HCQ
hepatotoxicity so cause it worsens 4. Biological 4. Acute flare ->
need to monitor Why treatment fails? the spine problem DMARDs - TNF IV
LFT if ALP doubled Non-compliance, diet 4. NSAIDS everyday and cytokine cyclophosphamide +
cannot use, high in red meat, - naproxen, blockers high dose
immunosuppression alcohol, dose not celecoxib, (Etanercept, prednisolone
; can mask TB; enough ibuprofen Infliximab) 5. Maintain
sulfasalazine; 5. Physiotherapy - 5. Intra – articular remission -
hydroxychloroquine Drink a lot of water to pilates, swimming, corticosteroid azathioprine
; leflunomide prevent dehydration sitting with injections 6. Refractory -
4. Biological which may precipitate touching the floor rituximab and
DMARD - Anti stones sideways belimumab
TNF, Anti IL- 6; 6. Surgery
greatest side effects Avoid aspirin cause
are surfaces may precipitate attack
underlying infection
(need to TEST TB Probenecid may cause
so do quantiferon uric acid stones but
test or more than 5 not as effective as
mm induration so allopurinol
start TB
prophylaxis) + Allopurinol - Steven
expensive Johnsons syndrome
5. Physiotherapy,
occupational Colchicine - causes
therapy nausea and diarrhea
6. Corticosteroids -
not more than Tx for lowering
7.5mg because hyperuricemia should
steroid has many SE not be indicated
during acute attack
Staging DAS 28 - ESR/CRP and
tender swollen joint of
UL 28 joints - to stage
the disease and monitor
treatment and need to
change drug
Complication Predisposes to septic Systemic amyloidosis Destructive Cervical fracture; heart Neuropsychiatric ds
arthritis arthropathy and block and amyloidosis and Raynaud's
secondary OA; also - death phenomenon
have tenosynovitis

Pseudogout → Calcium Pyrophosphate Crystal Deposition disease, rhomboid positively birefringent crystals, autosomal dominant; linear calcification of the knee (chondrocalcinosis); common after 65; tx NSAIDs
colchicine and steroids

Joint aspiration → important test for monoarthritis (usually due to infection, trauma, crystals): visual inspection, cell count, culture and sensitivity, Gram stain, crystals
SLE
Scleroderma
● Presence of thickened and hardened skin
● systemic vasculopathy of small and medium sized; excessive collagen by a systemic vasculopathy of the small and medium sized vessels
● Subtypes
○ Systemic sclerosis - cutaneous involvement + interstitial lung disease + renal involvement + cardiac involvement (ECG shows HB)
■ Limited cutaneous
■ Diffuse cutaneous
○ Localized scleroderma - only cutaneous involvement
● Pathophysiology
○ Vasculopathy - fibroproliferative lesion of the small arteries and arterioles
○ Immunological/inflammation - alteration of cellular and humoral antibody causing production of autoantibodies
○ Fibrosis - excessive and progressive deposition of collagen and ECM in skin and various organs

● Clinical manifestations Ask to put 3 fingers in the mouth and patient is unable due to tight skin

Localized scleroderma -
● En coup de sabre – Denote the resemblance of the lesions to the consequences of a sabre blow. The face or scalp lesion is accompanied
by marked abnormalities of underlying mesenchymal-derived tissues, including the skull
● Morphe - skin sclerosis on the trunk and limb can occur symmetrically

Limited systemic sclerosis - CREST syndrome

● ● Diagnosis
○ Autoantibodies
■ ANA

■ Anti-centromere
■ Topoisomerase I antibody
○ Clinical features
■ Esophagus involvement- Barium swallow
■ Cardiac and pulmonary involvement - ECG, Echocardiogram, chest X Ray, Pulmonary function test, HRCT
■ MSK involvement ( myositis)- Creatine phosphokinase (CPK) level and electromyography
■ Renal involvement- Serum Creatinine level, Urinalysis
● Treatment
○ Physiotherapy
○ Stop smoking
○ NSAIDs for Musculoskeletal pain.
○ H2 Blocker or PPI for esophageal reflux
○ Raynaud phenomenon- avoid cold and smoking, keep hands warm; if severe, use calcium channel blockers
○ Pulmonary complications- for pulmonary hypertension, treat with Bosentan (Endothelin Receptor Antagonists). For pulmonary fibrosis. Cyclophosphamide(useful for reduction of skin thickening in
patients with early diffuse scleroderma with rapidly progressive disease ) is used.
○ ACE inhibitors are used to prevent and treat renal hypertensive crisis.

Drug Dose Side effects

Methotrexate Weekly dose of 7.5-10mg orally, - Mouth ulcers, nausea,


increased up to 15-25mg as necessary diarrhea
(CI: pregnancy) to reduce disease activity - Neutropenia and/or
thrombocytopenia

Sulfasalazine Usual starting dose of 500mg daily, - Skin rashes/ mouth ulcers
increased to maintenance dose of 2-3g - Neutropenia and/or
(can be used during per day. thrombocytopenia
pregnancy)

Leflunomide 20mg daily (10mg daily if diarrhea) - Diarrhea


- Neutropenia and/or
thrombocytopenia

Hydroxychloroquine 200-400mg daily Retinopathy (rare)

Sjogren's syndrome
- dry mouth and dry eyes
- Schirmer's test - put a paper in lower eyelid for 5 min n see how much tear produced
- autoimmune disease - lymphocytic infiltration of the lacrimal and salivary gland
- autoantibodies - anti-Ro (SSA)

Lumbar spinal stenosis


- frequent cause of back pain in the elderly
- pain radiating into the buttock and pain on walking and on lumbar extension + decrease in vibratory sense and a wide base gait
- age related degenerative changes

Polymyalgia rheumatica
- headache; transient loss of vision in the right eye; morning stiffness and shoulder and neck pain + s/s improves through the say and absence of joint problem on PE; mild anemia; increased ESR
- diffuse myalgias and joint stiffness particular shoulders and hips
- must ask about jaw pain
- diagnosis is confirmed if S/S improve with moderate dose of steroids

Temporal arteritis
- headache; transient loss of vision in the right eye;shoulder and neck pain; mild anemia; increased ESR
- headache + amaurosis fugax + S/S of polymyalgia rheumatica + JAW CLAUDICATION
- only 25% of the PMR have giant cell arteritis
- temporal artery biopsy - vasculitis
- does not respond to moderate dose steroids so high dose steroids

Fibromyalgia
- >72 years old; multiple tender points; sleep disturbance; dropping things cause of weakness; lack of joint and muscle findings; more common in women
- normal CBC and ESR; 11 tender points
- Tx: cognitive behavioural therapy and graded aerobic exercise

Polymyosisits/Dermatomyositis
- Inflammation of the striated muscle ->proximal muscle weakness +/- skin
- 40 - 50 years; W:M = 3:1
- P/W progressive proximal myopathy (difficulty in rising from the chair; bathtub; climbing stairs), wasting of the shoulder and pelvic girdle muscle but non - tender
- As disease progresses -> failure of pharyngeal, laryngeal and respiratory muscle -> dysphonia and respiratory failure
- Dermatomyositis -> Heliotrope rash (purple eyelids + periorbital edema); gottron's papule (purple red raised vasculitic patches over the knuckles) -> long term may cause muscle fibrosis and joint contracture
- Ix: Elevated CK, ALTs/ASTs, LDH; ESR and CRP raised; screen autoantibodies (ANA, RF), EMG (fibrillations; spontaneous discharge; sharp waves); muscle biopsy; screen for malignancy
- Tx: Bed rest + exercise -> HIGH DOSE corticosteroids + methotrexate and azathioprine

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