Ahmad Salh Soboh

Rheumatology

Diseases: Harrison’s Book

1. Osteoarthri,s Chapter 394 – Page 2226
2. Rheumatoid Arthri,s Chapter 380 – Page 2136
Seronega(ve Spondyli(s
3. Axial Spondylarthri,s Chapter 384 – Page 2169
4. Ankylosing Spondyli,s Chapter 384 – Page 2169
5. Psoria,c Arthri,s Chapter 384 – Page 2175
6. Enteropathic Arthri,s Chapter 384 – Page 2178
7. Reac,ve Arthri,s Chapter 384 – Page 2173
Gout and Other Crystal-Associated Arthropathies
8. Gout
Chapter 395 – Page 2233
9. Pseudogout
Connec(ve Tissues
10. Systemic Lupus Erythematous (SLE) Chapter 378 – Page 2124
11. An,phospholipid Syndrome Chapter 379 – Page 2134
12. Systemic Sclerosis (Scleroderma) Chapter 382 – Page 2154
13. Sjögren’s Syndrome Chapter 383 – Page 2166
The Vasculi(s Syndromes
14. Giant Cell Arteri,s Chapter 386 – Page 2188
15. Takayasu Arteri,s Chapter 386 – Page 2189
16. Granulomatosis with Polyangii,s (WEGENER’S) Chapter 386 – Page 2182
17. EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURG-STRAUSS) Chapter 386 – Page 2186
18. Behçet’s Syndrome Chapter 387 – Page 2194
Inflammatory Myopathies
19. Dermatomyosi,s
20. Polymyosi,s Chapter 388 – Page 2194
21. Inclusion body Myosi,s
 Ahmad Salh Soboh

Osteoarthri*s
à Osteoarthri:s is the leading cause of arthri:s in the adult.
à Mechanical 'wear and tear' destroys ar:cular car:lage (“degenera:ve joint disease”).

Clinical Features:
è Tenderness
è Morning s:ffness ≤30 minutes and worsen with ac:vity.
è Limita:on of mo:on
è Swelling
è Deforma:on
è Instability
è Rhizarthrosis - Arthri:s of the first carpometacarpal (CMC) joint of the thumb
è Nodular OA
- Heberden’s nodes on the DIP joints
- Bouchard’s nodes on the PIP joints
è Joint s,ffness in the morning that worsens during the day

Diagnosis:
# Diagnosis is made with clinical and x-ray findings.
è Rou,ne Lab Test:
- CBC
- Crea:ne level
- CRP & ESR à To rule out Rheumatoid Arthri:s
- LFTs
è Specific An,bodies
#No specific an:bodies because it is mechanical changes. Lab tests are always NORMAL
- An:nuclear an:body (ANA) à To rule out connec:ve :ssue disorders.
- Rheumatoid factor à To rule out Rheumatoid Arthri:s
è The most accurate test is radiography of the affected joint. X-rays show:
o Joint space narrowing.
o Osteophytes.
o Dense subchondral bone.
o Bone cysts.

Treatment:
è Primary preven,on:
- Change lifestyle
- Obese pa:ents should lose weight
- Swimming
- Exercise
è For pain à Acetaminophen, Nonsteroidal An:-Inflammatory Drugs (NSAIDs), and
Cyclooxygenase-2 (COX-2) Inhibitors.
#When NSAIDs aren’t the choice
- CV risks
- GI risks
- Allergy
è Intra-ar:cular injec:on of Glucocor:coids
#When NSAIDs aren’t enough
 Ahmad Salh Soboh

Rheumatoid Arthri*s
à Rheumatoid arthri:s (RA) is a chronic inflammatory disease of unknown e:ology marked by
a symmetric, peripheral polyarthri:s.
à Risk factor of SMOKING

Clinical Features:
è Pain 5 or more of small joints (MCP, PIP, MTP) NOT DIP!!!
=
è Pain is symmetrical.
è Morning s:ffness >30 minutes and improve with ac:vity.
è RA deformi:es:
- Ulnar devia:on
- Baker cyst - swelling of bursa behind the knee
- Swan neck deformity
- Hummer toe

Diagnosis:
è Physical Examina,on
- Gaenslen’s sign
- Phalen’s maneuver
- Tinel's Test
è Rou,ne Lab Test:
·- CBC –
• Anemia of chronic disease is seen in most pa:ents with RA, and the degree of anemia is
propor:onal to the ac:vity of the disease.
The Body &• Thrombocytosis is common when RA is ac:ve, with platelet counts returning to normal as the
produce to inflamma:on is controlled.
many plat et - Crea:ne level – may normal
·- CRP & ESR - mostly elevated
·- LFTs – may normal
è Specific An,bodies
- RF – posi:ve
- An:-CCP – posi:ve
à if An:-CCP was nega:ve should do:
• An:-MCV – posi:ve
• An:-CAR-P an:bodies – posi:ve
è X-ray
- Abnormali:es – peripheral osteopenia, joint space narrowing, subchondral cysts, erosions.

Treatment:
è DMARDs – Methotrexate
#Hydroxychloroquine if she plans to be pregnant
è Glucocor:coids – Screening for infec:ons before
 Ahmad Salh Soboh

Spondylarthri6s

A group of diseases that aren’t linked to specific autoan:bodies and are characterized by a triad:
} Sacroilii:s - inflamma:on of the sacroiliac joints
} Spondyli:s - inflamma:on of axial spine
} Snthesi:s - inflamma:on of tendon, fascia, and ligament inser:on sites
Ankylosing Spondyli*s or Axial Spondylarthri*s
à Mostly affects young men – age of onset is <45 years

Clinical Features:
è Low back pain or Bupocks pain
è Morning s:ffness > 30 min
è Inflammatory Back Pain – The pain worsens at rest
and relieves with movement
è Enthesi,s Achilles Tendon
è Dactyli,s known as “sausage digit”
è Chest Pain
è Uvei:s
è Psoriasis
è First-degree AV block – symptoms à palpita:ons or syncope, slow or irregular pulse.
è Aor:c Regurgita:on

History taking
Diagnosis:
èThe loca)on and rhythm of pain
è History taking èDura)on of pain
è Physical examina,on èAddi)onal risk factors, such as: IBD, Uvei)s, Psoriasis (other skin
- Fabere test abnormali)es), Dactyli)s.
- Forward bending test èFamily history of Psoriasis or arthri)s
èHistory of Cardiac problems
- Occiput to the wall test
è Rou,ne Lab Test:
- CBC
- Crea:ne level
- CRP & ESR – May Elevated
- LFTs
è Specific An,bodies
- HLA B 27 – can be posi:ve.
- There are no specific lab tests.
è MRI à Showing sacroilii:s.
è The best ini:al test is an X-RAY of the sacroiliac (SI) joint to iden:fy if Ankylosing Spondyli:s or Axial
Spondylarthri:s. Order x-rays
X ray Posi*ve
- If showing sacroilii,s can confirm the diagnosis of Ankylosing Spondyli,s.
- If NOT showing sacroilii,s can confirm the diagnosis of Axial Spondylarthri,s. X ray Nega*ve

Treatment:
è NSAIDs
è Physical therapy
è TNF alfa inhibitors
# Glucocor,coids doesn’t work !!!!!!!!
 Ahmad Salh Soboh

Radiology Studies:

Lower third of sacroiliac joint is the more interes,ng:

Grade I: suspicious - blurring of the joint margins
Grade II: minimal sclerosis & joint margin erosions

#Not enough to make diagnosis of Ankylosing Spondyli,s it s:ll Axial.

Grade III - definite sclerosis and erosions, joint space narrowing/false widening

Grade IV – Ankylosis

Axial Spondyli3s
MRI – “shiny corners” or Romanus lesions Bamboo spine
 Ahmad Salh Soboh

Psoria(c Arthri(s
à It is a chronic inflammatory arthri:s associated with skin and nail psoriasis.

Clinical Features:
è Plaque Psoria:c
è five paperns are described:
(1) arthri:s of the DIP joints;
(2) asymmetric oligoarthri:s;
Scalp Seborrhea
(3) symmetric polyarthri:s similar to RA;
(4) axial involvement (spine and sacroiliac joints);
(5) arthri:s mu:lans, a highly destruc:ve form of disease.
è Dactyli,s known as “sausage digit”.
è Enthesi:s and tenosynovi:s are also common and are probably present in most pa:ents,
è Six paperns of nail involvement are iden:fied: pikng, horizontal ridging, onycholysis,
yellowish discolora,on of the nail margins, dystrophic hyperkeratosis, and combina:ons
of these findings.

Diagnosis:
è Rou,ne Lab Test:
- CBC – Normal, no chronic inflammatory anemia
- Crea:ne level
- CRP & ESR – correlates to disease ac,vity
- LFTs
è Specific Lab Test
- Uric Acid – Up to 20% of pa:ents have hyperuricemia.
è Specific An,bodies
- HLA -B27 posi:ve
è Radiology – The best ini:al test is an x-ray of the joint showing a “pencil in a cup” deformity
or Mouse ears.

Treatment:
è DMARDs – MTX, Sulfasalasine
è GCs - Intra-ar:cular injec:ons of glucocor:coids
è Biologic therapy – TNF alfa inhibitors:
- etanercept
- infliximab
 Ahmad Salh Soboh

Reac*ve Arthri*s
à It is an inflammatory arthri:s that develops ater the:
} Gastrointes,nal infec,ons caused by Shigella, Salmonella, Campylobacter, or Yersinia.
} Genitourinary tracts infec,ons, most commonly Neisseria gonorrhoeae, Chlamydia
trachoma:s and HIV.
à Reac:ve arthri:s frequently develops 1–4 weeks ater

Clinical Features:
A
è Joints of the lower extremi:es are affected mostly - knees, ankles, and feet are characteris:c.
è Dactyli,s known as “sausage digit”.
è Onychodystrophy known as “Reiter’s nails”.
è Characteris:c skin rash known as “blennorrhagica keratoderma.”

Blennorrhagica Keratoderma Reiter’s nails “sausage digit”

Diagnosis:
#
è Rou,ne Lab Test:
- CBC - Leukocytosis, thrombocytosis, and elevated serum immunoglobulins
- a mild normocy:c, normochromic anemia
- Crea:ne level
- CRP & ESR
- LFTs
è Specific Lab Test:
- Cultures should be taken from the blood, urine, stool, and throat as indicated.
è Specific An,bodies
- HLA-B27 - posi:ve in about 50%
- There are no specific lab tests.
è Radiology – sacroilii:s, enthesi:s
è

Treatment:
è NSAIDs
è An:bio:cs – ciprofloxacin, doxycycline, or azithromycin
è Glucocor:coids – Intra-ar:cular glucocor:coids may be considered
è DMARDs – Sulfasalazine
 Ahmad Salh Soboh

Enteropathic Arthri(s
à It develops in nearly 20% of all pa:ents with Chron’s disease and ulcera,ve coli,s.

Clinical Features:
A Enteropathic arthri:s most commonly is a peripheral non-erosive arthri:s, oten migratory.
è Usually correlates with bowel ac:vity and is usually self-limi:ng;
è Erythema nodosum and pyoderma gangrenosa may be cutaneous manifesta:ons with the
peripheral arthri:s.
è Isolated sacroilii:s without spine involvement may also occur.

Diagnosis:
è Rou,ne Lab Test:
- CBC
- Crea:ne level
- CRP & ESR
- LFTs
è Specific An,bodies
- There are no specific lab tests.

Treatment:
è Frequently, treatment of the underlying bowel disease leads to remission of the peripheral
arthri:s.
è If this persists, sulfasalazine can be effec:ve.
è NSAIDs are avoided as first-line therapy, because they may worsen the inflammatory bowel
disease
è If sulfasalazine fails to fully control symptoms, then an:-TNF agents should be used
 Ahmad Salh Soboh

Gout and Other Crystal-Associated Arthropathies

Gout
Risk Factors:
è Male sex
è Arterial Hypertension
è Obesity
è Diabetes mellitus type II
è Thiazide diure:cs
è Excess alcohol use

Clinical Features:
è Asymptoma:c hyperuricemia.
è Acute and intermipent (or intercri:cal) gout.
è Chronic tophaceous gout.
è The acute apack may be accompanied by fever, chills, and malaise.
è An affected joint typically is swollen, red, hot, and tender as well as tophi.

Diagnosis:
# Gout is clinical diagnosis
è Rou,ne Lab Test:
- CBC
- Crea:ne level
- CRP & ESR
- LFTs
è Specific Lab Test:
- Serum Uric Acid – Should not check during acute apack (During an acute apack, serum uric acid
may be normal or low).
- Urinalysis – Low pH

è Synovial fluid analysis

è Ultrasound may aid earlier diagnosis by showing a DOUBLE CONTOUR SIGN overlying the
ar:cular car:lage.
è X ray – “Rat-bite” erosions with sclero:c margins.
è Synovial fluid analysis shows typically needle shaped
crystals are seen both intracellularly and
extracellularly

Treatment:
è Colchicine – best op:on if not passed > 24 hours for apack debuted (avoid if kidney/liver failure)
è NSAIDs – if passed >24 hour from apack begin (avoid if kidney failure)
è Glucocor,coids – if passed >24 hour +renal/hepa:c failure
Primary Preven,on:
è Change diet (Avoid beer or alcohol and red meat)
è Stopped all hypertension drugs
è Management for uric acid by Allopurinol (avoid if renal failure) or Febuxostate
 Ahmad Salh Soboh

Pseudogout
à Calcium Pyrophosphate Deposi:on Disease, or “Pseudogout”.
à Remember the 4 Hs. The presence of pseudogout in a pa:ent age <50 should raise
suspicions about one of these metabolic abnormali:es:
o Hyperparathyroidism. (↑ parathyroid hormone in the bloodstream.)
o Hemochromatosis (it is a disorder in which the body can build up too much iron in the
skin, heart, liver, pancreas, pituitary gland, and joints.)
o Hypophosphatemia (is a condi:on in which your blood has a low level of phosphorous.)
o Hypomagnesemia (↓ Magnesium)

Clinical Features:
è Acute monoarthri:s
è Most frequently affected joint is a knee, however wrists are commonly involved as well.
è CPPD differs from gout in that large joints such as the knee and wrist are affected, but not
par:cularly the first MCP of the foot.

Diagnosis:
è Rou,ne Lab Test:
- CBC
- Crea:ne level
- CRP & ESR – Elevated.
- LFTs
è Specific Lab Test:
- Electrolytes:
o Magnesium ↓
- Thyroid Func:on Test
o PTH
è Ultrasound shows calcifica:on of the car:laginous structures of the joint (chondrocalcinosis).
è X-ray shows white lines of Chondrocalcinosis.
è Synovial fluid analysis shows demonstra:on of typical rhomboid or rodlike crystals.
# Without appropriate analysis of synovial fluid, it may be impossible to differen:ate pseudogout
from sep:c arthri:s or a gout.

Treatment:
è NSIADs
è Intra ar:cular injec:on of glucocor:costeroids.
 Ahmad Salh Soboh

Radiology Studies:

Gout Pseudogout

Synovial fluid analysis:

Gout Pseudogout
 Ahmad Salh Soboh

Connec6ve Tissues

Systemic Lupus Erythematous (SLE)

à Systemic lupus erythematosus (SLE) is an autoimmune disorder with a number of
autoan:bodies (ANA, double-stranded DNA).

Clinical Features:
è Fa:gue, fever, malaise, lymphadenopathy, weight loss
Skin manifesta,ons:
è (1) Malar rash.
è (2) Discoid rash.
è Alopecia (means hair loss)
è Hypopigmenta:on or hyperpigmenta:on.
è (3) Photosensi:vity: flare with exposure to UV-B light (thus are considered photosensi:ve)
and resolve with no scarring of the skin.
è (4) Oral ulcers are painless (sot and hard palate)

Discoid rash Oral ulcers Photosensi:vity

Alopecia Pigmenta:on Malar rash

(5) Joint Manifesta,ons:

è Polyarthri:s
(6) Serosi,s:
è Inflamma:on of the pleura and pericardium gives chest pain poten:ally with both
pericardial and pleural effusion.
è Pleuri,s
è Pericardi,s
è Myocardi,s
è Valvular insufficiencies, most commonly of the mitral or aor,c valves, or to embolic events.
(7) Renal Manifesta,ons:
è Nephri:s
è The most common glomerulonephri:s is membranous.
 Ahmad Salh Soboh

(8) Neurologic Manifesta,ons:

è Headache
è Seizures
è GBS
è Stroke from vasculi:s
(9) Hematologic Manifesta,ons:
è Hemoly:c anemia
è Lymphocytopenia (or Lymphopenia) is also common manifesta:on.
è Leukopenia may be an early clue to the diagnosis.
è Thrombocytopenia associated with a risk of bleeding or thrombosis.

J
Diagnosis:
è Rou,ne Lab Test:
at leastIf from
- CBC
- Crea:ne level this list to
- CRP & ESR mostly elevated
make
- LFTs
è Specific An,bodies the
Antinuclear &- (10) ANA (98%) à Homogenous Paqren
antibody Rigt dignosis .

- An:-dsDNA (70%)
.

(11) - An:-Sm (20%)

Associated with drug-induced lupus:
- An:phospholipid an:bodies 1. Procainamide
- An:-histone (70%) 2. Hydralazine
3. Isoniazid
- An:-Ro/SSA
An,-Ro SSA & An,-La SSB: They add li7le to the diagnosis if the DNA is posi,ve. These tests
- An:-La/SSB are most oBen found in Sjögren’s syndrome (65% of cases).

è 4 criteria from 11 confirms diagnosis.

è Should order COMPLEMENT LEVELS (C3 and C4)

COMPLIMENT LEVELS it is disease ac:vity measure:
à Hypocomplementemia - resul:ng in low C4, C3, and CH50 à high disease ac:vity.
à Hypercomplementemia or normal - low disease ac:vity.

Treatment:
è Primary Preven:on: Sun protec:on, Vaccina:ons, Exercise, No smoking, Body weight, Blood
pressure, Lipids, and Glucose.
Mild Moderate Severe
Hydroxychloroquine 1st
Glucocor)coids IV (low doses of systemic glucocor)coids may be necessary) 1st
Methotrexate or Azathioprine 2nd Methotrexate or Azathioprine 1st
Belimumab 2nd
Cyclophosphamide 1st
Rituximab
 Ahmad Salh Soboh

An*phospholipid Syndrome
à A An:phospholipid syndrome is an auto immune disease and an important cause of recurrent
arterial and venous thrombosis and miscarriages
à It’s more common in young females and might be primary or secondary, that occurs with SLE

Clinical Features:
è A sudden thrombo,c event (Deep Venous Thrombus (DVT) or arterial thrombus)
è Miscarriage is the sudden loss of a pregnancy before the 20th week.
è Livedo re,cularis à

è Arterial manifesta,ons:

Diagnosis:
è Rou,ne Lab Test:
- CBC
- Crea:ne level
- CRP
- ESR
- LFTs
è Specific An,bodies
- Lupus an:coagulant.
- An:cardiolipin an:bodies.
- An:-beta2-glycoprotein-I an:body
# As the rule 2 from 3 confirm diagnosis

Treatment:
è Warfarin alone or combined with aspirin
è Pregnancy morbidity is prevented by a combina:on of heparin with aspirin.
è Increasing evidence shows that azathioprine and hydroxychloroquine can be con:nued in
pregnancy.
è (ACE) inhibitor or mycophenolate mofe:l for renal disease, should be stopped.
è Methyldopa, labetalol and nifedipine should be used to control pressure.

Complica(ons:
è It will be secondary to SLE.
 Ahmad Salh Soboh

Scleroderma
à Scleroderma is the general term for (crest syndrome or systemic sclerosis).
à We can iden:fy by:
1. Crest Syndrome [limited scleroderma] (only CREST syndrome SCL-70 + An:centromere +ve)
2. Systemic Sclerosis (CREST syndrome SCL-70 + An:centromere +ve + Internal organs involvement).

Clinical Features:
è Raynaud phenomenon
è Gastroesophageal reflux
è Fa:gue
è Musculoskeletal pain
è Crest Syndrome à

Diagnosis:
# The diagnosis is made by obtaining a careful history and performing a physical examina:on.
è Rou,ne Lab Test:
- CBC
- Crea:ne level (Check the kidneys)
- CRP
- ESR
- LFTs
è Specific An,bodies
First step:
- ANA (superficial screening test) if posi:ve go to second step à
Second step:
ANA with western blo|ng or immunoblo|ng
- An:-SCL-70 = Posi:ve
- An:centromere = Posi:ve
è CT scans of the chest have an increased sensi:vity and may provide some insight into
disease ac:vity and severity (eg, fibrosis or ground glass opaci:es).
è Echocardiogram à to rule out cardiovascular diseases.
è Nailfold capillaroscopy
- Giant capillaries
- Avascular areas
- Microhemorrhages

Treatment:
è DMARDs – Methotrexate, Azathioprine (If no lungs or kidneys is involvement.)
è Pulmonary fibrosis: Glucocor:costeroids – it’s avoid in case of scleroderma, just use in case of
lung fibrosis.
è Hypertension – ACE inhibitors
è Esophageal dysmo,lity: PPIs for GERD
è Raynaud: Calcium Channel Blockers
 Ahmad Salh Soboh

Sjögren’s Syndrome
à Sjögren’s syndrome is a chronic, slowly progressive autoimmune disease characterized by
lymphocy:c infiltra:on of the exocrine glands resul:ng in xerostomia and dry eyes.

Clinical Features:
A
è Low-grade fever
è Generalized pain
è Myalgias
è Fa:gue
è Weakness
è Fibromyalgia
è Polyadenopathies
Symptoms related to diminished lacrimal and salivary gland func:on:
è Oral dryness (Xerostomia)
è Ocular dryness (Xerophthalmia), corneal ulcers, Keratoconjunc:vi:s, Itchy eyes with a
“sandy feeling”.
è Dry skin (Xerosis)
è Nasal dryness

Diagnosis:
è Rou,ne Lab Test:
- CBC
- Crea:ne level
- CRP
- ESR – Elevated
- LFTs
- RF - Posi:ve
è Specific An,bodies
First Step:
- ANA (superficial screening test) if posi:ve go to second step à
Second Step:
ANA with western blo|ng or immunoblo|ng
- An,-Ro SsA, An, La-SsB an:bodies are posi:ve. (If one of them elevated it will be
Sjögren syndrome.)
è Schirmer’s test à posi:ve when it is less than 10mm à
è Salivary gland ultra sound
We always try to avoid biopsy if you have enough criteria to make the diagnosis because
biopsy isn’t necessary.
è If we have lymphadenopathy, we have to do peripheral lymph nodes ultrasound.

Treatment:
è DMARDs
è Glucocor:coids
è Keep eye and mouth moist - water up to 2-2.5 L/d.
è b-DMARDs – Rituximab
 Ahmad Salh Soboh

The Vasculi6s Syndromes

 Ahmad Salh Soboh

GIANT CELL ARTERITIS AND POLYMYALGIA RHEUMATICA

à New onset headache ater age 50

Clinical Features:
è Headache
è Jaw claudica:on
è Visual symptoms Blindness .

è Malaise
è PMR (Polymyalgia rheuma:ca) – pain, s:ffness and inflamma:on in the muscles around the
shoulders, neck and hips.

Diagnosis:
è Physical examina,on:
- may reveal an abnormal temporal artery - enlarged, difficult to compress, nodular, or
pulseless.
è Rou,ne Lab Test:
- CBC - anemia
- Crea:ne level
- CRP
- ESR >100 mm/hour
- LFTs
è Biopsy specimen with artery showing vasculi:s characterized by a predominance of
MONONUCLEAR CELL INFILTRATION or granulomatous inflamma:on, usually with
mul:nucleated giant cells.
è Chest x-rays à an aor:c aneurysm is a well-known complica:on.

Treatment:
#When TA is suspected and ESR is elevated, start cor:costeroids immediately, before the temporal
artery biopsy is performed. Do not withhold treatment wai:ng for the biopsy to be done.
è PMR – Low-dose oral Glucocor:coids.
è GCA – High-dose oral Glucocor:coids.
è GCA + Vision loss – High-dose IV Glucocor:coids.
 Ahmad Salh Soboh

Takayasu Arteri*s
à Takayasu arteri:s is a chronic large artery vasculi:s that classically involves the aor:c arch at
branch points.

Clinical Features:
è Malaise
è Fever
è Night sweats
è Arthralgias
è Anorexia
è Weight loss
è Hypertension
è Unequal blood pressures in the extremi:es

Diagnosis:
#The diagnosis of Takayasu arteri:s should be suspected strongly in a young woman who
develops a decrease or absence of peripheral pulses, discrepancies in blood pressure, and
arterial bruits.
è Physical Examina,on:
- Vital Sign (unequal blood pressures in the extremi,es)
- Auscultate the lungs and the heart (Subclavian or aor,c bruit)
è Rou,ne Lab Test:
- CBC – Mild Anemia
- Crea:ne level
- CRP
- ESR – Elevated
- LFTs
è Angiography à
è CT/MRI à wall thickening, narrowing of lumen.
è Chest X-ray à Aor:c dila:on, widened medias:num.

#The presence of three or more of the six criteria was sensi:ve (91%) and specific (98%) for the
diagnosis of Takayasu arteri:s.
1. Onset at age <40 years
2. Limb claudica:on
3. Decreased brachial artery pulse
4. Unequal arm blood pressures (>10 mm Hg)
5. Subclavian or aor:c bruit
6. Angiographic evidence of narrowing or occlusion of the aorta or its primary branches,
or large limb arteri:s

Treatment:
è Glucocor,coid
è In individuals who are refractory to or unable to taper glucocor:coids, METHOTREXATE in
doses up to 25 mg per week has yielded encouraging results.
è Treat Hypertension
 Ahmad Salh Soboh

Granulomatosis with Polyangii*s (WEGENER’S)

à Granulomatosis with polyangii:s (Wegener’s) is a dis:nct clinicopathologic en:ty
characterized by granulomatous vasculi:s of the upper and lower respiratory tracts together
with glomerulonephri:s.

Clinical Features:

Nosebleeds Saddle-nose

Purpura
Strawberry gums
Diagnosis:
è Rou,ne Lab Test:
- CBC -
- Crea:ne level
- CRP
- ESR
- LFTs
è Specific An,bodies
First Step:
- ANCA +
Second Step:
Skip blo|ng
- PR3-ANCA (An,proteinase-3 an,bodies) or C-ANCA staining – Posi,ve
- MPO-ANCA or P-ANCA – Nega,ve
è Urinalysis:

è To confirm the diagnosis three of the following six features should be present:
1. Granulomatous inflamma:on by biopsy
2. Abnormal urynalisis
3. Sinus nasal inflamma:on
4. Subglo|c, tracheal or endobronchial stenosis
5. Abnormal chest x ray or CT
6. PR3 anca or C-anca staining

Treatment:
è Treat with Glucocor,coid (prednisone) plus cyclophosphamide or rituximab.
è Methotrexate (avoid with eGFR)
 Ahmad Salh Soboh

EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURG-STRAUSS)

à It is characterized by asthma, peripheral and :ssue eosinophilia, extravascular granuloma
forma:on, and vasculi:s of mul:ple organ systems.

Clinical Features:
Necro:zing granulomatous inflamma:on with eosinophils involving mul:ple organs, especially
lungs and heart.
è The syndrome has 3 phases, which may overlap:
1. This phase may persist for years. Pa:ents have allergic rhini:s, nasal polyposis, asthma,
or a combina:on.
2. Peripheral blood and :ssue eosinophilia is typical.
- Clinical presenta:on, which may resemble Loffler's syndrome, includes chronic
eosinophilic pneumonia and eosinophilic gastroenteri:s.
3. Poten:ally life-threatening vasculi:s develops.
- Systemic symptoms (eg, fever, malaise, weight loss, fa:gue) are common.

Diagnosis:
#
è Rou,ne Lab Test:
- CBC – Eosinophilia, elevated serum IgE levels.
- Crea:ne level
- CRP
- ESR
- LFTs
è Specific An,bodies
First Step:
- ANCA +
Second Step:
- PR3-ANCA (An,proteinase-3 an,bodies) or C-ANCA staining – Nega,ve
- MPO-ANCA or P-ANCA – Posi,ve
è Biopsy
- Diagnosis is made by biopsy of lung or skin :ssue (prominence of eosinophils)
- Biopsy is the most accurate test.

Treatment:
è Treat with Glucocor,coid (prednisone) plus cyclophosphamide or rituximab.
è Methotrexate (avoid with eGFR)
 Ahmad Salh Soboh

Behçet’s Syndrome
à Behçet disease is a disease of young people and males are more commonly affected than female.
à Infec:on-like symptoms…

Clinical Features:
è Painful oral ulcers
è Ocular inflamma:on
è Genital ulcers
è Skin lesions – Erythema nodosum (in women), palpable purpura
è Joint pain

Diagnosis:
è Rou,ne Lab Test:
- CBC
- Crea:ne level
- CRP
- ESR
- LFTs
è Specific Lab Tests:
- Immunoglobin D Test (IgD) – Posi:ve
è Specific An,bodies
- HLA-B51
è Posi,ve pathergy test with no other clinical explana:on
è Biopsies of mucocutaneous lesions and gastrointes:nal ulcers reveal a neutrophilic
vascular reac,on.
è Criteria to diagnosis Behçet’s Disease:

Treatment:
è Topical glucocor,coids
- Triamcinolone acetonide à Oral ulcers
- Betamethasone ointment à Genital ulcers
- Betamethasone drops à Anterior uvei:s, re:nal vasculi:s
- Dexamethasone à Re:nal vasculi:s
è Colchicine à Oral ulcers, genital ulcers, pseudofolliculi:s, erythema nodosum, anterior
uvei:s, re:nal vasculi:s (Used as Alterna:ve Therapy for Arthri:s)
è Biologic treatment when pa:ent with eye problems
 Ahmad Salh Soboh

Inflammatory Myopathies

à The clinically suspected diagnosis of PM, DM, IBM, or necro:zing myosi:s is confirmed by
analysis of serum muscle enzymes, EMG findings, and muscle biopsy

Polymyosi(s
à CHANGES IN THE PROXIMAL MUSCLES

Clinical Features:
è Proximal muscle weakness, affec:ng upper arms, shoulders, thighs, hips, and neck.
è Symmetrical muscle weakness.
è Arthri:s
è Muscle pain (myalgia)
è Difficulty swallowing (dysphagia)
è Fa:gue (can be extreme and life-altering)
è Low-grade fever
è Labored breathing (dyspnea)
è A hoarse or changing voice when speaking (dysphonia)
è Difficulty maintaining enough air to finish a sentence
è Difficulty turning in bed, ge|ng up from a seated posi:on, and raising arms over the head

Diagnosis:
#Clinical presenta:on
è Rou,ne Lab Test:
- CBC
- Crea:ne level
- CRP
- ESR
- LFTs (AST and ALT)
è Specific Lab Test:
- Crea:ne kinase (CK) à The CK is always elevated in pa:ents with ac:ve PM.
- Aldolase à it is always elevated in pa:ents with ac:ve PM.
- Lactate dehydrogenase à may elevated.
- Serum Gamma Glutamyl Transferase (GGT) à To rule out liver disease.
è Specific An,bodies
- An:-Jo-1 à Pa:ents with inflammatory myopathies, suppor:ng a possible autoimmune origin.
#ANA is frequently posi,ve, but nonspecific
è Muscle biopsy à Endomysial inflamma:on
è Electromyography à Myogenic papern

Treatment:
è Glucocor,coids - Ini:ally pulse than taper.
è DMARDs: MTX, Azathioprine, cyclophosphamide
è Physical therapy
 Ahmad Salh Soboh

Dermatomyosi*s
à CHANGES IN THE SKIN AND PROXIMAL MUSCLES
à Dermatomyosi:s is a rare disease that causes muscle inflamma:on and skin rash.

Clinical Features:
DM is a dis:nc:ve en:ty iden:fied by a characteris:c rash accompanying, or more oten
preceding, muscle weakness.
è Heliotrope rash (buqerfly)— blue-purple discolora:on
around eyes, upper eyelids with edema, bridge of nose,
and cheeks.
è Goqron papules—papular, erythematous, scaly lesions
over the knuckles (MCP, PIP, DIP.)
è V sign— rash on the face, neck, and anterior chest.
è Shawl sign—rash on shoulders and upper back,
elbows, and knees.
#may worsen auer sun exposure.
è Weakness can be mild, moderate, or severe enough to lead to quadriparesis.
è DM associated with malignancy ouen remits once the tumor is removed.
è DM usually occurs alone but may overlap with scleroderma and mixed connec:ve :ssue disease.

Diagnosis:
è Rou,ne Lab Test: Ask about:
- CBC è History of malignancy.
- Crea:ne level n Tell the pa)ent to do general check-up.
- CRP & ESR è What type of skin lesions are present?
è Ask about lungs ausculta)on?
- LFTs (AST and ALT)
è Specific Lab Test:
- Crea:ne kinase (CK) à Elevated.
- Aldolase à Elevated.
- Lactate dehydrogenase à Elevated.
- Serum Gamma Glutamyl Transferase (GGT) à To rule out liver disease.
è Specific An,bodies
- An:-Jo-1 à Pa:ents with inflammatory myopathies, suppor:ng a possible autoimmune origin.
è Specific An,bodies for DM
- An:-MI-2
- An:-MDA5
#ANA is frequently posi,ve, but nonspecific
è Muscle biopsy à perivascular and perimysial inflamma:on, or in the interfascicular septae.
è Electromyography à Myopathic or Myogenic papern
è Physical Examina,on à Lung ausculta:on to rule out lung fibrosis.
è Lung CT à to rule out Lung cancer (Paraneoplas:c syndromes)

Treatment:
è Glucocor,coids - Ini:ally pulse than taper.
è DMARDs: MTX, Azathioprine, cyclophosphamide
è Physical therapy
#If pa:ent has oncological diseases referral to oncologist.
 Ahmad Salh Soboh

Inclusion body Myosi*s

à CHANGES IN THE DISTAL MUSCLES
à In men pa:ents ≥50 years of age
à 20% of IBM is associated with systemic autoimmune or connec:ve :ssue diseases.
à Familial aggrega:on of typical IBM may occur; such cases have been designated as familial
inflammatory IBM.

Clinical Features:
è Weakness and atrophy of the distal muscles:
- Foot extensors
- Deep finger flexors.
- Small muscles of the hands, especially finger flexors
è Some pa:ents present with falls because their knees collapse due to early quadriceps weakness.
è Asymmetric and selec:vely involve the quadriceps, iliopsoas, triceps, biceps, and finger
flexors, resembling a lower motor neuron disease.
è Dysphagia is common
è Pa:ents can also have loss of deep tendon reflexes.

Diagnosis:
è Rou,ne Lab Test:
- CBC
- Crea:ne level
- CRP
- ESR
- LFTs (AST and ALT)
è Specific Lab Test:
- Crea:ne kinase (CK) à Slightly elevated or low
- Aldolase
- Lactate dehydrogenase (LDH)
- Serum Gamma Glutamyl Transferase (GGT) à To rule out liver disease.
è Muscle biopsy à Inclusion Bodies and Vacuoles
è Electromyography à Myopathic with mixed poten:als

Treatment:
è Responds poorly to therapy
è Start Rehibi:on and Physical Therapy.
 Ahmad Salh Soboh

PM or DM IBM

High Sensi9vity High Specificity NOTES

1. Osteoarthri9s Ultrasound
An7-CCP if Nega7ve:
CRP & ESR
2. Rheumatoid Arthri9s An7-MCV & CBC – Anemia
RF
Ant-CAR-P
Cardiac Problems (First-degree AV block,
Spondylarthri9s
Aor7c Regurgita7on)
MRI – Sacroilii9s
3. Ankylosing Spondyli9s HLA-B27 CRP & ESR X-ray Posi7ve for Sacroilii9s
X-ray Posi7ve for Sacroilii9s
MRI – Sacroilii9s
4. Axial Spondylarthri9s HLA-B27 CRP & ESR X-ray Nega7ve for Sacroilii9s
X-ray Nega7ve for Sacroilii9s
5. Psoria9c Arthri9s HLA-B27 Skin lesion
6. Enteropathic Arthri9s HLA-B27 Crohn Disease or Ulcera7ve Coli7s
Gastrointes7nal or Genitourinary tract
7. Reac9ve Arthri9s HLA-B27
infec7on.
8. Systemic Lupus Erythematous (SLE) ANA ANA An7-dsDNA, an7-Sm
1. An7-beta 2 glycoprotein I (An7-B2GPI)
ANA – HLA-
9. An9phospholipid Syndrome 2. An7-cardiolipin an7body 2 from 3 is APS
DR7
3. An7-Lupus an7coagulant
10. Systemic Sclerosis (Scleroderma) ANA ANA (>85%) an7-Scl-70
An7-Ro/SS-A
11. Sjögren’s Syndrome ANA Ulcers
(75%)
12. Wegener’s Granulomatosis ANCA ANCA+ PR3-ANCA or c-ANCA Strawberry gums
MPO-ANCA or p-ANCA
13. Churg-Strauss ANCA ANCA+ History of Asthma
Biopsy - Eosinophilia
14. Giant Cell Arteri9s ESR Biopsy New onset of headache >50
15. Takayasu Arteri9s ESR <50
16. Behçet’s Syndrome HLA-51
Urine Uric Synovial fluid – Needle shape
17. Gout
Acid crystals
Synovial fluid – rhomboid
18. Pseudogout
shape crystals
Biopsy – gold Weakness proximal muscles
19. Polymyosi9s An7-Jo-1
standard Biopsy: Endomysial no vacuoles
Biopsy – gold Weakness proximal muscles + Skin lesion
20. Dermatomyosi9s An7-Jo-1 An7-Mi-2, An7-MDA5
standard Biopsy: perivascular and perimysial
Biopsy – gold Weakness distal muscles + Age>55
21. Inclusion Body Myosi9s
standard Biopsy: Vacuoles
 Ahmad Salh Soboh

Joints Pains:
à All of them

Skin Lesions:
1. Psoria:c Arthri:s
2. Systemic Lupus Erythematous (SLE)
3. Systemic Sclerosis (Scleroderma)
4. Dermatomyosi:s

Oral ulcers:
1. Systemic Lupus Erythematous (SLE)
2. Sjögren’s Syndrome
3. Wegener’s Granulomatosis
4. Behçet’s Syndrome

Eye problems:
1. Sjögren’s Syndrome (Xerophthalmia - Ocular dryness)
2. Behçet’s Syndrome (Conjunc:vi:s - Ocular inflamma:on)
3. Giant Cell Arteri:s (Visual problems – Blindness most common)

Secondary Diseases:
1. Secondary Systemic Lupus Erythematous (SLE) à to Rheumatoid Arthri:s
2. Secondary Sjögren’s Syndrome à to Rheumatoid Arthri:s
3. Secondary An:phospholipid Syndrome à to SLE
4. Secondary Sjögren’s Syndrome à to SLE
5. Secondary Sjögren’s Syndrome à to Systemic Sclerosis (Scleroderma)
6. Secondary Axial spondyli:s à to Gout