rheumatology & immunology

Question 1

What disease is associated with giant cell arteritis?

Answer 1

polymyalgia rheumatica (PMR): 40–50% of patients with giant cell arteritis also have PMR

Question 2

Immediate administration of what is crucial in preventing what in GCA

Answer 2

Immediate administration of high-dose glucocorticoids is crucial to prevent permanent vision loss in patients with giant cell arteritis!

Question 3

Large vessel vasculitis

Answer 3

Giant cell arteritis (TA)

Takayasu arteritis

Question 4

What is takayasu arteritis

Answer 4

granulomatous inflammation of the aorta and branches

Question 5

Features of Takayasu arteritis

Answer 5

Typically affects women aged 30–40. Presents with fever, arthralgia, weight loss, poor peripheral pulses (decrease bilateral brachial and radial pulses) limb ischemia, hypertension, and/or arterial bruits.

Question 6

Medium-sized vessel vasculitis

Answer 6

Kawasaki disease
Polyarteritis nodosa
Thromboangiitis obliterans

Question 7

Polyarteritis nodosa

Answer 7

often associated with Hep B or C infection
tissue ischemia affecting skin, muscles joints kidneys
lungs are not involved

Question 8

young adult presenting with stroke or MI what rheum disorder should be considered

Answer 8

Polyarteritis nodosa

Question 9

Thromboangiitis obliterans

Answer 9

Smoking most important risk factor
young men
located in distal arteries of Upper and lower extremities
has normal ESR and CRP

Question 10

young man with intermittent claudication, Raynaud’s phenomenon, and migratory thrombophlebitis has what

Answer 10

Thromboangiitis obliterans

Question 11

Imaging of thromboangiitis obliterans shows what?

Answer 11

arteriography shows segmental lesions that occlude distal vessels of extremities with corkscrew-shaped collateral vessels around the site of occlusion

Question 12

Wegener granulomatosis

Answer 12

C’ disease: Curvy nose (saddle-nose deformity), Chronic sinusitis, Cough, Conjunctivitis and Corneal ulceration, Cardiac arrhythmias, nonCaseating granulomas on biopsy, cANCA, Corticosteroids and Cyclophosphamide as treatment.

Question 13

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Answer 13

pANCA and polyneuropathy (foot or wrist drop), allergic rhinitis/sinusitis/asthma, vasculitis, eosinophilia, and skin nodules

Question 14

Microscopic polyangiitis

Answer 14

pauci-immune glomerulonephritis; hypertension
pulmonary vasculitis with hemoptysis
palpable purpura
spares nasopharynx and no granulomas

Question 15

ANCA-associated small vessel vasculitis

Answer 15

Granulomatosis with polyangiits–> cANCA
Eosinophilic granulomatosis with polyangiitis–>pANCA
Microscopic polyangiitis–> pANCA

Question 16

Difference between microscopic polyangiitis adn Wegner’s

Answer 16

presentation of microscopic polyangiitis is similar to that of Wegener granulomatosis, but it does not affect vessels in the upper and lower respiratory tract (no sinusitis or rhinitis), does not involve granuloma formation, and is associated with pANCA, not cANCA.

Question 17

Microscopic polyangiits has what positive

Answer 17

has MyeloPeroxidase antibodies (i.e., p-ANCA).

Question 18

Non-ANCA-associated vasculitis of small vessels

Answer 18

Henoch-Schonlein purpura
Cryoglobulinemic vasculitis
Behcet disease

Question 19

Immunoglobulin A vasculitis/Henoch-Schonlein purpura typically presents how?

Answer 19

It is often preceded by an upper respiratory tract infection and typically presents with a tetrad of symptoms: palpable purpura, arthritis/arthralgia, abdominal pain, and renal disease.

Question 20

why is HSP different from most purpura

Answer 20

platelet count in HSP is normal or elevated, as opposed to other causes of purpura!

Question 21

Cryoglobulinemic vasculitis

Answer 21

characterized by temperature-dependent deposition of immune complexes in blood vessel walls and subsequent inflammation of involved vessels and surrounding tissue

Question 22

What is is cryoglobulienmia highly associated with?

Answer 22

HCV

Question 23

Patient with fatigue, palpable purpura with increase RF and hx of Hep c disease has what?

Answer 23

Cryoglobulinemic vasculitis

Question 24

Behcet disease associated with

Answer 24

HLA-B51

in 20-40 yos

Question 25

Behcet mnemonic=

PATHERGY

Answer 25

Positive pathergy test,
Apthous mouth ulcers
Thrombosis 
Hemoptysis (pulmonary artery aneurysm)
Eye lesions (uveitis)
Genital ulcers
Young at presentation

Question 26

65 yo female presents with new blurry vision, HA, and tender temporal artery with bilateral pain in shoulders
what does she ahve

Answer 26

Giant cell arteritis

Question 27

Labs of Giant cell arteritis

Answer 27

elevated ESR
Temporal artery biopsy (gold standard) shows granulomatous inflammation with giant cells and intima proliferation that results in stenosis.

Question 28

35 yo female presents with different blood pressure in two arms, abdominal bruit
with syncope and angina
what does she have and how to treat

Answer 28

Takayasu arteritis

tx with glucocorticoids

Question 29

How to treat most vasculitis

Answer 29

Corticosteroids