Vasculitis Paper - Shanley

Question 1

Give the diagnosis: Necrotizing arteritis of medium or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries, or venules, and not associated with ANCA

Answer 1

Polyarteritis nodosa Medium artery necrotizing arteritis seen on biopsy, negative ANCA, no MCLNS, and no evidence of glomerulonephritis

Question 2

Name the two medium vessel vasculitides.

Answer 2

Polyarteritis nodosa Kawasaki disease

Question 3

Name the two large vessel vasculitides.

Answer 3

Giant cell arteritis Takayasu arteritis

Question 4

Microscopic polyangiitis (MPA) Granulomatosis with polyangiitis (Wegener’s) (GPA) Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) These all make up what kind of vasculitides?

Answer 4

Small vessel vasculitis Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV)

Question 5

Anti–glomerular basement membrane (anti-GBM) disease Cryoglobulinemic vasculitis (CV) IgA vasculitis (Henoch-Scho ̈nlein) (IgAV) Hypocomplementemic urticarial vasculitis (HUV) (anti-C1q vasculitis) These all make up what kind of vasculitides?

Answer 5

Small vessel vasculitis Immune complex SVV

Question 6

Name the diagnosis: Arteritis, often granulomatous and usually affecting the aorta and/or its major branches, with a predilection for the branches of the carotid and vertebral arteries. Giant cells are frequently but not always observed in biopsy specimens

Answer 6

Giant cell arteritis (temporal arteritis if temporal arteries are involved)

Question 7

Name the diagnosis: Arteritis, often granulomatous, predominantly affecting the aorta and/or its major branches. Onset usually occurs before the age of 50 years

Answer 7

Takayasu arteritis Onset usually occurs before the age of 50 years, which is a major distinction from giant cell arteritis, whose onset usually occurs after age 50.

Question 8

Name the diagnosis Arteritis associated with the mucocutaneous lymph node syndrome and predominantly affecting medium and small arteries. Coronary arteries are often involved. Aorta and large arteries may be involved. Usually occurs in infants and young children.

Answer 8

Kawasaki disease

Question 9

Name the diagnosis: Necrotizing vasculitis, with few or no immune deposits, predominantly affecting small vessels (i.e., capillaries, venules, arterioles, and small arteries), associated with myeloperoxidase (MPO) ANCA or proteinase 3 (PR3) ANCA. Not all patients have ANCA. Add a prefix indicating ANCA reactivity, e.g., MPO-ANCA, PR3-ANCA, ANCA- negative.

Answer 9

ANCA-associated vasculitis (AAV)

Question 10

Name the diagnosis: Necrotizing vasculitis, with few or no immune deposits, predominantly affecting small vessels (i.e., capillaries, venules, or arterioles). Necrotizing arteritis involving small and medium arteries may be present. Necrotizing glomerulonephritis is very common. Pulmonary capillaritis often occurs. Granulomatous inflammation is absent.

Answer 10

Microscopic polyangiitis

Question 11

Name the diagnosis: Necrotizing granulomatous inflammation usually involving the upper and lower respiratory tract, and necrotizing vasculitis affecting predominantly small to medium vessels (e.g., capillaries, venules, arterioles, arteries and veins). Necrotizing glomerulonephritis is common.

Answer 11

Granulomatosis with polyangiitis (Wegener’s) (GPA)

Question 12

Name the diagnosis: Eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium vessels, and associated with asthma and eosinophilia. ANCA is more frequent when glomerulonephritis is present.

Answer 12

Eosinophilic granulomatosis with polyangiitis (Churg- Strauss) (EGPA)

Question 13

Name the diagnosis: Vasculitis with moderate to marked vessel wall deposits of immunoglobulin and/or complement components predominantly affecting small vessels (i.e., capillaries, venules, arterioles, and small arteries). Glomerulonephritis is frequent.

Answer 13

Immune complex vasculitis

Question 14

Name the diagnosis: Vasculitis affecting glomerular capillaries, pulmonary capillaries, or both, with GBM deposition of anti-GBM autoantibodies. Lung involvement causes pulmonary hemorrhage, and renal involvement causes glomerulonephritis with necrosis and crescents.

Answer 14

Anti–glomerular basement membrane (anti-GBM) disease

Question 15

Name the diagnosis: Vasculitis with cryoglobulin immune deposits affecting small vessels (predominantly capillaries, venules, or arterioles) and associated with serum cryoglobulins. Skin, glomeruli, and peri- pheral nerves are often involved.

Answer 15

Cryoglobulinemic vasculitis (CV)

Question 16

Name the diagnosis: Vasculitis, with IgA1-dominant immune deposits, affecting small vessels (predominantly capillaries, venules, or arterioles). Often involves skin and gastrointestinal tract, and frequently causes arthritis. Glomerulonephritis indistinguishable from IgA nephropathy may occur.

Answer 16

IgA vasculitis (Henoch-Scho ̈nlein) (IgAV)

Question 17

Name the diagnosis: Vasculitis accompanied by urticaria and hypocomplementemia affecting small vessels (i.e., capillaries, venules, or arterioles), and associated with anti-C1q antibodies. Glomerulonephritis, arthritis, obstructive pulmonary disease, and ocular inflammation are common.

Answer 17

Hypocomplementemic urticarial vasculitis (HUV) (anti-C1q vasculitis)

Question 18

Name the diagnosis: Vasculitis that can affect arteries or veins. Characterized by recurrent oral and/or genital aphthous ulcers accompanied by cutaneous, ocular, articular, gastrointestinal, and/or central nervous system inflammatory lesions. Small vessel vasculitis, thromboangiitis, thrombosis, arteritis, and arterial aneurysms may occur.

Answer 18

Behcet disease

Question 19

Name the diagnosis: Vasculitis characterized by ocular inflammatory lesions, including interstitial keratitis, uveitis, and episcleritis, and inner ear disease, including sensorineural hearing loss and vestibular dysfunction. Vasculitic manifestations may include arteritis (affecting small, medium, or large arteries), aortitis, aortic aneurysms, and aortic and mitral valvulitis.

Answer 19

Cogan’s syndrome