Vasculitis

Question 1

General causes of vasculitis

Answer 1

Immune mediated inflammation
Direct invasion of vascular wall by infectious pathogens
Physical and chemical injury

Question 2

Types of large vasculitides

Answer 2

Giant cell arteritis
Takayasu arteritis

Question 3

Types of medium vasculitis

Answer 3

Polyarteritis nodosa
Kawasaki disease/mucocutaneous lymph node syndrome

Question 4

Types of small vasculitides

Answer 4

Microscopic polyangiitis
Granulomatosis with polyangiitis/Wegner’s granulomatosis
Allergic granulomatosis with polyangiitis/Churg Strauss Syndrome
IgA vasculitis/Henock Schonlein purpura
Mixed cryoglobulinemia

Question 5

Diagnostic marker of granulomatosis with polyangiitis (Wegner’s granulomatosis)

Answer 5

c-ANCA/PR3-ANCA

Question 6

Diagnostic marker of microscopic polyangiitis or allergic granulomatosis with polyangiitis (Churg Strauss syndrome)

Answer 6

p-ANCA/MPO-ANCA

Question 7

Main arteries affected in giant cell/temporal arteritis

Answer 7

Temporal
Vertebral
Ophthalmic

Question 8

Vasculitis characterized by medial granulomatous inflammation centered on the internal elastic lamina, which produces elastic fragmentation

Answer 8

Giant cell/temporal arteritis

Question 9

Vasculitis commonly associated with polymyalgia rheumatica

Answer 9

Giant cell/temporal arteritis

Question 10

Granulomatous vasculitis of medium and large arteries that commonly involves the aortic arch

Answer 10

Takayasu arteritis

Question 11

Large vessel vasculitis more common in females of Asian descent, under the age of 50.

Answer 11

Takayasu arteritis

Question 12

Vasculitis of small and medium sized muscular arteries that involves renal and visceral vessels, but spares pulmonary circulation

Answer 12

Polyarteritis nodosa

Question 13

Vasculitis associated with chronic hep B in 30% of cases

Answer 13

Polyarteritis nodosa

Question 14

Microscopy shows segmental transmural necrotizing inflammation of small to medium sized arteries, vessels at various stages of acute and chronic inflammation, and fibrinoid necrosis in acute stages

Answer 14

Polyarteritis nodosa

Question 15

Treatment of polyarteritis nodosa

Answer 15

Corticosteroids
Resistant cases –> cyclophosphamide

Question 16

Acute, febrile, self-limited vasculitis in children <4 yo

Answer 16

Kawasaki disease

Question 17

Toddler-aged pt presents with conjunctival and oral erythema, strawberry tongue, blistering and edema of hands and feet, and desquamative rash of palms and soles. Microscopy shows dense transmural inflammatory infiltrate.

Answer 17

Kawasaki disease

Question 18

Increased risk of what is associated with Kawasaki disease

Answer 18

Coronary A aneurysms

Question 19

Treatment for Kawasaki disease

Answer 19

IV immunoglobulin
Aspirin –> reduce risk of coronary A aneurysm

Question 20

Pt presents with palpable purpura, renal disease, and GIT bleeding. They have asthma. Chest x-ray shows lung infiltrates. They have increased serum IgE.

Answer 20

Eosinophilic granulomatosis with angiitis (Churg Strauss)

Question 21

Small vessel necrotizing vasculitis with MPO-ANCAs present in <50% of cases.

Answer 21

Eosinophilic granulomatosis with angiitis (Churg Strauss)

Question 22

Most common vasculitis in children (1-15 yo)

Answer 22

Immunoglobulin A vasculitis (Henoch Schoenlein purpura)

Question 23

Vasculitis associated with HLA-B51

Answer 23

Behcet disease

Question 24

Triad of Behcet disease

Answer 24

Recurrent oral aphthous ulcers
Genital ulcers
Uveitis
(can’t kiss, can’t pee, can’t see)

Question 25

Necrotizing vasculitis that generally affects capillaries where all lesions tend to be the same age. Associated with MPO-ANCA in most cases.

Answer 25

Microscopic polyangiitis

Question 26

Necrotizing glomerulonephritis in 90%. MPO-ANCA positive.

Answer 26

Microscopic polyangiitis

Question 27

Vasculitis characterized by necrotizing granulomas of URT and/or LRT, necrotizing granulomatous vasculitis of small/medium vessels, and focal necrotizing/crescentic glomerulonephritis.

Answer 27

Granulomatosis with polyangiitis

Question 28

Vasculitis that is a form of T cell mediated hypersensitivity. Associated with PR3-ANCA in 95% of cases.

Answer 28

Granulomatosis with polyangiitis

Question 29

Microscopy of lungs show granulomas with geographic patterns of central necrosis and accompanying vasculitis. Granulomas coalesce, forming cavitary nodules. CXR shows large nodular densities

Answer 29

Granulomatosis with polyangiitis

Question 30

Pt presents with chronic sinusitis, hemoptysis, dyspnea, and hematuria. They have a nasal septum perforation on exam. They are positive for PR3-ANCA.

Answer 30

Granulomatosis with polyangiitis

Question 31

Segmental, thrombosing, acute and chronic inflammation of medium sized and small arteries, extending into contiguous veins and nerves. Exclusively seen in male heavy cigarette smokers, <40 yo.

Answer 31

Thromboangiitis obliterans (Buerger disease)

Question 32

Vasculitis associated with hepatitis due to mixed IgG and IgA complex deposition. Characteristic triad of palpable purpura, weakness, and arthralgias.

Answer 32

Mixed cryoglobulinemia

Question 33

Causes of secondary Raynaud phenomenon

Answer 33

SLE Scleroderma Buerger disease Atherosclerosis