Vasculitiseseseses

Question 1

Temporal (Giant Cell) Arteritis

Answer 1

LARGE VESSEL Usually elderly females. Unilateral headache (temporal artery), jaw claudication. May lead to irreversible blindness due to ophthalmic artery occlusion. Associated with polymyalgia rheumatic. Most commonly affects branches of carotid artery. Focal granulomatous in ammation A . elevated ESR (really the only lab finding). Treat with high-dose corticosteroids prior to temporal artery biopsy to prevent blindness.

Question 2

Polyarteritis Nodosa

Answer 2

MEDIUM VESSEL Young adults. Hepatitis B seropositivity in 30% of patients. Fever, weight loss, malaise, headache. GI: abdominal pain, melena. Hypertension, neurologic dysfunction, cutaneous eruptions, renal damage. Typically involves renal and visceral vessels, not pulmonary arteries. Immune complex mediated. Transmural in ammation of the arterial wall with brinoid necrosis. Innumerable renal microaneurysms C and spasms on arteriogram. Treat with corticosteroids, cyclophosphamide.

Question 3

Granulomatosis with Polyangitis (Wegener)

Answer 3

SMALL VESSEL Upper respiratory tract: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis. Lower respiratory tract: hemoptysis, cough, dyspnea. Renal: hematuria, red cell casts. Triad: ƒ Focal necrotizing vasculitis ƒ Necrotizing granulomas in the lung and upper airway ƒ Necrotizing glomerulonephritis PR3-ANCA/c-ANCA G (anti-proteinase 3). CXR: large nodular densities. Treat with cyclophosphamide, corticosteroids.

Question 4

Microscopic Polyangitis

Answer 4

SMALL VESSEL Necrotizing vasculitis commonly involving lung, kidneys, and skin with pauci-immune glomerulonephritis and palpable purpura. Presentation similar to granulomatosis with polyangiitis but without nasopharyngeal involvement. No granulomas. MPO-ANCA/p-ANCA H (anti- myeloperoxidase). Treat with cyclophosphamide, corticosteroids. NO upper airway involvement

Question 5

Eosinophilic Granulomatosis with Polyangiitis (Churg Stauss)

Answer 5

SMALL VESSEL Asthma, sinusitis, skin nodules or purpura, peripheral neuropathy (e.g., wrist/foot drop). Can also involve heart, GI, kidneys (pauci- immune glomerulonephritis). Granulomatous, necrotizing vasculitis with eosinophilia. MPO-ANCA/p-ANCA, IgE level.

Question 6

Henoch-Schonlein Purpura

Answer 6

Most common childhood systemic vasculitis. Often follows URI. Classic triad: ƒ Skin: palpable purpura on buttocks/legs J ƒ Arthralgias ƒ GI: abdominal pain Vasculitis 2° to IgA immune complex deposition. Associated with IgA nephropathy (Berger disease).

Question 7

Takayasu Arteritis

Answer 7

LARGE VESSEL
Usually Asian females < 40 years old. “Pulseless disease” (weak upper extremity
pulses), fever, night sweats, arthritis, myalgias, skin nodules, ocular disturbances.
Granulomatous thickening and narrowing of aortic arch B and proximal great vessels.
ESR.
Treat with corticosteroids.

Question 8

Kawasaki Disease

Answer 8

Asian children < 4 years old. Mucocutaneous lymph node syndrome:

Conjunctival injection, Rash (polymorphous desquamating), Adenopathy (cervical), Strawberry tongue (oral mucositis) D , Hand- foot changes (edema, erythema), fever.

May develop coronary artery aneurysms E ;

thrombosis or rupture can cause death. Treat with IV immunoglobulin and aspirin.

Question 9

Buerger disease (thromboangiitis obliterans)

Answer 9

Heavy smokers, males < 40 years old. Intermittent claudication may lead to

gangrene F , autoamputation of digits,

super cial nodular phlebitis. Raynaud phenomenon is often present.

Segmental thrombosing vasculitis. Treat with smoking cessation.