Cardio - Vasculitides

Question 1

Giant cell (temporal) arteritis: epidemiology & presentation

Answer 1

Usually elderly females. Unilateral headache (temporal artery), jaw claudication. May lead to irreversible blindness due to ophthalmic artery occlusion. Associated with polymyalgia rheumatica.

Question 2

Giant cell (temporal) arteritis: pathology, labs

Answer 2

Most commonly affects branches of carotid artery. Focal granulomatous inflammation A . q ESR. Treat with high-dose corticosteroids prior to temporal artery biopsy to prevent blindness.

Question 3

Takayasu arteritis: Epidemiology/ presentation

Answer 3

Usually Asian females < 40 years old. “Pulseless disease” (weak upper extremity pulses), fever, night sweats, arthritis, myalgias, skin nodules, ocular disturbances.

Question 4

Takayasu arteritis: PATHOLOGY/LABS

Answer 4

Granulomatous thickening and narrowing of aortic arch and proximal great vessels B . high ESR. Treat with corticosteroids

Question 5

Polyarteritis nodosa: presentation

Answer 5

Usually middle-aged men. Hepatitis B seropositivity in 30% of patients. Fever, weight loss, malaise, headache. GI: abdominal pain, melena. Hypertension, neurologic dysfunction, cutaneous eruptions, renal damage.

Question 6

Polyarteritis nodosa: labs

Answer 6

Typically involves renal and visceral vessels, not pulmonary arteries. Transmural inflammation of the arterial wall with fibrinoid necrosis. Different stages of inflammation may coexist in different vessels. Innumerable renal microaneurysms C and spasms on arteriogram. Treat with corticosteroids, cyclophosphamide

Question 7

Kawasaki disease (mucocutaneous lymph node syndrome): presentation

Question 8

Kawasaki disease (mucocutaneous lymph node syndrome): labs

Answer 8

CRASH and burn. May develop coronary artery aneurysms E ; thrombosis or rupture can cause death. Treat with IV immunoglobulin and aspirin.

Question 9

Buerger disease (thromboangiitis obliterans): presentation

Answer 9

Heavy smokers, males < 40 years old. Intermittent claudication may lead to gangrene F , autoamputation of digits, superficial nodular phlebitis. Raynaud phenomenon is often present.

Question 10

Buerger disease: labs

Answer 10

Segmental thrombosing vasculitis. Treat with smoking cessation.

Question 11

Granulomatosis with polyangiitis (Wegener): pres

Answer 11

Upper respiratory tract: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis. Lower respiratory tract: hemoptysis, cough, dyspnea. Renal: hematuria, red cell casts.

Question 12

Granulomatosis with polyangiitis (Wegener): labs

Answer 12

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 13

Microscopic polyangiitis: pres

Answer 13

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.

Question 14

Microscopic polyangiitis:labs

Question 15

Eosinophilic granulomatosis with polyangiitis (ChurgStrauss)” pres

Answer 15

Asthma, sinusitis, skin nodules or purpura, peripheral neuropathy (eg, wrist/foot drop). Can also involve heart, GI, kidneys (pauciimmune glomerulonephritis).

Question 16

Eosinophilic granulomatosis with polyangiitis (ChurgStrauss) labs

Answer 16

Granulomatous, necrotizing vasculitis with eosinophilia I . MPO-ANCA/p-ANCA, q IgE level.

Question 17

Henoch-Schönlein purpura: pres

Answer 17

Most common childhood systemic vasculitis. Often follows URI. Classic triad: ƒ Skin: palpable purpura on buttocks/legs J ƒ Arthralgias ƒ GI: abdominal pain

Question 18

Henoch-Schönlein purpura labs

Answer 18

Vasculitis 2° to IgA immune complex deposition. Associated with IgA nephropathy (Berger disease).