PATH - Vasculitides

Question 1

Giant cell (temporal) arteritis

Answer 1

Large-vessel vasculitis

Most commonly affects branches of carotid artery

Usually elderly females.

Unilateral headache (temporal artery), jaw claudication.

May lead to irreversible blindness due to ophthalmic artery occlusion.

Focal granulomatous inflammation
INC ESR

Associated with polymyalgia rheumatica

Question 2

Takayasu arteritis

Answer 2

Large-vessel vasculitis

Granulomatous thickening and narrowing of aortic arch and proximal great vessels

Usually Asian females

Question 3

Polyarteritis nodosa

Answer 3

Medium-vessel vasculitis

Typically involves renal and visceral vessels, not pulmonary arteries.

Immune complex mediated.

Transmural inflammation of the arterial wall with fibrinoid necrosis

Innumerable renal microaneurysms and spasms
on arteriogram

Fever, weight loss, malaise, headache.
GI: abdominal pain, melena.
Hypertension, neurologic dysfunction,
cutaneous eruptions, renal damage

Question 4

Kawasaki disease (mucocutaneous lymph node syndrome)

Answer 4

Medium-vessel vasculitis

Asian children

Question 5

Buerger disease (thromboangiitis obliterans)

Answer 5

Medium-vessel vasculitis

Segmental thrombosing vasculitis.

Heavy smokers, males

Question 6

Granulomatosis with polyangiitis

Wegener

Answer 6

Small-vessel vasculitis

-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
-Necrotizing granulomas in the lung and
upper airway
ƒ-Necrotizing glomerulonephritis

*PR3-ANCA/c-ANCA (anti-proteinase 3).

CXR: large nodular densities.

Question 7

Microscopic polyangiitis

Answer 7

Small-vessel vasculitis

Necrotizing vasculitis commonly involving lung, kidneys, and skin with pauci-immune glomerulonephritis and palpable purpura

*MPO-ANCA/p-ANCA (anti-myeloperoxidase)

No granulomas

Question 8

Eosinophilic granulomatosis with

polyangiitis (Churg-Strauss)

Answer 8

Small-vessel vasculitis

Granulomatous, necrotizing vasculitis with eosinophilia

Asthma, sinusitis, skin nodules or purpura, peripheral neuropathy (eg, wrist/foot drop).

Can also involve heart, GI, kidneys (pauciimmune
glomerulonephritis).

*MPO-ANCA/p-ANCA,  IgE level.

Question 9

Henoch-Schönlein purpura

Answer 9

Small-vessel vasculitis

Most common childhood systemic vasculitis

Vasculitis 2° to IgA immune complex deposition

Often follows URI.

Classic triad:
ƒ-Skin: palpable purpura on buttocks/legs
-Arthralgias
-GI: abdominal pain

Associated with IgA nephropathy (Berger
disease)