Vasculitides

Rapid Review·Cardiovascular

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PATHOLOGY

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Inflammatory destruction of vessels, organized by the size of the vessel involved.

Key takeaways

The organizing principle

Vessel size predicts the clinical syndrome

Large: pulse and perfusion problems of major branches.
Medium: organ infarcts and aneurysms.
Small: purpura, glomerulonephritis, and alveolar hemorrhage.

Large vessels

Giant cell (temporal) arteritis

females over 50. Unilateral headache, jaw claudication, temporal artery tenderness, and a risk of blindness from anterior ischemic optic neuropathy. Granulomatous inflammation of temporal, vertebral, and ophthalmic arteries. Associated with polymyalgia rheumatica. Raised ESR and CRP; diagnosed on temporal artery biopsy. Give high-dose glucocorticoids BEFORE the biopsy.

Takayasu arteritis

Asian females under 40. "Pulseless disease" with weak upper extremity pulses, plus fever, night sweats, arthritis, myalgias. Granulomatous thickening and narrowing of the aortic arch and proximal great vessels. Raised ESR. Treat with glucocorticoids.

Large-vessel vasculitis
Feature	Giant cell (temporal) arteritis	Takayasu arteritis
Demographics	Females over 50	Asian females under 40
Vessels	Temporal, vertebral, ophthalmic	Aortic arch and proximal great vessels
Signature	Jaw claudication, unilateral headache, blindness risk	"Pulseless disease", weak upper limb pulses
Pathology	Granulomatous	Granulomatous thickening
Association	Polymyalgia rheumatica	Fever, night sweats
Labs	Raised ESR and CRP	Raised ESR
Treatment	Steroids BEFORE biopsy	Glucocorticoids

Medium vessels

Kawasaki disease

Asian children under 4. By "CRASH and burn":

Conjunctivitis, bilateral and nonexudative
Rash, polymorphous and desquamating
Adenopathy, cervical
Strawberry tongue
Hand and foot changes: edema and erythema
and burn = fever for 5 days or more
Complication: coronary artery aneurysms, where thrombosis or rupture can be fatal. Treat with IVIG and aspirin, one of the few paediatric indications for aspirin.

Polyarteritis nodosa

middle-aged males, 30% hepatitis B seropositive. Fever, weight loss, abdominal pain, melena, hypertension, neurologic dysfunction, cutaneous eruptions, renal damage.

Involves renal and visceral vessels and SPARES the lungs, which is the key negative.
Transmural inflammation with fibrinoid necrosis, and "string of pearls" microaneurysms on arteriogram.
"PAN affects the SKIN": Skin, Kidneys, Intestines, Nerves.
Treat with glucocorticoids and cyclophosphamide.

Buerger disease (thromboangiitis obliterans)

heavy tobacco smoking, males under 40. Intermittent claudication, gangrene, Raynaud phenomenon, autoamputation of digits, superficial nodular phlebitis. Segmental thrombosing vasculitis involving veins and nerves as well as arteries. Treatment is smoking cessation, and nothing else works.

Medium-vessel vasculitis
Feature	Kawasaki disease	Polyarteritis nodosa	Buerger disease
Demographics	Asian children under 4	Middle-aged males	Male smokers under 40
Association	None	Hepatitis B, 30%	Heavy tobacco
Signature	CRASH and burn	"String of pearls"; SPARES the lungs	Autoamputation of digits, Raynaud
Pathology	Coronary arteritis	Transmural inflammation with fibrinoid necrosis	Segmental thrombosing, veins and nerves too
Complication	Coronary artery aneurysms	Renal, GI and neurologic damage	Gangrene
Treatment	IVIG plus aspirin	Glucocorticoids, cyclophosphamide	Smoking cessation

Small vessels, ANCA-associated

Granulomatosis with polyangiitis

c-ANCA (PR3-ANCA).

Upper respiratory: nasal septum perforation (saddle nose), chronic sinusitis, otitis media, mastoiditis.
Lower respiratory: hemoptysis, dyspnea, infiltrates.
Renal: pauci-immune rapidly progressive glomerulonephritis.
Skin: palpable purpura.
Treat with glucocorticoids plus rituximab or cyclophosphamide.

Microscopic polyangiitis

p-ANCA (MPO-ANCA). Necrotizing vasculitis of lungs, kidneys, and skin, with pauci-immune glomerulonephritis and palpable purpura. No nasopharyngeal involvement, which is the distinction from GPA. Treat with cyclophosphamide and glucocorticoids.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

asthma, eosinophilia, and systemic vasculitis. Granulomatous necrotizing vasculitis with eosinophils and raised IgE. Eosinophilic infiltration causes peripheral neuropathy.

The ANCA-associated small-vessel vasculitides
Feature	Granulomatosis with polyangiitis	Microscopic polyangiitis	Eosinophilic GPA (Churg-Strauss)
ANCA	c-ANCA (PR3)	p-ANCA (MPO)	p-ANCA
Upper respiratory	YES, saddle nose and sinusitis	NO, the key distinction	Sinusitis common
Lower respiratory	Hemoptysis, infiltrates	Yes	Asthma
Renal	Pauci-immune RPGN	Pauci-immune GN	Yes
Signature lab	None specific	None specific	Eosinophilia, raised IgE
Granulomas	Yes	No	Yes, necrotizing with eosinophils

Small vessels, immune complex

IgA vasculitis (Henoch-Schönlein purpura)

the most common childhood vasculitis, often following a URI. Classic triad of hinge pain (arthralgias), stomach pain (sometimes with intussusception), and palpable purpura on the buttocks and legs. Associated with IgA nephropathy. Treat supportively, with glucocorticoids if needed.

Mixed cryoglobulinemia

often hepatitis C. Triad of palpable purpura, weakness, and arthralgias. Cryoglobulins precipitate in the cold, from mixed IgG and IgM immune complex deposition.

Hypocomplementemic urticarial vasculitis (anti-C1q)

often SLE. Urticaria, purpuric rash, arthralgias, with low C1q and raised anti-C1q antibodies.

Immune-complex small-vessel vasculitides
Disease	Trigger or association	Signature
IgA vasculitis (HSP)	Childhood, follows a URI	Hinge pain, stomach pain, palpable purpura on buttocks and legs; IgA nephropathy
Mixed cryoglobulinemia	Hepatitis C	Purpura, weakness, arthralgias; precipitates in cold
Hypocomplementemic urticarial	SLE	Low C1q, raised anti-C1q antibodies

A 68-year-old woman has a new unilateral headache and jaw pain when chewing. Biopsy is booked for Thursday. What do you do today?

Start high-dose glucocorticoids now. The biopsy can wait; the blindness cannot.

How it's tested

In giant cell arteritis you give steroids BEFORE the biopsy, because the biopsy can wait but the blindness cannot, and that sequence is the single most tested management point in vasculitis. The GPA versus microscopic polyangiitis distinction comes down to the nasopharynx: c-ANCA with a saddle nose is granulomatosis with polyangiitis, while p-ANCA sparing the upper airway is microscopic polyangiitis. For polyarteritis nodosa, the negative finding carries the question, since it spares the lungs.

Go deeper

First Aid 2026 — CV/Pathology (vasculitides) · B&B — Vasculitis by vessel size · Mehlman — HY Cardio (vasculitides, ANCA associations)

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