Vasculitis

GENERAL PRINCIPLES

• Vasculitis is characterized by inflammation of blood vessels, leading to tissue damage and necrosis. This diagnosis includes a broad spectrum of disorders with various causes that involve vessels of different types, sizes, and locations.

The immunopathologic process may involve immune complexes.

• Although in most cases the inciting agent has not been identified, some are associated with chronic hepatitis B and C.

• Vasculitis “mimics” should be considered, including bacterial endocarditis, HIV infection, atrial myxoma, paraneoplastic syndromes, cholesterol emboli, and cocaine and amphetamine use.

• Vasculitides are classified by vessel sizes (Table 25-7).

TABLE 25-7

VASCULITIS CLASSIFICATION

Vessel Size	Types of Vessel	Types of Vasculitis
Large vessel	Aorta and main branches	• Takayasu arteritis • Giant cell arteritis
Medium vessel	Main visceral arteries and veins and their initial branches	• Polyarteritis nodosa • Kawasaki disease
Small vessel	Arterioles, venules, and capillaries	ANCA-associated vasculitis: • Granulomatosis with polyangiitis • Eosinophilic granulomatosis with polyangiitis • Microscopic polyangiitis
		Immune complex mediated: • Cryoglobulinemic vasculitis • Henoch-Schonlein purpura (HSP) • Hypocomplementemic urticarial vasculitis
Variable vessel	Can involve any vessel size	• Behcet disease • Cogan syndrome

• Vasculitis should be suspected whenever a patient with systemic symptoms has organ dysfunction. Symptoms are associated with the size of the affected vessel (Table 25-8).

TABLE 25-8

VASCULITIS FEATURES BY VESSEL SIZE

Organ System	Small Vessel	Medium Vessel	Large Vessel
Dermatologic	Palpable purpura	Livedo reticularis	Cyanosis
Renal	Hematuria with RBC casts, proteinuria	Hematuria, flank pain	Hypertension
Gastrointestinal	GI bleeding	Bowel perforation	Bowel infarction
Neurologic	Polyneuropathy	Polyneuropathy, strokes	Strokes

• We will review some of the most common vasculitis in the following sections.

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Source: Ancha S., Auberle C., Cash D., Harsh M., Hickman J., Kounga C.. The Washington Manual of Medical Therapeutics, 37th edition, LWW, 2022. —1250p.. 1250

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