Vasculitis resident survival guide

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dina Elantably, MD, MSc[2]

Synonyms and keywords: Arteritis, Angiitis, Vasculitides (plural)

Overview

Vasculitis is the presence of inflammatory leukocytes in the walls of the blood vessels with reactive damage to mural structures leading to compromise of the lumen with downstream ischemia, necrosis, and bleeding. The exact pathogenesis is unknown, and vasculitis can be primary or secondary to underlying disease. The extent and severity of vasculitides may vary from self-limited cutaneous vasculitis to severe fatal systemic vasculitides.

Causes

Vasculitides are categorised primarily by vessel size, together with aetiology, pathogenesis, pathology, demographics and clinical features

The 2012 Chapel Hill Consensus Conference (CHCC) classified the vasculitides as follows:

Large Vessel Vasculitis
Takayasu arteritis
Giant cell arteritis
Medium vessel vasculitis
Polyarteritis nodosa
Kawasaki disease
Small vessel vasculitis
ANCA-associated vasculitis: Microscopic polyangiitis, Granulomatosis with polyangiitis (Wegener's), and Eosinophilic granulomatosis with polyangiitis (Churg-Strauss).
Immune complex small-vessel vasculitis: Anti-glomerular basement membrane disease, Cryoglobulinemic vasculitis, IgA vasculitis (Henoch-Schönlein), and Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)
Variable-vessel vasculitis
Behçet's syndrome
Cogan's syndrome
Single-organ vasculitis
Cutaneous leukocytoclastic angiitis
Cutaneous arteritis
Primary central nervous system vasculitis
Isolated aortitis
Vasculitis associated with systemic disease
Lupus vasculitis
Rheumatoid vasculitis
Sarcoid vasculitis
Others
Vasculitis associated with probable etiology
Hepatitis C virus-associated cryoglobulinemic vasculitis
Hepatitis B virus-associated vasculitis
Syphilis-associated aortitis
Drug-associated immune complex vasculitis
Drug-associated ANCA-associated vasculitis
Cancer-associated vasculitis
other

Diagnosis

Shown below is an algorithm summarizing the diagnosis of Primary systemic vasculitis according to the Royal College of Physicians guidelines. The diagnosis of the individual vasculitides is generally based on patterns of organ injury, the size of the vessels affected, histopathological features, and characteristic findings on diagnostic imaging.

History: Previous drug usage, Family history of autoimmune rheumatic disease , Fever, sweats, Weight loss, anorexia, Malaise, fatigue, persistent skin rashes, Cutaneous ulcer, Myalgia, Arthralgia, epistaxis, Sinusitis, Painful red eye, Sight loss, Wrist drop, foot drop, Stroke, Seizure, Headache, Scalp tenderness, Jaw claudication, Asthma, Limb claudication, Abdominal pain, Haematemesis, melaena, haematochezia, Frothy urine, haematuria, Scrotal pain

Clinical Findings: cachexia, pallor, purpuric/petechial rash, digital ulcers or gangrene, arthritis, muscle weakness, nasal crusting, nasal bridge collapse, optic neuritis, uveitis, episcleritis, temporal artery tenderness and nodularity, neurological deficit, peripheral neuropathy, absent peripheral pulses

Laboratory tests

Additional tests

Treatment

Shown below is an algorithm summarizing the treatment of [[disease name]] according the the [...] guidelines.

Do's

• The content in this section is in bullet points.

Don'ts

• The content in this section is in bullet points.

References

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