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| 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.[2] Diagnosis is based on a combination of history, clinical findings, laboratory tests, additional tests.
| 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: | |||||||||||||||||
| 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
- ↑ Jennette JC, Falk RJ, Bacon PA et al (2013) 2012 Revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum 65:1–11. https://doi.org/10.1002/art.37715
- ↑ An update on the general management approach to common vasculitides Mooikhin Hng, Sizheng S Zhao, Robert J Moots Clinical Medicine Nov 2020, 20 (6) 572-579; DOI: 10.7861/clinmed.2020-0747