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] 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: complete blood cell count (CBC), serum creatinine, liver function studies, erythrocyte sedimentation rate (ESR) and/or C-reactive protein (CRP), serologies for viral hepatitis, serum cryoglobulins, and a urinalysis with urinary sediment. Blood cultures should be drawn to help exclude infection (eg, infective endocarditis), ANA, complement, ANCA | Additional tests: chest radiograph or high-resolution computed tomography (HRCT) of the chest, Electromyography, lumbar puncture, Magnetic resonance imaging (MRI), MR angiograms, CT angiograms, vascular ultrasound, and positron emission tomography (PET) | Biopsy: temporal artery biopsy should always be performed in cases of suspected GCA, skin biopsies of purpuric lesions and renal biopsies in glomerulonephritis | |||||||||||||||||||||
Treatment
Shown below is an algorithm summarizing the treatment of Systemic vasculitis according to The British Society for Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) [3], The European League Against Rheumatism (EULAR) and European Renal Association – European Dialysis and Transplant Association (ERA-EDTA)[4] and Canadian Vasculitis Research Network (CanVasc) [5]guidelines. The treatment generally goes through 3 phases: Remission induction, Remission maintenance, and Monitoring.
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
- ↑ Ntatsaki E, Carruthers D, Chakravarty K, et al. BSR, and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology 2014;53:2306–9.
- ↑ Yates M, Watts R, Bajema I, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis 2016;75:1583–94.
- ↑ McGeoch L, Twilt M, Famorca L, et al. CanVasc recommendations for the management of antineutrophil cytoplasm antibody-associated vasculitides. J Rheumatol 2016;43:97–120.