Vasculitis resident survival guide
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dina Elantably, MD[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:
|
Immune complex small-vessel 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 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 | |||||||||||||||||||||||||||||||||||
| 1-Exclude vasculitis “mimics” and secondary causes: Blood cultures Echocardiogram Hepatitis screen (B and C) HIV test Antiglomerular basement membrane antibody Antiphospholipid antibodies | 2-Assess extent of vasculitis: Urine dipstick and microscopy (all patients) Chest radiography (all patients) | 3- Confirm the Diagnosis: Skin Biopsy/temporal artery biopsy and/or angiogram | 4- Identify specific cause of primary vasculitis: ANCA, | ||||||||||||||||||||||||||||||||
Treatment
Shown below is an algorithm summarizing the treatment of ANCA associated vasculitis (AAV) 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:
1- Remission induction: Involves the use of medium to high doses of glucocorticoids with the use of other immunosuppressive agents.
2- Remission maintenance: Once remission has been attained, the dose of glucocorticoids is usually steadily lowered, as tolerated.
3- Monitoring: Patients require monitoring for both disease activity and drug toxicity during the active treatment phase and recurrence of vasculitis.
| Induction of Remission | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Cyclophosphamide + Glucocorticoid | OR Rituximab + Glucocorticoid | No organ threatening involved: Mycophenolate Mofetyl or Methotrexate | Vital organ/life threatening/creatinine>500 mmol/L : Add Plasma exchange | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Disease control on Drug "Remission" | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Switch to Azathioprine or MTX & Taper Glucocorticoid | Disease control on Drug "Remission" | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Taper Aza or MTX | Continue on RTX and Taper Glucocorticoids | {{{ }}} | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Stop RTX | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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.