vasculitis
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Etiology
- primary
- secondary
- drug-related vasculitis
- serum sickness
- vasculitis associated with infection
- vasculitis associated with malignancy (esp lymphoid)
- hairy cell leukemia associated with polyarteritis nodosa
- lymphomatoid granulomatosis
- hypocomplementemic urticarial vasculitis
- hypergammaglobulinemic purpura
- cryoglobulinemia (hepatitis C)
- radiation vasculitis
- vascular rejection associated with transplantation
- connective tissue disease-associated vasculitis
- Goodpasture's syndrome
- sarcoidosis
- relapsing polychondritis
- antiphospholipid antibody syndrome
- Behcet's disease
- thromboangiitis obliterans (Buerger's disease)
- livedoid vasculitis
- ulcerative colitis
- primary biliary cirrhosis
- myositis (inflammatory myopathy)
* see small vessel vasculitis for differential diagnosis
Pathology
- inflammation of blood vessels[2]
- stenosis with subsequent tissue ischemia
- aneurysms
- hemorrhage
Clinical manifestations
- general
- skin manifestations
- palpable purpura, tender cutaneous nodules, petechiae, necrotic cutaneous ulcers, livedo reticularis, urticaria (persisting > 24 hours), digital infarcts
- arthralgias/arthritis (serositis)
- myalgia
- claudication
- phlebitis
- headache
- cerebrovascular accident:
- generally ischemic stroke
- hemorrhagic stroke is uncommon
- mononeuritis multiplex
- hypertension
- pulmonary manifestations
- abnormal pain or intestinal hemorrhage
- rapidly progressive glomerulonephritis[3]
Laboratory
- serology
- urinalysis: abnormal sediment of renal origin (RBC casts)
- non-specific indicators of inflammation
- serum complement
- see ARUP consult[7]
Differential diagnosis
Management
- primary vasculitis
- see specific etiology
- high-dose glucocorticoids with or without other immunosuppressive agents
- in the absence of internal organ involvement, high-dose glucocorticoids may not be indicated[3]
- secondary vasculities
- treat underlying etiology
- discontinue offending agents
More general terms
More specific terms
- ANCA-associated vasculitis
- angiitis
- arteritis
- autoimmune vasculitis
- Behcet syndrome
- cerebral granulomatous angiitis
- cocaine-levamisole-induced cutaneous vasculitis
- Cogan's syndrome; diffuse interstitial keratitis
- cryoglobulinemia (cryoglobulinemic vasculitis)
- cutaneous vasculitis; predominantly cutaneous vasculitis
- erythema induratum (nodular vasculitis)
- Hughes-Stovin syndrome
- isolated (primary) central nervous system vasculitis (angiitis) (PACNS)
- Kawasaki disease; mucocutaneous lymph node disease
- leukocytoclastic vasculitis; hypersensitivity vasculitis; trisymptome
- livedoid vasculitis
- medium vessel vasculitis
- phlebitis
- polyarteritis
- polymyalgia rheumatica (PMR)
- secondary vasculitis
- small vessel vasculitis
- venulitis
Additional terms
References
- ↑ Clinical Diagnosis & Management by Laboratory Methods, 19th edition, J.B. Henry (ed), W.B. Saunders Co., Philadelphia, PA. 1996, pg 1025-35
- ↑ 2.0 2.1 Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 848-51
- ↑ 3.0 3.1 3.2 Medical Knowledge Self Assessment Program (MKSAP) 11,15,16,17,18. American College of Physicians, Philadelphia 1998,2009,2012,2015,2018.
Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022 - ↑ Khasnis A and Langford CA. Update on vasculitis. J Allergy Clin Immunol 2009 Jun; 123:1226. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19501230
- ↑ Jennette JC et al. 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum 2013 Jan; 65:1 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/23045170 <Internet> http://onlinelibrary.wiley.com/doi/10.1002/art.37715/abstract;jsessionid=0CF89D730EAE2455B030700B2676A7FB.d02t01
- ↑ Mukhtyar C, Guillevin L, Cid MC et al EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009 Mar;68(3):310-7 PMID: https://www.ncbi.nlm.nih.gov/pubmed/18413444
- ↑ 7.0 7.1 ARUP Consult: Systemic Vasculitis The Physician's Guide to Laboratory Test Selection & Interpretation https://arupconsult.com/content/vasculitis
ARUP consult (Algorithm) Vasculitis in Children Testing https://arupconsult.com/algorithm/vasculitis-children-testing-algorithm Vasculitis in Adults Testing https://arupconsult.com/algorithm/vasculitis-adults-testing-algorithm - ↑ Cacoub P, Terrier B, Saadoun D Hepatitis C virus-induced vasculitis: therapeutic options. Ann Rheum Dis. 2014 Jan;73(1):24-30 PMID: https://www.ncbi.nlm.nih.gov/pubmed/23921995
- ↑ Marzano AV, Vezzoli P, Berti E. Skin involvement in cutaneous and systemic vasculitis. Autoimmun Rev. 2013 Feb;12(4):467-76 PMID: https://www.ncbi.nlm.nih.gov/pubmed/22959234
- ↑ Jennette JC, Falk RJ Small-Vessel Vasculitis. N Engl J Med 1997; 337:1512-1523. November 20, 1997 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/9366584 <Internet> http://www.nejm.org/doi/full/10.1056/NEJM199711203372106
- ↑ Sharma A, Dhooria A, Aggarwal A, Rathi M, Chandran V. Connective Tissue Disorder-Associated Vasculitis. Curr Rheumatol Rep. 2016 Jun;18(6):31. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/27097818
- ↑ Perkins A, Sontheimer C, Otjen JP, Shenoi S. Scurvy masquerading as juvenile idiopathic arthritis or vasculitis with elevated inflammatory markers: A case series. J Pediatr 2019 Dec 13; S0022-3476(19)31470-2 PMID: https://www.ncbi.nlm.nih.gov/pubmed/31843213
- ↑ National Heart, Lung, and Blood Institute (NHLBI) Vasculitis https://www.nhlbi.nih.gov/health-topics/vasculitis