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vasculitis

Etiology: 1) primary a) small vessel vasculitis* - antineutrophil cytoplasmic antibody [ANCA] associated - microscopic polyangiitis - Wegener's granulomatosis - Churg-Strauss syndrome - leukocytoclastic vasculitis - Henoch-Scholein purpura (IgA vasculitis) - cutaneous leukocytoclastic vasculitis - chronic leukocytoclastic vasculitis - hypersensitivity vasculitis - cryoglobulinemia b) medium vessel vasculitis - Kawasaki's disease - polyarteritis nodosa - isolated central nervous system vasculitis c) large vessel vasculitis - giant cell (temporal) arteritis - polymyalgia rheumatica (not a vasculitis) - Takayasu's syndrome 2) secondary - drug-related vasculitis - serum sickness - vasculitis associated with infection - Streptococcus - Mycoplasma - Legionella - Neisseria meningitidis - Rickettsia rickettsii (Rocky Mountain spotted fever) - hepatitis A, hepatitis B, hepatitis C - HIV1 - parvovirus B19 - bacterial endocarditis - 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 - rheumatoid arthritis (RA) - systemic lupus erythematosus (SLE) - Sjogren's syndrome - scleroderma - polymyositis/dermatomyositis - 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: 1) general - fatigue, fever, weight loss, anorexia 2) skin manifestations - palpable purpura, tender cutaneous nodules, petechiae, necrotic cutaneous ulcers, livedo reticularis, urticaria (persisting > 24 hours), digital infarcts 3) arthralgias/arthritis (serositis) 4) myalgia 5) claudication 6) phlebitis 7) headache 8) cerebrovascular accident: a) generally ischemic stroke b) hemorrhagic stroke is uncommon 9) mononeuritis multiplex 10) hypertension 12) pulmonary manifestations a) pulmonary hemorrhage b) pulmonary nodules with cavities 13) abnormal pain or intestinal hemorrhage 14) rapidly progressive glomerulonephritis [3] Laboratory: 1) serology a) anti neutrophil cytoplasmic antibody (ANCA) b) anti C1q antibodies c) anti glomerular basement membrane (GBM) antibodies d) anti hepatitis B antibodies e) antinuclear antibodies (ANA) f) cryoglobulins g) anti SSA/Ro h) anti SSB/La i) rheumatoid factor 2) urinalysis: abnormal sediment of renal origin (RBC casts) 3) non-specific indicators of inflammation a) complete blood count: 1] anemia 2] thrombocytosis 3] eosinophilia c) serum albumin: hypoalbuminemia d) elevated ESR e) increased serum transaminases 4) serum complement 5) see ARUP consult [7] Differential diagnosis: - see pseudovasculitis - scurvy Management: 1) primary vasculitis a) see specific etiology b) high-dose glucocorticoids with or without other immunosuppressive agents c) in the absence of internal organ involvement, high-dose glucocorticoids may not be indicated [3] 2) secondary vasculities a) treat underlying etiology b) discontinue offending agents

Interactions

disease interactions

Related

drug-related vasculitis pseudovasculitis

Specific

ANCA-associated vasculitis angiitis 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 polyarteritis polymyalgia rheumatica (PMR) secondary vasculitis small vessel vasculitis

General

inflammation vascular disease (vasculopathy)

References

  1. Clinical Diagnosis & Management by Laboratory Methods, 19th edition, J.B. Henry (ed), W.B. Saunders Co., Philadelphia, PA. 1996, pg 1025-35
  2. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 848-51
  3. 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
  4. Khasnis A and Langford CA. Update on vasculitis. J Allergy Clin Immunol 2009 Jun; 123:1226. PMID: 19501230
  5. Jennette JC et al. 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum 2013 Jan; 65:1 PMID: 23045170 http://onlinelibrary.wiley.com/doi/10.1002/art.37715/abstract;jsessionid=0CF89D730EAE2455B030700B2676A7FB.d02t01
  6. 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: 18413444
  7. 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
  8. Cacoub P, Terrier B, Saadoun D Hepatitis C virus-induced vasculitis: therapeutic options. Ann Rheum Dis. 2014 Jan;73(1):24-30 PMID: 23921995
  9. Marzano AV, Vezzoli P, Berti E. Skin involvement in cutaneous and systemic vasculitis. Autoimmun Rev. 2013 Feb;12(4):467-76 PMID: 22959234
  10. Jennette JC, Falk RJ Small-Vessel Vasculitis. N Engl J Med 1997; 337:1512-1523. November 20, 1997 PMID: 9366584 http://www.nejm.org/doi/full/10.1056/NEJM199711203372106
  11. 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: 27097818
  12. 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: 31843213
  13. National Heart, Lung, and Blood Institute (NHLBI) Vasculitis https://www.nhlbi.nih.gov/health-topics/vasculitis