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Abstract

Rheumatoid arthritis (RA) is associated with vasculitis in approximately 1% of patients. Rheumatoid vasculitis (RV) refers specifically to a destructive inflammatory process with protean clinical features that center on the blood vessel wall. The disease strongly resembles polyarteritis nodosa in its predilection for particular organs: the skin, peripheral nerves, gastrointestinal tract, and heart. RV can affect a wide range of blood vessel types, from medium-sized muscular arteries to smaller arterioles even post-capillary venules (the size of blood vessels involved in many forms of small-vessel vasculitis). RV usually occurs in patients with severe, longstanding, nodular, destructive RA. Palpable purpura, cutaneous ulcers (particularly in the malleolar region), digital infarctions, and peripheral sensory neuropathy are common manifestations. Tissue biopsy establishes the diagnosis of RV. Deep skin biopsies from the border of ulcers may capture medium-sized vessels that are involved. Nerve conduction studies help identify involved nerves for biopsy. Muscle biopsies should be performed simultaneously with nerve biopsies to increase the diagnostic yield of the procedure.

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References

  • Achkar AA, Stanson AW, Johnson CM et al Rheumatoid vasculitis manifesting as intra-abdominal hemorrhage. Mayo Clin Proc 1995; 70(6):565–9

    Article  PubMed  CAS  Google Scholar 

  • Ball J. Rheumatoid arthritis and polyarteritis nodosa. Ann Rheum Dis 1954; 13:277–290

    Article  PubMed  CAS  Google Scholar 

  • Boers M, Croonen AM, Dijkmans BAC, et al Renal findings in rheumatoid arthritis: Clinical aspects of 132 necropsies. Ann Rheum Dis 1987; 46:658–663

    Article  PubMed  CAS  Google Scholar 

  • Bywaters EGL. A variant of rheumatoid arthritis characterized by recurrent digital pad nodules and palmar fasciitis, closely resembling palindromic rheumatism. Ann Rheum Dis 1949; 8:1–30

    Article  PubMed  CAS  Google Scholar 

  • Bywaters EGL. Peripheral vascular obstruction in rheumatoid arthritis and its relationship to other vascular lesions. Ann Rheum Dis 1957; 16:84–103

    Article  PubMed  CAS  Google Scholar 

  • Conn DL, Schroeter AL, McDuffie FC. Immunopathologic study of sural nerves in rheumatoid arthritis. Arthritis Rheum 1972; 15:135

    Article  PubMed  CAS  Google Scholar 

  • Cruikschank B. The arteritis of rheumatoid arthritis. Ann Rheum Dis 1954; 13:136–145

    Article  Google Scholar 

  • Epstein W V, Engleman EP. The relation of the rheumatoid factor content of serum to clinical neurovascular manifestations of rheumatoid arthritis. Arthritis Rheum 1959; 2:250–258

    Article  PubMed  CAS  Google Scholar 

  • Fong LP, Sainz de la Maza M, Rice BA et al Immunopathology of scleritis. Ophthalmology 1991; 98(4):472–9

    PubMed  CAS  Google Scholar 

  • Geirsson AJ, Sturfelt G, Truedsson L, et al Clinical and serological features of severe vasculitis in rheumatoid arthritis: Prognostic implications. Ann Rheum Dis 1987; 46:727–733

    Article  PubMed  CAS  Google Scholar 

  • Harper L, Cockwell P, Howie AJ, et al Focal segmental necrotizing glomerulonephritis in rheumatoid arthritis. QJM 1997; 90:125

    Article  PubMed  CAS  Google Scholar 

  • Hazes JM. Management of Extraarticular Disease and Complications. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH (eds) Extraarticular Features of Rheumatoid Arthritis and Systemic Involvement. Rheumatology (4th edn). Philadelphia, PA, Mosby, 2008, pp. 897–914

    Google Scholar 

  • Hurlimann D, Forster A, Noll, G et al Anti-tumor necrosis factor-alpha treatment improves endothelial function in patients with rheumatoid arthritis. Circulation 2002; 106:2184

    Article  PubMed  Google Scholar 

  • Johnson RL, Smyth CJ, Holt GW, et al Steroid therapy and vascular lesions in rheumatoid arthritis. Arthritis Rheum 1959; 2:224–249

    Article  PubMed  CAS  Google Scholar 

  • Kumeda, Y, Inaba, M, Goto, H, et al Increased thickness of the arterial intima-media detected by ultrasonography in patients with rheumatoid arthritis. Arthritis Rheum 2002; 46:1489

    Article  PubMed  Google Scholar 

  • McCluskey PJ, Watson PG, Lightman S, et al Posterior scleritis: Clinical features, systemic associations, and outcome in a large series of patients. Ophthalmology 1999; 106:2380–2386

    Article  PubMed  CAS  Google Scholar 

  • Mongan ES, Cass RM, Jacox RF, et al A study of the relation of seronegative and seropositive rheumatoid arthritis to each other and to necrotizing vasculitis. Am J Med 1969; 47:23–35

    Article  PubMed  CAS  Google Scholar 

  • Okhravi N, Odufuwa B, McCluskey P, et al Scleritis. Surv Ophthalm 2005; 50(4):351–363

    Article  Google Scholar 

  • Pagnoux, C, Mahr, A, Cohen, P, Guillevin, L. Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: Analysis of 62 patients with polyarteritis nodosa, microscopic poly-angiitis, Wegener granulomatosis, Churg-Strauss syndrome, or rheumatoid arthritis-associated vasculitis. Medicine (Baltimore) 2005; 84:115

    Article  Google Scholar 

  • Park YB, Ahn CW, Choi HK, et al Atherosclerosis in rheumatoid arthritis: Morphologic evidence obtained by carotid ultrasound. Arthritis Rheum 2002; 46:1714

    Article  PubMed  Google Scholar 

  • Price-Forbes AN, Watts RA, Lane SE, et al Do we need to treat isolated nailfold vasculitis (NFV) in rheumatoid arthritis (RA) more aggressively? Abstract 848, American College of Rheumatology annual scientific meeting, October 2002

    Google Scholar 

  • Puechal X, Said G, Hilliquin P, et al Peripheral neuropathy with necrotiz-ing vasculitis in rheumatoid arthritis. A clinicopathologic and prognostic study of thirty-two patients. Arthritis Rheum 1995; 38:1618–1629

    Article  PubMed  CAS  Google Scholar 

  • Puechal X, Miceli-Richard C, Mejjad O, et al Anti-tumour necrosis factor treatment in patients with refractory systemic vasculitis associated with rheumatoid arthritis. Ann Rheum Dis 2008; 67:880–884

    Article  PubMed  CAS  Google Scholar 

  • Said G, Lacroix C. Primary and secondary vasculitis neuropathy. J Neurol 2005; 252:633–641

    Article  PubMed  Google Scholar 

  • Schmid FR, Cooper NS, Ziff M, et al Arteritis in rheumatoid arthritis. Am J Med 1961; 30:56–83

    Article  PubMed  CAS  Google Scholar 

  • Scott DG, Bacon PA, Tribe CR. Systemic rheumatoid vasculitis: A clinical and laboratory study of 50 cases. Medicine (Baltimore) 1981; 60:288

    CAS  Google Scholar 

  • Sokoloff L. The heart in rheumatoid arthritis. Am Heart J 1953; 45:635

    Article  PubMed  CAS  Google Scholar 

  • Sokoloff L, McCluskey RT, Bunim JJ. Vascularity of the early subcutaneous nodule in rheumatoid arthritis. Arch Pathol 1953; 55: 475–479

    CAS  Google Scholar 

  • Squirrell DM, Winfield J, Amos RS. Peripheral ulcerative keratitis “corneal melt” and rheumatoid arthritis: A case series. Rheumatology 1999; 1245–1248

    Google Scholar 

  • Turesson C, Matteson EL. Management of extraarticular disease manifestations in rheumatoid arthritis. Curr Opin Rheum 2004;16:206–211

    Article  Google Scholar 

  • Turesson C, Matteson EL. Extraarticular features of rheumatoid arthritis and systemic involvement. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH (eds) Rheumatology (4th edn). Philadelphia, PA, Mosby, 2008, pp. 773–783

    Google Scholar 

  • Turesson C, O'Fallon WM, Crowson C, et al Extraarticular disease manifestations in rheumatoid arthritis: Incidence, trends, and risk factors over 46 years. Annals of Rheumatic Disease 2003; 62:722–727

    Article  CAS  Google Scholar 

  • Turesson, C, McClelland, R, Christianson, TJ, Matteson, EL. No decrease over time in the incidence of vasculitis or other extra-artic-ular manifestations in rheumatoid arthritis (abstract). Arthritis Rheum 2004; 50:S380

    Article  Google Scholar 

  • Turesson C, Schaid DJ, Weyand CM, et al The impact of HLA-DRB1 genes on extraarticular disease manifestations in rheumatoid arthritis. Arthritis Res Ther 2005; 7:R1386–R1393

    Article  PubMed  CAS  Google Scholar 

  • Van Albada-Kuipers GA, Bruijn JA, Westedt ML, et al Coronary arteritis complicating rheumatoid arthritis. Ann Rheum Dis 1986; 45:963

    Article  PubMed  Google Scholar 

  • Van Gaalen FA, Linn-Rasker SP, van Venrooij WJ, et al Autoantibodies to cyclic citrullinated pepties predict progression to rheumatoid arthritis in patients with undifferentiated arthritis. Arthritis Rheum 2004; 50(3):709–715

    Article  PubMed  Google Scholar 

  • Vollertsen RS, Conn DL, Ballard DJ, et al Rheumatoid vasculitis: Survival and associated risk factors. Medicine 1986; 65(6):365–375

    Article  PubMed  CAS  Google Scholar 

  • Voskuyl AE, Zwinderman AH, Wested ML, et al The mortality of rheumatoid vasculitis compared with rheumatoid arthritis. Arthritis Rheum 1996; 39:266–271

    Article  PubMed  CAS  Google Scholar 

  • Wallberg-Jonsson, S, Cvetkovic, JT, Sundqvist, KG, et al Activation of the immune system and inflammatory activity in relation to markers of atherothrombotic disease and atherosclerosis in rheumatoid arthritis. J Rheumatol 2002; 29:875

    PubMed  CAS  Google Scholar 

  • Watts RA, Carruthers DM, Scott DG. Isolated nail fold vasculitis in rheumatoid arthritis. Ann Rheum Dis 1995; 54:927

    Article  PubMed  CAS  Google Scholar 

  • Watts RA, Mooney J, Lane SE, Scott DG. Rheumatoid vasculitis: Becoming extinct?. Rheumatology (Oxford) 2004; 43:920

    Article  CAS  Google Scholar 

  • Yousem SA, Colby TV, Carrington CB. Lung biopsy in rheumatoid arthritis. Am Rev Respir Dis 1985; 131:770

    PubMed  CAS  Google Scholar 

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Stone, J.H., Matteson, E.L. (2009). Rheumatoid Vasculitis. In: Stone, J.H. (eds) A Clinician's Pearls and Myths in Rheumatology. Springer, London. https://doi.org/10.1007/978-1-84800-934-9_2

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  • DOI: https://doi.org/10.1007/978-1-84800-934-9_2

  • Publisher Name: Springer, London

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