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Published in: Current Rheumatology Reports 7/2017

01-07-2017 | Vasculitis (L Espinoza, Section Editor)

Rheumatoid Vasculitis: A Diminishing Yet Devastating Menace

Authors: Shweta Kishore, Lisa Maher, Vikas Majithia

Published in: Current Rheumatology Reports | Issue 7/2017

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Abstract

Purpose of Review

Rheumatoid vasculitis (RV) is an unusual complication of long-standing rheumatoid arthritis, which is characterized by the development of necrotizing or leukocytoclastic vasculitis involving small or medium-sized vessels. In this review, we aim to provide an update on the epidemiology, pathogenesis, clinical presentation, and management of this challenging extra-articular manifestation.

Recent Findings

RV is heterogenous in its clinical presentation depending on the organ and size of blood vessels involved. The most common organs involved are the skin and peripheral nerve. Based on recent population studies, the incidence has significantly decreased with early recognition and the advent of immunosuppressive drugs and biologics; however, the mortality rates remain high.

Summary

RV remains a serious extra-articular manifestation of RA that needs to be promptly recognized and treated. No consensus is available on treatment, given the ongoing debate of whether the biologics can trigger or treat RV.
Literature
2.
go back to reference Voskuyl AE, Zwinderman AH, Westedt ML, et al. Factors associated with the development of vasculitis in rheumatoid arthritis: results of a case-control study. Ann Rheum Dis. 1996;55(2):190–2.CrossRefPubMedPubMedCentral Voskuyl AE, Zwinderman AH, Westedt ML, et al. Factors associated with the development of vasculitis in rheumatoid arthritis: results of a case-control study. Ann Rheum Dis. 1996;55(2):190–2.CrossRefPubMedPubMedCentral
4.
go back to reference Scott DG, Bacon PA, Tribe CR. Systemic rheumatoid vasculitis: a clinical and laboratory study of 50 cases. Medicine (Baltimore). 1981;60(4):288–97.CrossRef Scott DG, Bacon PA, Tribe CR. Systemic rheumatoid vasculitis: a clinical and laboratory study of 50 cases. Medicine (Baltimore). 1981;60(4):288–97.CrossRef
5.
go back to reference Flipo RM, Cardon T, Copin MD, et al. ICAM-1, E-selectin, and TNF alpha expression in labial salivary glands of patients with rheumatoid vasculitis. Ann Rheum Dis. 1997;56(1):41–4.CrossRefPubMedPubMedCentral Flipo RM, Cardon T, Copin MD, et al. ICAM-1, E-selectin, and TNF alpha expression in labial salivary glands of patients with rheumatoid vasculitis. Ann Rheum Dis. 1997;56(1):41–4.CrossRefPubMedPubMedCentral
6.
go back to reference Martens PB, Goronzy JJ, Schaid D, et al. Expansion of unusual CD4+ T cells in severe rheumatoid arthritis. Arthritis Rheum. 1997;40(6):1106–14.CrossRefPubMed Martens PB, Goronzy JJ, Schaid D, et al. Expansion of unusual CD4+ T cells in severe rheumatoid arthritis. Arthritis Rheum. 1997;40(6):1106–14.CrossRefPubMed
7.
go back to reference Yen J, Moore B, Nakajima T, et al. Major histocompatibility complex class I-recognizing receptors are disease risk genes in rheumatoid arthritis. J Exp Med. 2001;193(10):1159–67.CrossRefPubMedPubMedCentral Yen J, Moore B, Nakajima T, et al. Major histocompatibility complex class I-recognizing receptors are disease risk genes in rheumatoid arthritis. J Exp Med. 2001;193(10):1159–67.CrossRefPubMedPubMedCentral
8.
go back to reference •• Makol A, Crowson CS, Wetter DA, et al. Vasculitis associated with rheumatoid arthritis: a case-control study. Rheumatology (Oxford). 2014;53(5):890–9. doi:10.1093/rheumatology/ket475. This is a retrospective case-control study where 86 RV patients were compared to controls to identify risk factors for development of RV. CrossRef •• Makol A, Crowson CS, Wetter DA, et al. Vasculitis associated with rheumatoid arthritis: a case-control study. Rheumatology (Oxford). 2014;53(5):890–9. doi:10.​1093/​rheumatology/​ket475. This is a retrospective case-control study where 86 RV patients were compared to controls to identify risk factors for development of RV. CrossRef
9.
go back to reference Albano SA, Santana-Sahagun E, Weisman M. Cigarette smoking and rheumatoid arthritis. Semin Arthritis Rheum. 2001;31(3):146–59.CrossRefPubMed Albano SA, Santana-Sahagun E, Weisman M. Cigarette smoking and rheumatoid arthritis. Semin Arthritis Rheum. 2001;31(3):146–59.CrossRefPubMed
10.
go back to reference Gorman J, David-Vaudey E, Pai M, et al. Particular HLA-DRB1 shared epitope genotypes are strongly associated with rheumatoid vasculitis. Arthritis Rheum. 2004;50(11):3476–84.CrossRefPubMed Gorman J, David-Vaudey E, Pai M, et al. Particular HLA-DRB1 shared epitope genotypes are strongly associated with rheumatoid vasculitis. Arthritis Rheum. 2004;50(11):3476–84.CrossRefPubMed
11.
go back to reference Turesson C, Schaid DJ, Weyand CM, et al. Association of HLA-C3 and smoking with vasculitis in patients with rheumatoid arthritis. Arthritis Rheum. 2006;54(9):2776–83.CrossRefPubMed Turesson C, Schaid DJ, Weyand CM, et al. Association of HLA-C3 and smoking with vasculitis in patients with rheumatoid arthritis. Arthritis Rheum. 2006;54(9):2776–83.CrossRefPubMed
13.
go back to reference •• Ntatsaki E, Mooney J, Scott DG, et al. Systemic rheumatoid vasculitis in the era of modern immunosuppressive therapy. Rheumatology (Oxford). 2014;53(1):145–52. doi:10.1093/rheumatology/ket326. In this study, the clinical manifestations of RV patients (2001–2010) were reviewed and compared to a previous cohort (1988–2000). CrossRef •• Ntatsaki E, Mooney J, Scott DG, et al. Systemic rheumatoid vasculitis in the era of modern immunosuppressive therapy. Rheumatology (Oxford). 2014;53(1):145–52. doi:10.​1093/​rheumatology/​ket326. In this study, the clinical manifestations of RV patients (2001–2010) were reviewed and compared to a previous cohort (1988–2000). CrossRef
14.
go back to reference Watts RA, Carruthers CA, Symmons DP. The incidence of rheumatoid vasculitis in the Norwich Health Authority. Br J Rheumatol. 1994;33(9):832–3.CrossRefPubMed Watts RA, Carruthers CA, Symmons DP. The incidence of rheumatoid vasculitis in the Norwich Health Authority. Br J Rheumatol. 1994;33(9):832–3.CrossRefPubMed
16.
go back to reference Genta MS, Genta RM, Gabay C. Systemic rheumatoid vasculitis: a review. Semin Arthritis Rheum. 2006;36(2):88–98.CrossRefPubMed Genta MS, Genta RM, Gabay C. Systemic rheumatoid vasculitis: a review. Semin Arthritis Rheum. 2006;36(2):88–98.CrossRefPubMed
17.
go back to reference Pagnoux C, Mahr A, Cohen P, et al. Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome, or rheumatoid arthritis-associated vasculitis. Medicine (Baltimore). 2005;84(2):115–28.CrossRef Pagnoux C, Mahr A, Cohen P, et al. Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome, or rheumatoid arthritis-associated vasculitis. Medicine (Baltimore). 2005;84(2):115–28.CrossRef
18.
go back to reference van Laar JM, Smit VT, de Beus WM, et al. Rheumatoid vasculitis presenting as appendicitis. Clin Exp Rheumatol. 1998;16(6):736–8.PubMed van Laar JM, Smit VT, de Beus WM, et al. Rheumatoid vasculitis presenting as appendicitis. Clin Exp Rheumatol. 1998;16(6):736–8.PubMed
20.
go back to reference Souza AW, Rosa DP, Buosi AL, et al. Testicular vasculitis: a rare manifestation of rheumatoid arthritis. Rev Bras Reumatol. 2013;53(4):365–7.CrossRefPubMed Souza AW, Rosa DP, Buosi AL, et al. Testicular vasculitis: a rare manifestation of rheumatoid arthritis. Rev Bras Reumatol. 2013;53(4):365–7.CrossRefPubMed
23.
go back to reference Gravallese EM, Corson JM, Coblyn JS, et al. Rheumatoid aortitis: a rarely recognized but clinically significant entity. Medicine (Baltimore). 1989;68(2):95–106.CrossRef Gravallese EM, Corson JM, Coblyn JS, et al. Rheumatoid aortitis: a rarely recognized but clinically significant entity. Medicine (Baltimore). 1989;68(2):95–106.CrossRef
24.
go back to reference Bannatayne GA. Rheumatoid arthritis, its pathology, morbid anatomy and treatment. 2nd ed. Bristol, England: John Wright; 1898. p. 182. Bannatayne GA. Rheumatoid arthritis, its pathology, morbid anatomy and treatment. 2nd ed. Bristol, England: John Wright; 1898. p. 182.
25.
go back to reference Watts RA, Mooney J, Lane SE, et al. Rheumatoid vasculitis: becoming extinct? Rheumatology (Oxford). 2004;43(7):920–3.CrossRef Watts RA, Mooney J, Lane SE, et al. Rheumatoid vasculitis: becoming extinct? Rheumatology (Oxford). 2004;43(7):920–3.CrossRef
27.
go back to reference Scott DG, Bacon PA. Intravenous cyclophosphamide plus methylprednisolone in treatment of systemic rheumatoid vasculitis. Am J Med. 1984;76(3):377–84.CrossRefPubMed Scott DG, Bacon PA. Intravenous cyclophosphamide plus methylprednisolone in treatment of systemic rheumatoid vasculitis. Am J Med. 1984;76(3):377–84.CrossRefPubMed
28.
go back to reference Turesson C, Jacobsson LT. Epidemiology of extra-articular manifestations in rheumatoid arthritis. Scand J Rheumatol. 2004;33(2):65–72.CrossRefPubMed Turesson C, Jacobsson LT. Epidemiology of extra-articular manifestations in rheumatoid arthritis. Scand J Rheumatol. 2004;33(2):65–72.CrossRefPubMed
30.
go back to reference Puéchal X, Said G, Hilliquin P, et al. Peripheral neuropathy with necrotizing vasculitis in rheumatoid arthritis. A clinicopathologic and prognostic study of thirty-two patients. Arthritis Rheum. 1995;38(11):1618–29.CrossRefPubMed Puéchal X, Said G, Hilliquin P, et al. Peripheral neuropathy with necrotizing vasculitis in rheumatoid arthritis. A clinicopathologic and prognostic study of thirty-two patients. Arthritis Rheum. 1995;38(11):1618–29.CrossRefPubMed
31.
go back to reference Laskari K, Ahmadi-Simab K, Lamken M, et al. Are anti-cyclic citrullinated peptide autoantibodies seromarkers for rheumatoid vasculitis in a cohort of patients with systemic vasculitis? Ann Rheum Dis. 2010;69(2):469–71.CrossRefPubMed Laskari K, Ahmadi-Simab K, Lamken M, et al. Are anti-cyclic citrullinated peptide autoantibodies seromarkers for rheumatoid vasculitis in a cohort of patients with systemic vasculitis? Ann Rheum Dis. 2010;69(2):469–71.CrossRefPubMed
32.
go back to reference Coremans IE, Hagen EC, Daha MR, et al. Antilactoferrin antibodies in patients with rheumatoid arthritis are associated with vasculitis. Arthritis Rheum. 1992;35(12):1466–75.CrossRefPubMed Coremans IE, Hagen EC, Daha MR, et al. Antilactoferrin antibodies in patients with rheumatoid arthritis are associated with vasculitis. Arthritis Rheum. 1992;35(12):1466–75.CrossRefPubMed
33.
go back to reference Magro CM, Crowson AN. The spectrum of cutaneous lesions in rheumatoid arthritis: a clinical and pathological study of 43 patients. J Cutan Pathol. 2003;30(1):1–10.CrossRefPubMed Magro CM, Crowson AN. The spectrum of cutaneous lesions in rheumatoid arthritis: a clinical and pathological study of 43 patients. J Cutan Pathol. 2003;30(1):1–10.CrossRefPubMed
36.
go back to reference Goldman JA, Casey HL, McIlwain H, et al. Limited plasmapheresis in rheumatoid arthritis with vasculitis. Arthritis Rheum. 1979;22(10):1146–50.CrossRefPubMed Goldman JA, Casey HL, McIlwain H, et al. Limited plasmapheresis in rheumatoid arthritis with vasculitis. Arthritis Rheum. 1979;22(10):1146–50.CrossRefPubMed
37.
go back to reference 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(6):880–4.CrossRefPubMed 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(6):880–4.CrossRefPubMed
38.
go back to reference • Puechal X, Gottenberg JE, Berthelot JM, et al. Rituximab therapy for systemic vasculitis associated with rheumatoid arthritis: results from the AutoImmunity and Rituximab Registry. Arthritis Care Res (Hoboken). 2012;64(3):331–9. doi:10.1002/acr.20689. The authors assessed the outcome of RV patients treated with rituximab and found it to be a suitable therapeutic option to induce remission. CrossRef • Puechal X, Gottenberg JE, Berthelot JM, et al. Rituximab therapy for systemic vasculitis associated with rheumatoid arthritis: results from the AutoImmunity and Rituximab Registry. Arthritis Care Res (Hoboken). 2012;64(3):331–9. doi:10.​1002/​acr.​20689. The authors assessed the outcome of RV patients treated with rituximab and found it to be a suitable therapeutic option to induce remission. CrossRef
41.
go back to reference Sumida K, Ubara Y, Takemoto F, et al. Successful treatment with humanized anti-interleukin 6 receptor antibody for multidrug-refractory and anti-tumour necrosis factor-resistant systemic rheumatoid vasculitis. Clin Exp Rheumatol. 2011;29(1 Suppl 64):S133.PubMed Sumida K, Ubara Y, Takemoto F, et al. Successful treatment with humanized anti-interleukin 6 receptor antibody for multidrug-refractory and anti-tumour necrosis factor-resistant systemic rheumatoid vasculitis. Clin Exp Rheumatol. 2011;29(1 Suppl 64):S133.PubMed
Metadata
Title
Rheumatoid Vasculitis: A Diminishing Yet Devastating Menace
Authors
Shweta Kishore
Lisa Maher
Vikas Majithia
Publication date
01-07-2017
Publisher
Springer US
Published in
Current Rheumatology Reports / Issue 7/2017
Print ISSN: 1523-3774
Electronic ISSN: 1534-6307
DOI
https://doi.org/10.1007/s11926-017-0667-3

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