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Scleroderma associated with ANCA-associated vasculitis

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Abstract

We have recently reported on two cases of scleroderma patients with ANCA-associated vasculitis for the first time in Korea. In order to explore the nature of this disease combination, we pooled together all the previously known cases and statistically analyzed them. Out of the 50 selected cases, survival analysis was done for comparison of the scleroderma disease period and the clinical factors associated with ANCA-associated vasculitis (AAV). Kaplan-Meier analysis revealed that patients having anti-topoisomerase antibody (anti-Scl-70) and, probably, PR-3 ANCA are at a higher risk for developing AAV than patients without both anti-topoisomerase antibody and anti-centromere antibody (ACA), and patients with MPO-ANCA. Multivariate Cox regression analysis revealed having anti-topoisomerase antibody as a risk factor for developing AAV [OR 3.1 (95% CI 1.11–8.55), P=0.031]. We suggest that having anti-topoisomerase antibodies may play a role among scleroderma patients in developing AAV.

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References

  1. Endo H, Hosono T, Kondo H (1994) Antineutrophil cytoplasmic autoantibodies in 6 patients with renal failure and systemic sclerosis. J Rheumatol 21:864–870

    PubMed  CAS  Google Scholar 

  2. Akimoto S, Ishikawa O, Tamura T, Miyachi Y (1996) Antineutrophil cytoplasmic autoantibodies in patients with systemic sclerosis. Br J Dermatol 134:407–410

    Article  PubMed  CAS  Google Scholar 

  3. Kiraz S, Simsek H, Ertenli I, Benekli M, Kadayifci A (1996) Antineutrophil cytoplasmic antibodies in systemic sclerosis. Clin Rheumatol 15:519–520

    Article  PubMed  CAS  Google Scholar 

  4. Locke IC, Worrall JG, Leaker B, Black CM, Cambridge G (1997) Autoantibodies to myeloperoxidase in systemic sclerosis. J Rheumatol 24:86–89

    PubMed  CAS  Google Scholar 

  5. Bellisai F, Morozzi G, Bacarelli MR, Radice A, Sinico RA, Wieslander J, Sebastiani GD, Campanella V, Marcolongo R, Galeazzi M (2001) Anti-proteinase 3 antibodies in diffuse systemic sclerosis (SSc) with normotensive renal impairment: is it suggestive for an overlapping between SSc and idiopathic vasculitis? Reumatismo 53:33–39

    PubMed  Google Scholar 

  6. Casari S, Haeney M, Farrand S, Herrick A (2002) Antineutrophil cytoplasmic antibodies a "Red Flag" in patients with systemic sclerosis. J Rheumatol 29:2666–2667

    PubMed  Google Scholar 

  7. Caramaschi P, Biasi D, Tonolli E, Carletto A, Bambara LM (2002) Antineutrophil cytoplasmic antibodies in scleroderma patients: first report of a case with anti-proteinase 3 antibodies and review of the literature. Joint Bone Spine 69:177–180

    Article  PubMed  Google Scholar 

  8. Ruffatti A, Sinico RA, Radice A, Ossi E, Cozzi F, Tonello M, Grypiotis P, Todesco S (2002) Autoantibodies to proteinase 3 and myeloperoxidase in systemic sclerosis. J Rheumatol 29:918–923

    PubMed  CAS  Google Scholar 

  9. Rho YH, Choi SJ, Ji JD, Lee YH, Song GG (2005) Scleroderma associated with ANCA-associated vasculitis. A case report. Rheumatol Int (in submission). DOI 10.1007/s00296-005-0003-5

  10. Le Thi Huong D, Gatfosse M, Papo T, Beaufils H, Faucher C, Frances C, Godeau P (1994) Wegener’s granulomatosis and CREST syndrome. Br J Rheumatol 33:1087–1088

    Article  CAS  Google Scholar 

  11. Kobayashi M, Saito M, Minoshima S, Arimura Y, Nagasawa T (1995) A case of progressive systemic sclerosis with crescentic glomerulonephritis associated with myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) and anti-glomerular basement membrane antibody (anti-GBM Ab). Nippon Jinzo Gakkai Shi 37:207–211

    PubMed  CAS  Google Scholar 

  12. Vazquez-Del Mercado M, Mendoza-Topete A, Best-Aguilera CR, Garcia-De La Torre I (1996) Diffuse alveolar hemorrhage in limited cutaneous systemic sclerosis with positive perinuclear antineutrophil cytoplasmic antibodies. J Rheumatol 23:1821–1823

    PubMed  CAS  Google Scholar 

  13. Carvajal I, Bernis C, Sanz P, Garcia A, Garcia-Vadillo A, Traver JA (1997) Antineutrophil cytoplasmic autoantibodies (ANCA) and systemic sclerosis. Nephrol Dial Transplant 12:576–577

    Article  PubMed  CAS  Google Scholar 

  14. Hillis GS, Khan IH, Simpson JG, Rees AJ (1997) Scleroderma, D-penicillamine treatment, and progressive renal failure associated with positive antimyeloperoxidase antineutrophil cytoplasmic antibodies. Am J Kidney Dis 30:279–281

    PubMed  CAS  Google Scholar 

  15. Karpinski J, Jothy S, Radoux V, Levy M, Baran D (1997) D-penicillamine-induced crescentic glomerulonephritis and antimyeloperoxidase antibodies in a patient with scleroderma. Case report and review of the literature. Am J Nephrol 17:528–532

    Article  PubMed  CAS  Google Scholar 

  16. Omote A, Muramatsu M, Sugimoto Y, Hosono S, Murakami R, Tanaka H, Watanabe Y, Sano H, Kato K (1997) Myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibodies-related scleroderma renal crisis treated with double-filtration plasmapheresis. Intern Med 36:508–513

    Article  PubMed  CAS  Google Scholar 

  17. Uriuhara A, Morita S, Yamada K, Masugi J, Yamada H, Hasegawa Y, Yokono K, Kasuga M (1997) A case of MPO-ANCA positive progressive systemic sclerosis with status epileptics. Ryumachi 37:483–486

    PubMed  CAS  Google Scholar 

  18. Marchand-Courville S, Dhib M, Fillastre JP, Godin M (1998) Extracapillary glomerulonephritis secondary to D-penicillamine. Apropos of 1 case and review of the literature. Nephrologie 19:25–32

    PubMed  CAS  Google Scholar 

  19. Anders HJ, Wiebecke B, Haedecke C, Sanden S, Combe C, Schlondorff D (1999) MPO-ANCA-Positive crescentic glomerulonephritis: a distinct entity of scleroderma renal disease? Am J Kidney Dis 33:e3

    PubMed  CAS  Google Scholar 

  20. Harashima S, Yoshizawa S, Horiuchi T, Nakashima H, Niho Y, Kusaba T, Hayashida I, Shinozaki M, Katafuchi R, Hirakata H (1999) A case of systemic sclerosis with crescentic glomerulonephritis associated with perinuclear-antineutrophil cytoplasmic antibody (p-ANCA). Nihon Rinsho Meneki Gakkai Kaishi 22:86–92

    PubMed  CAS  Google Scholar 

  21. Katrib A, Sturgess A, Bertouch JV (1999) Systemic sclerosis and antineutrophil cytoplasmic autoantibody-associated renal failure. Rheumatol Int 19:61–63

    Article  PubMed  CAS  Google Scholar 

  22. Kyndt X, Ducq P, Bridoux F, Reumaux D, Makdassi R, Gheerbrant JD, Vanhille P (1999) Extracapillary glomerulonephritis with anti-myeloperoxidase antibodies in 2 patients with systemic scleroderma treated with penicillamine D. Presse Med 28:67–70

    PubMed  CAS  Google Scholar 

  23. Villaverde V, Balsa A, Cabezas JA, Fernandez-Prada M, Torre A, Mola EM (1999) Normotensive renal failure in a patient with systemic sclerosis and p-antineutrophil cytoplasmic autoantibodies which developed into Paget’s disease of bone after immunosuppressive therapy. Rheumatology (Oxford) 38:190–191

    Article  CAS  Google Scholar 

  24. Maes B, Van Mieghem A, Messiaen T, Kuypers D, Van Damme B, Vanrenterghem Y (2000) Limited cutaneous systemic sclerosis associated with MPO-ANCA positive renal small vessel vasculitis of the microscopic polyangiitis type. Am J Kidney Dis 36:E16

    PubMed  CAS  Google Scholar 

  25. Martinez Ara J, Picazo ML, Torre A, Pascual D, Diaz Rodriguez C, Rinon C (2000) Progressive systemic sclerosis associated with anti-myeloperoxidase ANCA vasculitis with renal and cutaneous involvement. Nefrologia 20:383–386

    PubMed  CAS  Google Scholar 

  26. Mizutani A, Tanaka I, Katayama M, Oshima H, Komatsu Y, Asano S, Kato K, Matsumura H, Ishii K, Miyama H, Nagai T, Fukaya S, Yoshida S, Hasegawa M, Kawashima S, Torikai K (2000) A case of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) related glomerulonephritis associated with systemic sclerosis treated by steroid pulse therapy: a case report. Ryumachi 40:828–832

    PubMed  CAS  Google Scholar 

  27. Yamashita K, Yorioka N, Kyuden Y, Naito T, Tanji C, Ueda C, Usui K, Shigemoto K, Harada S, Yamakido M (2000) A case of CREST syndrome and myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis. Clin Nephrol 53:296–300

    PubMed  CAS  Google Scholar 

  28. Wutzl AL, Foley RN, O’Driscoll BR, Reeve RS, Chisholm R, Herrick AL (2001) Microscopic polyangiitis presenting as pulmonary-renal syndrome in a patient with long-standing diffuse cutaneous systemic sclerosis and antibodies to myeloperoxidase. Arthritis Rheum 45:533–536

    Article  PubMed  CAS  Google Scholar 

  29. Kumakura S, Ishikura H, Kondo M, Murakawa Y, Kobayashi S (2002) Hemophagocytosis associated with MPO-ANCA positive vasculitis in systemic sclerosis. Clin Exp Rheumatol 20:411–414

    PubMed  CAS  Google Scholar 

  30. Miyamura T, Yamamoto M, Shimada H, Suematsu E (2002) Systemic sclerosis associated with microscopic polyangitis presenting with high myeloperoxidase (MPO) titer and necrotizing angitis: a case report. Ryumachi 42:910–914

    PubMed  Google Scholar 

  31. Miyata N, Kobayashi T, Matsukawa Y, Sawada S, Nishinarita S, Horie T (2002) Anti-neutrophil cytoplasmic autoantibody-associated rapid progressive glomerulonephritis complicated with both limited and diffuse scleroderma. Nihon Rinsho Meneki Gakkai Kaishi 25:473–479

    PubMed  Google Scholar 

  32. Tomioka M, Hinoshita F, Miyauchi N, Akiyama Y, Saima S, Hiroe M (2004) ANCA-related crescentic glomerulonephritis in a patient with scleroderma without marked dermatological change and malignant hypertension. Intern Med 43:496–502

    Article  PubMed  Google Scholar 

  33. Wigley FM, Hummers LK (2003) Clinical features of systemic sclerosis. In: Hochberg MC, Silman AJ, Smolen JS, Wienblatt ME, Weismann MH (eds) Rheumatology, 3rd ed. Edinburgh, Mosby, pp 1463–1477

    Google Scholar 

  34. Mayes MD (2003) Scleroderma epidemiology. Rheum Dis Clin North Am 29:239–254

    Article  PubMed  Google Scholar 

  35. Arbuckle MR, McClain MT, Rubertone MV, Scofield RH, Dennis GJ, James JA, Harley JB (2003) Development of autoantibodies before the clinical onset of systemic lupus erythematosus. N Engl J Med 349:1526–1533

    Article  PubMed  CAS  Google Scholar 

  36. Homma S, Matsushita H, Nakata K (2004) Pulmonary fibrosis in myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitides. Respirology 9:190–196

    Article  PubMed  Google Scholar 

  37. Lhote F, Cohen P, Genereau T, Gayraud M, Guillevin L (1996) Microscopic polyangiitis: clinical aspects and treatment. Ann Med Interne (Paris) 147:165–177

    CAS  Google Scholar 

  38. Gaudin PB, Askin FB, Falk RJ, Jennette JC (1995) The pathologic spectrum of pulmonary lesions in patients with anti-neutrophil cytoplasmic autoantibodies specific for anti-proteinase 3 and anti-myeloperoxidase. Am J Clin Pathol 104:7–16

    PubMed  CAS  Google Scholar 

  39. Nada AK, Torres VE, Ryu JH, Lie JT, Holley KE (1990) Pulmonary fibrosis as an unusual clinical manifestation of a pulmonary-renal vasculitis in elderly patients. Mayo Clin Proc 65:847–856

    PubMed  CAS  Google Scholar 

  40. Eschun GM, Mink SN, Sharma S (2003) Pulmonary interstitial fibrosis as a presenting manifestation in perinuclear antineutrophilic cytoplasmic antibody microscopic polyangiitis. Chest 123:297–301

    Article  PubMed  Google Scholar 

  41. Donohoe JF (1992) Scleroderma and the kidney. Kidney Int 41:462–477

    Article  PubMed  CAS  Google Scholar 

  42. Steen VD, Costantino JP, Shapiro AP, Medsger TA Jr (1990) Outcome of renal crisis in systemic sclerosis: relation to availability of angiotensin converting enzyme (ACE) inhibitors. Ann Intern Med 113:352–357

    PubMed  CAS  Google Scholar 

  43. Steen VD, Medsger TA Jr (1998) Case-control study of corticosteroids and other drugs that either precipitate or protect from the development of scleroderma renal crisis. Arthritis Rheum 41:1613–1619

    Article  PubMed  CAS  Google Scholar 

  44. Khanna D, Aggarwal A, Bhakuni DS, Dayal R, Misra R (2003) Bactericidal/permeability-increasing protein and cathepsin G are the major antigenic targets of antineutrophil cytoplasmic autoantibodies in systemic sclerosis. J Rheumatol 30:1248–1252

    PubMed  CAS  Google Scholar 

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Correspondence to Gwan Gyu Song.

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The authors have declared that there is no conflict of interests or affiliation to any interest group, and no publication of this study has been done.

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Rho, Y.H., Choi, S.J., Lee, Y.H. et al. Scleroderma associated with ANCA-associated vasculitis. Rheumatol Int 26, 369–375 (2006). https://doi.org/10.1007/s00296-005-0011-5

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