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Scleroderma Renal Crisis

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Scleroderma

Abstract

Systemic sclerosis (SSc) is a multisystem autoimmune rheumatic disease that results in substantial morbidity and significant mortality. Indeed, it is the autoimmune rheumatic disease with the highest case-specific mortality, and this occurs as a result of organ-based complications. Historically, renal manifestations were a major cause of death, but recent series show that most patients dying from SSc do so as a result of cardiopulmonary complications including, pulmonary hypertension, lung fibrosis, and myocardial involvement. (Nihtyanova SI, Tang EC, Coghlan JG, Wells AU, Black CM, Denton CP. Improved survival in systemic sclerosis is associated with better ascertainment of internal organ disease: a retrospective cohort study. QJM. 2010;103(2):109–15.) In many cases, there is co-occurrence of these different manifestations, and this may account for the particular severity of SSc. Although there are a range of ways in which the kidneys can be affected in SSc, the most significant renal manifestation is the scleroderma renal crisis (SRC), and this was historically a major cause of scleroderma-associated death.

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References

  1. Nihtyanova SI, Tang EC, Coghlan JG, Wells AU, Black CM, Denton CP. Improved survival in systemic sclerosis is associated with better ascertainment of internal organ disease: a retrospective cohort study. QJM. 2010;103(2):109–15.

    Article  PubMed  CAS  Google Scholar 

  2. Mpofu S, Rhodes JM, Mpofu CM, Moots RJ. An unusual cause of acute renal failure in systemic sclerosis. Ann Rheum Dis. 2003;62(12):1133–4.

    Article  PubMed  CAS  Google Scholar 

  3. Steen VD, Costantino JP, Shapiro AP, Medsger Jr TA. Outcome of renal crisis in systemic sclerosis: relation to availability of angiotensin converting enzyme (ACE) inhibitors [see comments]. Ann Intern Med. 1990;113(5):352–7.

    PubMed  CAS  Google Scholar 

  4. Arnaud L, Huart A, Plaisier E, Francois H, Mougenot B, Tiev K, et al. ANCA-related crescentic glomerulonephritis in systemic sclerosis: revisiting the “normotensive scleroderma renal crisis”. Clin Nephrol. 2007;68(3):165–70.

    PubMed  CAS  Google Scholar 

  5. Steen VD, Syzd A, Johnson JP, Greenberg A, Medsger Jr TA. Kidney disease other than renal crisis in patients with diffuse scleroderma. J Rheumatol. 2005;32(4):649–55.

    PubMed  Google Scholar 

  6. Auspitz H. Ein beit zur lehre vom haute-sklerem der erwachsenen. Wrin Med Wschr. 1863;13:739–55.

    Google Scholar 

  7. Osler W. The principles and practice of medicine. New York: Appleton; 1892. p. 993–8.

    Google Scholar 

  8. Goetz RH. The pathology of progressive systemic sclerosis (generalized scleroderma) with special reference to changes in the viscera. Clin Proc Graduate Medical Association. 1945;4:337–92.

    Google Scholar 

  9. Moore HC, Sheehan HL. The kidney of scleroderma. Lancet. 1952;1:68.

    Article  PubMed  CAS  Google Scholar 

  10. Denton CP, Lapadula G, Mouthon L, Muller-Ladner U. Renal complications and scleroderma renal crisis. Rheumatology (Oxford). 2009;48(Suppl 3):iii32–5.

    Article  Google Scholar 

  11. Steen VD, Medsger Jr TA. Case-control study of corticosteroids and other drugs that either precipitate or protect from the development of scleroderma renal crisis. Arthritis Rheum. 1998;41(9):1613–9.

    Article  PubMed  CAS  Google Scholar 

  12. Steen VD, Medsger Jr TA, Rodnan GP. D-Penicillamine therapy in progressive systemic sclerosis (scleroderma): a retrospective analysis. Ann Intern Med. 1982;97(5):652–9.

    PubMed  CAS  Google Scholar 

  13. Penn H, Howie AJ, Kingdon EJ, Bunn CC, Stratton RJ, Black CM, et al. Scleroderma renal crisis: patient characteristics and long-term outcomes. QJM. 2007;100(8):485–94.

    Article  PubMed  CAS  Google Scholar 

  14. Teixeira L, Mouthon L, Mahr A, Berezne A, Agard C, Mehrenberger M, et al. Mortality and risk factors of scleroderma renal crisis: a French retrospective study of 50 patients. Ann Rheum Dis. 2008;67(1):110–6.

    Article  PubMed  CAS  Google Scholar 

  15. Steen VD, Lanz Jr JK, Conte C, Owens GR, Medsger Jr TA. Therapy for severe interstitial lung disease in systemic sclerosis. A retrospective study [see comments]. Arthritis Rheum. 1994;37(9):1290–6.

    Article  PubMed  CAS  Google Scholar 

  16. Traub YM, Shapiro AP, Rodnan GP, Medsger TA, McDonald Jr RH, Steen VD, et al. Hypertension and renal failure (scleroderma renal crisis) in progressive systemic sclerosis. Review of a 25-year experience with 68 cases. Medicine (Baltimore). 1983;62(6):335–52.

    CAS  Google Scholar 

  17. Steen VD, Medsger Jr TA. Epidemiology and natural history of systemic sclerosis. Rheum Dis Clin North Am. 1990;16(1):1–10.

    PubMed  CAS  Google Scholar 

  18. Donohoe JF. Scleroderma and the kidney. Kidney Int. 1992;41(2):462–77.

    Article  PubMed  CAS  Google Scholar 

  19. Steen VD, Ziegler GL, Rodnan GP, Medsger Jr TA. Clinical and laboratory associations of anticentromere antibody in patients with progressive systemic sclerosis. Arthritis Rheum. 1984;27(2):125–31.

    Article  PubMed  CAS  Google Scholar 

  20. Steen VD, Medsger Jr TA, Osial Jr TA, Ziegler GL, Shapiro AP, Rodnan GP. Factors predicting development of renal involvement in progressive systemic sclerosis. Am J Med. 1984;76(5):779–86.

    Article  PubMed  CAS  Google Scholar 

  21. Korzets Z, Schneider M, Savin H, Ben Chetrit S, Bernheim J, Shitrit P, et al. Intriguing presentation of scleroderma renal crisis (scleroderma renal crisis sine scleroderma sine hypertension). Nephrol Dial Transplant. 1998;13(11):2953–6.

    Article  PubMed  CAS  Google Scholar 

  22. Molina JF, Anaya JM, Cabrera GE, Hoffman E, Espinoza LR. Systemic sclerosis sine scleroderma: an unusual presentation in scleroderma renal crisis. J Rheumatol. 1995;22(3):557–60.

    PubMed  CAS  Google Scholar 

  23. Phan TG, Cass A, Gillin A, Trew P, Fertig N, Sturgess A. Anti-RNA polymerase III antibodies in the diagnosis of scleroderma renal crisis sine scleroderma. J Rheumatol. 1999;26(11):2489–92.

    PubMed  CAS  Google Scholar 

  24. Leroy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger Jr TA, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988;15(2):202–5.

    PubMed  CAS  Google Scholar 

  25. Steen VD, Medsger Jr TA. The palpable tendon friction rub: an important physical examination finding in patients with systemic sclerosis [see comments]. Arthritis Rheum. 1997;40(6):1146–51.

    Article  PubMed  CAS  Google Scholar 

  26. Bunn CC, Denton CP, Shi-wen X, Knight C, Black CM. Anti-RNA polymerases and other autoantibody specificities in systemic sclerosis. Br J Rheumatol. 1998;37(1):15–20.

    Article  PubMed  CAS  Google Scholar 

  27. Okano Y, Steen VD, Medsger Jr TA. Autoantibody reactive with RNA polymerase III in systemic sclerosis. Ann Intern Med. 1993;119(10):1005–13.

    PubMed  CAS  Google Scholar 

  28. Rivolta R, Mascagni B, Berruti V, Quarto DP, Elli A, Scorza R, et al. Renal vascular damage in systemic sclerosis patients without clinical evidence of nephropathy. Arthritis Rheum. 1996;39(6):1030–4.

    Article  PubMed  CAS  Google Scholar 

  29. Kovalchik MT, Guggenheim SJ, Silverman MH, Robertson JS, Steigerwald JC. The kidney in progressive systemic sclerosis: a prospective study. Ann Intern Med. 1978;89(6):881–7.

    PubMed  CAS  Google Scholar 

  30. Clements PJ, Lachenbruch PA, Furst DE, Maxwell M, Danovitch G, Paulus HE. Abnormalities of renal physiology in systemic sclerosis. A prospective study with 10-year followup. Arthritis Rheum. 1994;37(1):67–74.

    Article  PubMed  CAS  Google Scholar 

  31. McWhorter JE, Leroy EC. Pericardial disease in scleroderma (systemic sclerosis). Am J Med. 1974;57(4):566–75.

    Article  PubMed  Google Scholar 

  32. Helfrich DJ, Banner B, Steen VD, Medsger Jr TA. Normotensive renal failure in systemic sclerosis. Arthritis Rheum. 1989;32(9):1128–34.

    Article  PubMed  CAS  Google Scholar 

  33. Kohno K, Katayama T, Majima K, Fujisawa M, Iida S, Fukami K, et al. A case of normotensive scleroderma renal crisis after high-dose methylprednisolone treatment [In Process Citation]. Clin Nephrol. 2000;53(6):479–82.

    PubMed  CAS  Google Scholar 

  34. Bar J, Ehrenfeld M, Rozenman J, Perelman M, Sidi Y, Gur H. Pulmonary-renal syndrome in systemic sclerosis. Semin Arthritis Rheum. 2001;30(6):403–10.

    Article  PubMed  CAS  Google Scholar 

  35. Kapur A, Ballou SP, Renston JP, Luna E, Chung-Park M. Recurrent acute scleroderma renal crisis complicated by thrombotic thrombocytopenic purpura. J Rheumatol. 1997;24(12):2469–72.

    PubMed  CAS  Google Scholar 

  36. Kfoury Baz EM, Mahfouz RA, Masri AF, Jamaleddine GW. Thrombotic thrombocytopenic purpura in a case of scleroderma renal crisis treated with twice-daily therapeutic plasma exchange. Ren Fail. 2001;23(5):737–42.

    Article  PubMed  CAS  Google Scholar 

  37. Manadan AM, Harris C, Block JA. Thrombotic thrombocytopenic purpura in the setting of systemic sclerosis. Semin Arthritis Rheum. 2005;34(4):683–8.

    Article  PubMed  Google Scholar 

  38. Lian EC. Pathogenesis of thrombotic thrombocytopenic purpura: ADAMTS13 deficiency and beyond. Semin Thromb Hemost. 2005;31(6):625–32.

    Article  PubMed  CAS  Google Scholar 

  39. Torok KS, Cortese Hassett A, Kiss JE, Lucas M, Medsger TA. Scleroderma renal crisis and thrombotic thrombocytopenic purpura – are they related? Arthritis Rheum. 2008;58(10):S396.

    Google Scholar 

  40. Follansbee W. The cardiovascular manifestations of systemic sclerosis (scleroderma). In: O’Rourke RA, editor. Current problems in cardiology. Chicago: Yearbook Medical Publishers; 1986. p. 245–98.

    Google Scholar 

  41. Stratton RJ, Coghlan JG, Pearson JD, Burns A, Sweny P, Abraham DJ, et al. Different patterns of endothelial cell activation in renal and pulmonary vascular disease in scleroderma. QJM. 1998;91(8):561–6.

    Article  PubMed  CAS  Google Scholar 

  42. Batal I, Domsic R, Medsger TJ, Bastacky S. Scleroderma renal crisis: a pathology perspective. Int J Rheumatol. 2010;54:3704–8.

    Google Scholar 

  43. Nihtyanova SI, Parker JC, Black CM, Bunn CC, Denton CP. A longitudinal study of anti-RNA polymerase III antibody levels in systemic sclerosis. Rheumatology (Oxford). 2009;48(10):1218–21.

    Article  CAS  Google Scholar 

  44. Pham PT, Pham PC, Danovitch GM, Gritsch HA, Singer J, Wallace WD, et al. Predictors and risk factors for recurrent scleroderma renal crisis in the kidney allograft: case report and review of the literature. Am J Transplant. 2005;5(10):2565–9.

    Article  PubMed  Google Scholar 

  45. Kowal-Bielecka O, Landewe R, Avouac J, Chwiesko S, Miniati I, Czirjak L, et al. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis. 2009;68(5):620–8.

    Article  PubMed  CAS  Google Scholar 

  46. Caskey FJ, Thacker EJ, Johnston PA, Barnes JN. Failure of losartan to control blood pressure in scleroderma renal crisis. Lancet. 1997;349(9052):620.

    Article  PubMed  CAS  Google Scholar 

  47. Cheung WY, Gibson IW, Rush D, Jeffery J, Karpinski M. Late recurrence of scleroderma renal crisis in a renal transplant recipient despite angiotensin II blockade. Am J Kidney Dis. 2005;45(5):930–4.

    Article  PubMed  Google Scholar 

  48. Dhaun N, Macintyre IM, Bellamy CO, Kluth DC. Endothelin receptor antagonism and renin inhibition as treatment options for scleroderma kidney. Am J Kidney Dis. 2009;54(4):726–31.

    Article  PubMed  CAS  Google Scholar 

  49. Vancheeswaran R, Magoulas T, Efrat G, Wheeler-Jones C, Olsen I, Penny R, et al. Circulating endothelin-1 levels in systemic sclerosis subsets – a marker of fibrosis or vascular dysfunction? [see comments]. J Rheumatol. 1994;21(10):1838–44.

    PubMed  CAS  Google Scholar 

  50. Kobayashi H, Nishimaki T, Kaise S, Suzuki T, Watanabe K, Kasukawa R, et al. Immunohistological study endothelin-1 and endothelin-A and B receptors in two patients with scleroderma renal crisis. Clin Rheumatol. 1999;18(5):425–7.

    Article  PubMed  CAS  Google Scholar 

  51. Mouthon L, Mehrenberger M, Teixeira L, Fakhouri F, Berezne A, Guillevin L, et al. Endothelin-1 expression in scleroderma renal crisis. Hum Pathol. 2011;42(1):95–102.

    Article  PubMed  CAS  Google Scholar 

  52. Fonseca C, Lindahl GE, Ponticos M, Sestini P, Renzoni EA, Holmes AM, et al. A polymorphism in the CTGF promoter region associated with systemic sclerosis. N Engl J Med. 2007;357(12):1210–20.

    Article  PubMed  CAS  Google Scholar 

  53. Wipff J, Gallier G, Dieude P, Avouac J, Tiev K, Hachulla E, et al. Angiotensin-converting enzyme gene does not contribute to genetic susceptibility to systemic sclerosis in European Caucasians. J Rheumatol. 2009;36(2):337–40.

    Article  PubMed  Google Scholar 

  54. Omote A, Muramatsu M, Sugimoto Y, Hosono S, Murakami R, Tanaka H, et al. Myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibodies – related scleroderma renal crisis treated with double-filtration plasmapheresis. Intern Med. 1997;36(7):508–13.

    Article  PubMed  CAS  Google Scholar 

  55. Steen VD, Medsger Jr TA. Long-term outcomes of scleroderma renal crisis. Ann Intern Med. 2000;133(8):600–3.

    PubMed  CAS  Google Scholar 

  56. Penn H, Denton CP. Diagnosis, management and prevention of scleroderma renal disease. Curr Opin Rheumatol. 2008;20(6):692–6.

    Article  PubMed  Google Scholar 

  57. Chang YJ, Spiera H. Renal transplantation in scleroderma. Medicine (Baltimore). 1999;78(6):382–5.

    Article  CAS  Google Scholar 

  58. Gibney EM, Parikh CR, Jani A, Fischer MJ, Collier D, Wiseman AC. Kidney transplantation for systemic sclerosis improves survival and may modulate disease activity. Am J Transplant. 2004;4(12):2027–31.

    Article  PubMed  Google Scholar 

  59. Batal I, Domsic RT, Shafer A, Medsger Jr TA, Kiss LP, Randhawa P, et al. Renal biopsy findings predicting outcome in scleroderma renal crisis. Hum Pathol. 2009;40(3):332–40.

    Article  PubMed  Google Scholar 

  60. Herrera-Esparza R, Aguilar JL, Saucedo A, Gonzalez I, Lopez-Robles E, Avalos-Diaz E. Scleroderma with type III glomerulonephritis and MPO-ANCA antibodies in the serum. J Eur Acad Dermatol Venereol. 2005;19(5):617–20.

    Article  PubMed  CAS  Google Scholar 

  61. Casari S, Haeney M, Farrand S, Herrick A. Antineutrophil cytoplasmic antibodies a “Red Flag” in patients with systemic sclerosis. J Rheumatol. 2002;29(12):2666–7.

    PubMed  Google Scholar 

  62. Mimura I, Hori Y, Matsukawa T, Uozaki H, Tojo A, Fujita T. Noncrescentic ANCA-associated renal crisis in systemic sclerosis. Clin Nephrol. 2008;70(2):183–5.

    PubMed  CAS  Google Scholar 

  63. Anders HJ, Wiebecke B, Haedecke C, Sanden S, Combe C, Schlondorff D. MPO-ANCA-positive crescentic glomerulonephritis: a distinct entity of scleroderma renal disease? Am J Kidney Dis. 1999;33(4):e3.

    Article  PubMed  CAS  Google Scholar 

  64. Kamen DL, Wigley FM, Brown AN. Antineutrophil cytoplasmic antibody-positive crescentic glomerulonephritis in scleroderma – a different kind of renal crisis. J Rheumatol. 2006;33(9):1886–8.

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Inflammation, Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School, London, UK

    Christopher P. Denton PhD, FRCP (Professor of Experimental Rheumatology)

  2. Department of Medicine, Rheumatology, Clinical Immunology and Allergy, Georgetown University Hospital, Washington, DC, USA

    Virginia Steen MD

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  1. Christopher P. Denton PhD, FRCP
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Correspondence to Christopher P. Denton PhD, FRCP .

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Editors and Affiliations

  1. Feinberg School of Medicine, Northwestern University, E. Huron Street 240, Chicago, 60611-2909, Illinois, USA

    John Varga

  2. Royal Free and University College, Medical School, University College London, Rowland Street, London, NW3 2PF, United Kingdom

    Christopher P. Denton

  3. School of Medicine, Div. Rheumatology, Johns Hopkins University, Eastern Avenue 5200, Baltimore, 21224, Maryland, USA

    Fredrick M. Wigley

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Denton, C.P., Steen, V. (2012). Scleroderma Renal Crisis. In: Varga, J., Denton, C., Wigley, F. (eds) Scleroderma. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-5774-0_29

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