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. 1998 Nov;57(11):682–686. doi: 10.1136/ard.57.11.682

Mortality and causes of death in a Swedish series of systemic sclerosis patients

R Hesselstrand 1, A Scheja 1, A Akesson 1
PMCID: PMC1752504  PMID: 9924211

Abstract

OBJECTIVES—To analyse survival rates and the causes of death in a systemic sclerosis (SSc) population, and to evaluate the occurrence of fatal malignant neoplasms and their possible association with oral cyclophosphamide (CYC) treatment.
METHODS—Survival was calculated for 249 SSc patients followed up for up to 13 years. Mean (SD) follow up was 5.8 (4.2) years. The 49 deceased patients were subdivided according to causes of death and its relation to SSc. Fatal malignancies in CYC treated patients were compared with those occurring in non-CYC treated patients.
RESULTS—The overall 5 and 10 year survival rates were 86% and 69% respectively. There was a 4.6-fold increased risk of death, as compared with the general population. Prognosis was worse in the diffuse cutaneous involvement (dSSc) and male subgroups than in the limited cutaneous involvement (lSSc) and female subgroups. Of the 49 deaths, 24 were attributable to pulmonary complications such as pulmonary fibrosis, pulmonary hypertension, pneumonia or pulmonary malignancy. Treatment with oral CYC did not increase the risk of dying of cancer.
CONCLUSIONS—Mortality is increased both in the SSc population as a whole and in its different subsets (dSSc and lSSc). Prognosis is worst among male patients with dSSc. However, the 5 year survival rate was better than those reported from earlier studies. Most patients die of cardiopulmonary disease. Five of seven fatal lung cancers were adenocarcinomas, possibly caused by chronic inflammatory disease of the lung. In this study, CYC treatment was not associated with an increased incidence of fatal malignant neoplasms.

 Keywords: scleroderma systemic; mortality; malignancy; cyclophosphamide

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Figure 1  .

Figure 1  

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Sex as a determinant of survival in systemic sclerosis (p<0.0001). Differences analysed by a proportional hazards model using the Breslow test.

Figure 2  .

Figure 2  

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Skin involvement as a determinant of survival in systemic sclerosis (p<0.0005). Differences analysed by a proportional hazards model using the Breslow test.

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Abu-Shakra M., Guillemin F., Lee P. Cancer in systemic sclerosis. Arthritis Rheum. 1993 Apr;36(4):460–464. doi: 10.1002/art.1780360405. [DOI] [PubMed] [Google Scholar]
  2. Abu-Shakra M., Lee P. Mortality in systemic sclerosis: a comparison with the general population. J Rheumatol. 1995 Nov;22(11):2100–2102. [PubMed] [Google Scholar]
  3. Akesson A., Scheja A., Lundin A., Wollheim F. A. Improved pulmonary function in systemic sclerosis after treatment with cyclophosphamide. Arthritis Rheum. 1994 May;37(5):729–735. doi: 10.1002/art.1780370518. [DOI] [PubMed] [Google Scholar]
  4. Akesson A., Wollheim F. A. Organ manifestations in 100 patients with progressive systemic sclerosis: a comparison between the CREST syndrome and diffuse scleroderma. Br J Rheumatol. 1989 Aug;28(4):281–286. doi: 10.1093/rheumatology/28.4.281. [DOI] [PubMed] [Google Scholar]
  5. Altman R. D., Medsger T. A., Jr, Bloch D. A., Michel B. A. Predictors of survival in systemic sclerosis (scleroderma). Arthritis Rheum. 1991 Apr;34(4):403–413. doi: 10.1002/art.1780340405. [DOI] [PubMed] [Google Scholar]
  6. Barnett A. J., Miller M. H., Littlejohn G. O. A survival study of patients with scleroderma diagnosed over 30 years (1953-1983): the value of a simple cutaneous classification in the early stages of the disease. J Rheumatol. 1988 Feb;15(2):276–283. [PubMed] [Google Scholar]
  7. Bryan C., Howard Y., Brennan P., Black C., Silman A. Survival following the onset of scleroderma: results from a retrospective inception cohort study of the UK patient population. Br J Rheumatol. 1996 Nov;35(11):1122–1126. doi: 10.1093/rheumatology/35.11.1122. [DOI] [PubMed] [Google Scholar]
  8. Chandran G., Smith M., Ahern M. J., Roberts-Thomson P. J. A study of scleroderma in South Australia: prevalence, subset characteristics and nailfold capillaroscopy. Aust N Z J Med. 1995 Dec;25(6):688–694. doi: 10.1111/j.1445-5994.1995.tb02854.x. [DOI] [PubMed] [Google Scholar]
  9. Czirják L., Nagy Z., Szegedi G. Survival analysis of 118 patients with systemic sclerosis. J Intern Med. 1993 Sep;234(3):335–337. doi: 10.1111/j.1365-2796.1993.tb00753.x. [DOI] [PubMed] [Google Scholar]
  10. Dau P. C., Kahaleh M. B., Sagebiel R. W. Plasmapheresis and immunosuppressive drug therapy in scleroderma. Arthritis Rheum. 1981 Sep;24(9):1128–1136. doi: 10.1002/art.1780240903. [DOI] [PubMed] [Google Scholar]
  11. Geirsson A. J., Blom-Bülow B., Pahlm O., Akesson A. Cardiac involvement in systemic sclerosis. Semin Arthritis Rheum. 1989 Oct;19(2):110–116. doi: 10.1016/0049-0172(89)90055-3. [DOI] [PubMed] [Google Scholar]
  12. Geirsson A. J., Steinsson K., Guthmundsson S., Sigurthsson V. Systemic sclerosis in Iceland. A nationwide epidemiological study. Ann Rheum Dis. 1994 Aug;53(8):502–505. doi: 10.1136/ard.53.8.502. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Giordano M., Valentini G., Migliaresi S., Picillo U., Vatti M. Different antibody patterns and different prognoses in patients with scleroderma with various extent of skin sclerosis. J Rheumatol. 1986 Oct;13(5):911–916. [PubMed] [Google Scholar]
  14. Harris E. C., Barraclough B. M. Suicide as an outcome for medical disorders. Medicine (Baltimore) 1994 Nov;73(6):281–296. doi: 10.1097/00005792-199411000-00001. [DOI] [PubMed] [Google Scholar]
  15. Kaburaki J., Lee C. C., Kuwana M., Tojo T., Ikeda Y., Takano M., Funatsu Y. Initial predictors of survival in patients with systemic sclerosis (scleroderma). Keio J Med. 1992 Sep;41(3):141–145. doi: 10.2302/kjm.41.141. [DOI] [PubMed] [Google Scholar]
  16. Kuwana M., Kaburaki J., Okano Y., Tojo T., Homma M. Clinical and prognostic associations based on serum antinuclear antibodies in Japanese patients with systemic sclerosis. Arthritis Rheum. 1994 Jan;37(1):75–83. doi: 10.1002/art.1780370111. [DOI] [PubMed] [Google Scholar]
  17. Lally E. V., Jimenez S. A., Kaplan S. R. Progressive systemic sclerosis: mode of presentation, rapidly progressive disease course, and mortality based on an analysis of 91 patients. Semin Arthritis Rheum. 1988 Aug;18(1):1–13. doi: 10.1016/0049-0172(88)90030-3. [DOI] [PubMed] [Google Scholar]
  18. LeRoy E. C., Black C., Fleischmajer R., Jablonska S., Krieg T., Medsger T. A., Jr, Rowell N., Wollheim F. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988 Feb;15(2):202–205. [PubMed] [Google Scholar]
  19. Lee P., Langevitz P., Alderdice C. A., Aubrey M., Baer P. A., Baron M., Buskila D., Dutz J. P., Khostanteen I., Piper S. Mortality in systemic sclerosis (scleroderma). Q J Med. 1992 Feb;82(298):139–148. [PubMed] [Google Scholar]
  20. Medsger T. A., Jr, Masi A. T., Rodnan G. P., Benedek T. G., Robinson H. Survival with systemic sclerosis (scleroderma). A life-table analysis of clinical and demographic factors in 309 patients. Ann Intern Med. 1971 Sep;75(3):369–376. doi: 10.7326/0003-4819-75-3-369. [DOI] [PubMed] [Google Scholar]
  21. Medsger T. A., Jr, Masi A. T. Survival with scleroderma. II. A life-table analysis of clinical and demographic factors in 358 male U.S. veteran patients. J Chronic Dis. 1973 Oct;26(10):647–660. doi: 10.1016/0021-9681(73)90054-4. [DOI] [PubMed] [Google Scholar]
  22. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum. 1980 May;23(5):581–590. doi: 10.1002/art.1780230510. [DOI] [PubMed] [Google Scholar]
  23. Radis C. D., Kahl L. E., Baker G. L., Wasko M. C., Cash J. M., Gallatin A., Stolzer B. L., Agarwal A. K., Medsger T. A., Jr, Kwoh C. K. Effects of cyclophosphamide on the development of malignancy and on long-term survival of patients with rheumatoid arthritis. A 20-year followup study. Arthritis Rheum. 1995 Aug;38(8):1120–1127. doi: 10.1002/art.1780380815. [DOI] [PubMed] [Google Scholar]
  24. Rocco V. K., Hurd E. R. Scleroderma and scleroderma-like disorders. Semin Arthritis Rheum. 1986 Aug;16(1):22–69. doi: 10.1016/0049-0172(86)90040-5. [DOI] [PubMed] [Google Scholar]
  25. Rosenthal A. K., McLaughlin J. K., Linet M. S., Persson I. Scleroderma and malignancy: an epidemiological study. Ann Rheum Dis. 1993 Jul;52(7):531–533. doi: 10.1136/ard.52.7.531. [DOI] [PMC free article] [PubMed] [Google Scholar]
  26. Sakkas L. I., Moore D. F., Akritidis N. C. Cancer in families with systemic sclerosis. Am J Med Sci. 1995 Dec;310(6):223–225. [PubMed] [Google Scholar]
  27. Silman A. J. Mortality from scleroderma in England and Wales 1968-1985. Ann Rheum Dis. 1991 Feb;50(2):95–96. doi: 10.1136/ard.50.2.95. [DOI] [PMC free article] [PubMed] [Google Scholar]
  28. Silver R. M., Warrick J. H., Kinsella M. B., Staudt L. S., Baumann M. H., Strange C. Cyclophosphamide and low-dose prednisone therapy in patients with systemic sclerosis (scleroderma) with interstitial lung disease. J Rheumatol. 1993 May;20(5):838–844. [PubMed] [Google Scholar]
  29. Steen V. D., Powell D. L., Medsger T. A., Jr Clinical correlations and prognosis based on serum autoantibodies in patients with systemic sclerosis. Arthritis Rheum. 1988 Feb;31(2):196–203. doi: 10.1002/art.1780310207. [DOI] [PubMed] [Google Scholar]
  30. Vayssairat M., Baudot N., Abuaf N., Johanet C. Long-term follow-up study of 164 patients with definite systemic sclerosis: classification considerations. Clin Rheumatol. 1992 Sep;11(3):356–363. doi: 10.1007/BF02207193. [DOI] [PubMed] [Google Scholar]
  31. Youssef P., Brama T., Englert H., Bertouch J. Limited scleroderma is associated with increased prevalence of macrovascular disease. J Rheumatol. 1995 Mar;22(3):469–472. [PubMed] [Google Scholar]

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