• We are sorry, but NCBI web applications do not support your browser and may not function properly. More information
Logo of annrheumdAnnals of the Rheumatic DiseasesCurrent TOCInstructions for authors
Ann Rheum Dis. Nov 1998; 57(11): 682–686.
PMCID: PMC1752504

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


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

Full Text

The Full Text of this article is available as a PDF (115K).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.
  • LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA, Jr, Rowell N, Wollheim F. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988 Feb;15(2):202–205. [PubMed]
  • Rocco VK, Hurd ER. Scleroderma and scleroderma-like disorders. Semin Arthritis Rheum. 1986 Aug;16(1):22–69. [PubMed]
  • Lee P, Langevitz P, Alderdice CA, Aubrey M, Baer PA, Baron M, Buskila D, Dutz JP, Khostanteen I, Piper S, et al. Mortality in systemic sclerosis (scleroderma). Q J Med. 1992 Feb;82(298):139–148. [PubMed]
  • Lally EV, Jimenez SA, Kaplan SR. 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. [PubMed]
  • Silman AJ. Mortality from scleroderma in England and Wales 1968-1985. Ann Rheum Dis. 1991 Feb;50(2):95–96. [PMC free article] [PubMed]
  • Barnett AJ, Miller MH, Littlejohn GO. 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]
  • Abu-Shakra M, Lee P. Mortality in systemic sclerosis: a comparison with the general population. J Rheumatol. 1995 Nov;22(11):2100–2102. [PubMed]
  • Altman RD, Medsger TA, Jr, Bloch DA, Michel BA. Predictors of survival in systemic sclerosis (scleroderma). Arthritis Rheum. 1991 Apr;34(4):403–413. [PubMed]
  • 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. [PubMed]
  • 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]
  • 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. [PubMed]
  • Steen VD, Powell DL, Medsger TA., Jr Clinical correlations and prognosis based on serum autoantibodies in patients with systemic sclerosis. Arthritis Rheum. 1988 Feb;31(2):196–203. [PubMed]
  • Rosenthal AK, McLaughlin JK, Linet MS, Persson I. Scleroderma and malignancy: an epidemiological study. Ann Rheum Dis. 1993 Jul;52(7):531–533. [PMC free article] [PubMed]
  • Abu-Shakra M, Guillemin F, Lee P. Cancer in systemic sclerosis. Arthritis Rheum. 1993 Apr;36(4):460–464. [PubMed]
  • Radis CD, Kahl LE, Baker GL, Wasko MC, Cash JM, Gallatin A, Stolzer BL, Agarwal AK, Medsger TA, Jr, Kwoh CK. 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. [PubMed]
  • 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. [PubMed]
  • Harris EC, Barraclough BM. Suicide as an outcome for medical disorders. Medicine (Baltimore) 1994 Nov;73(6):281–296. [PubMed]
  • Medsger TA, Jr, Masi AT, Rodnan GP, Benedek TG, 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. [PubMed]
  • Kaburaki J, Lee CC, 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. [PubMed]
  • Czirják L, Nagy Z, Szegedi G. Survival analysis of 118 patients with systemic sclerosis. J Intern Med. 1993 Sep;234(3):335–337. [PubMed]
  • Geirsson AJ, Steinsson K, Guthmundsson S, Sigurthsson V. Systemic sclerosis in Iceland. A nationwide epidemiological study. Ann Rheum Dis. 1994 Aug;53(8):502–505. [PMC free article] [PubMed]
  • Chandran G, Smith M, Ahern MJ, Roberts-Thomson PJ. A study of scleroderma in South Australia: prevalence, subset characteristics and nailfold capillaroscopy. Aust N Z J Med. 1995 Dec;25(6):688–694. [PubMed]
  • 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. [PubMed]
  • Medsger TA, Jr, Masi AT. 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. [PubMed]
  • Akesson A, Scheja A, Lundin A, Wollheim FA. Improved pulmonary function in systemic sclerosis after treatment with cyclophosphamide. Arthritis Rheum. 1994 May;37(5):729–735. [PubMed]
  • Dau PC, Kahaleh MB, Sagebiel RW. Plasmapheresis and immunosuppressive drug therapy in scleroderma. Arthritis Rheum. 1981 Sep;24(9):1128–1136. [PubMed]
  • Silver RM, Warrick JH, Kinsella MB, Staudt LS, Baumann MH, 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]
  • Geirsson AJ, Blom-Bülow B, Pahlm O, Akesson A. Cardiac involvement in systemic sclerosis. Semin Arthritis Rheum. 1989 Oct;19(2):110–116. [PubMed]
  • 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]
  • Akesson A, Wollheim FA. 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. [PubMed]
  • Sakkas LI, Moore DF, Akritidis NC. Cancer in families with systemic sclerosis. Am J Med Sci. 1995 Dec;310(6):223–225. [PubMed]

Figures and Tables

Figure 1
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
Skin involvement as a determinant of survival in systemic sclerosis (p<0.0005). Differences analysed by a proportional hazards model using the Breslow test.

Articles from Annals of the Rheumatic Diseases are provided here courtesy of BMJ Group


Related citations in PubMed

See reviews...See all...

Cited by other articles in PMC

See all...


  • Compound
    PubChem Compound links
  • MedGen
    Related information in MedGen
  • PubMed
    PubMed citations for these articles
  • Substance
    PubChem Substance links

Recent Activity

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

See more...