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Mortality in systemic sclerosis—a single centre study from the UK

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Abstract

This study aims to determine the cause and predictors of mortality in a cohort of patients with systemic sclerosis (SSc) and assess whether the mortality rate differs significantly from the general population. Patients enrolled onto the Royal National Hospital for Rheumatic Diseases Connective Tissue Disease database between 1999 and 2010 were included in this study. The NHS Strategic Tracing Service and UK Registry of Births, Marriages and Deaths were used to establish date and cause of deaths. A retrospective case note review collected information on clinical phenotype and serology. A standardised mortality ratio (SMR) was calculated and survival was determined using Kaplan–Meier estimates. Univariate and multivariate predictors of survival were assessed using proportional hazards regression modelling. Amongst this cohort of 204 patients (25 males, 40 diffuse SSc), the mean age at diagnosis was 51.6 years (SD13.7) and the mean duration of follow-up was 12.5 years (SD 8.8 years). In the deceased group (53 patients), the mean age of death was 72.0 years (SD 12.3 years). The mean disease duration at death was 14.2 years (SD 8.5 years). The overall SMR was 1.34 (95 % confidence interval (CI) 1.00–1.75). The SMR was higher in males (1.54 [95 % CI 0.67–3.04] vs. 1.30 [95 % CI 0.95–1.74]). The leading causes of death in this cohort were infection, respiratory disease and malignancy. The most common cause of SSc-related mortality was pulmonary complications. Factors adversely affecting survival were older age at diagnosis, male gender, interstitial lung disease (ILD) and anti-RNA polymerase III antibody. The mortality rate of our cohort, who had predominantly limited disease, was higher than that of the general population; although not as high as reported in previous retrospective studies.

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References

  1. Black CM, Matucci-Cerinic M, Guillevin L (2009) Progress in systemic sclerosis: a 10-year perspective. Rheumatology (Oxford) 48(Suppl 3):iii1–iii2

    Article  Google Scholar 

  2. Elhai M, Meune C, Avouac J, Kahan A, Allanore Y (2012) Trends in mortality in patients with systemic sclerosis over 40 years: a systematic review and meta-analysis of cohort studies. Rheumatology (Oxford) 51(6):1017–1026

    Article  Google Scholar 

  3. Chung L, Krishnan E, Chakravarty EF (2007) Hospitalizations and mortality in systemic sclerosis: results from the Nationwide Inpatient Sample. Rheumatology (Oxford) 46(12):1808–1813

    Article  CAS  Google Scholar 

  4. Steen VD, Medsger TA (2007) Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis 66(7):940–944

    Article  PubMed  Google Scholar 

  5. Tyndall AJ, Bannert B, Vonk M, Airo P, Cozzi F, Carreira PE et al (2010) Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis 69(10):1809–1815

    Article  PubMed  Google Scholar 

  6. Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee (1980) Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 23(5):581–590

    Article  Google Scholar 

  7. LeRoy EC, Medsger TA Jr (2001) Criteria for the classification of early systemic sclerosis. J Rheumatol 28(7):1573–1576

    PubMed  CAS  Google Scholar 

  8. LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA Jr et al (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15(2):202–205

    PubMed  CAS  Google Scholar 

  9. Badesch DB, Champion HC, Sanchez MA, Hoeper MM, Loyd JE, Manes A et al (2009) Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol 54(1 Suppl):S55–S66

    Article  PubMed  Google Scholar 

  10. R Development Core Team (2011) R: A language and environment for statistical computing. Vienna: R Foundation for Statistical Computing; URL: http://www.R-project.org. Accessed 1 Apr 2012

  11. Mayes MD, Lacey JV Jr, Beebe-Dimmer J, Gillespie BW, Cooper B, Laing TJ et al (2003) Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum 48(8):2246–2255

    Article  PubMed  Google Scholar 

  12. Walker UA, Tyndall A, Czirjak L, Denton CP, Farge-Bancel D, Kowal-Bielecka O et al (2009) Geographical variation of disease manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research (EUSTAR) group database. Ann Rheum Dis 68(6):856–862

    Article  PubMed  CAS  Google Scholar 

  13. Ioannidis JP, Vlachoyiannopoulos PG, Haidich AB, Medsger TA Jr, Lucas M, Michet CJ et al (2005) Mortality in systemic sclerosis: an international meta-analysis of individual patient data. Am J Med 118(1):2–10

    Article  PubMed  Google Scholar 

  14. Hissaria P, Lester S, Hakendorf P, Woodman R, Patterson K, Hill C et al (2011) Survival in scleroderma: results from the population-based South Australian Register. Int Med J 41(5):381–390

    Article  CAS  Google Scholar 

  15. Vettori S, Cuomo G, Abignano G, Iudici M, Valentini G (2010) Survival and death causes in 251 systemic sclerosis patients from a single Italian center. Reumatismo 62(3):202–209

    PubMed  CAS  Google Scholar 

  16. Joven BE, Almodovar R, Carmona L, Carreira PE (2010) Survival, causes of death, and risk factors associated with mortality in Spanish systemic sclerosis patients: results from a single university hospital. Semin Arthritis Rheum 39(4):285–293

    Article  PubMed  Google Scholar 

  17. Bryan C, Howard Y, Brennan P, Black C, Silman A (1996) Survival following the onset of scleroderma: results from a retrospective inception cohort study of the UK patient population. Br J Rheumatol 35(11):1122–1126

    Article  PubMed  CAS  Google Scholar 

  18. Simeon CP, Armadans L, Fonollosa V, Solans R, Selva A, Villar M et al (2003) Mortality and prognostic factors in Spanish patients with systemic sclerosis. Rheumatology (Oxford) 42(1):71–75

    Article  CAS  Google Scholar 

  19. Abu-Shakra M, Lee P (1995) Mortality in systemic sclerosis: a comparison with the general population. J Rheumatol 22(11):2100–2102

    PubMed  CAS  Google Scholar 

  20. Arias-Nunez MC, Llorca J, Vazquez-Rodriguez TR, Gomez-Acebo I, Miranda-Filloy JA, Martin J et al (2008) Systemic sclerosis in north-western Spain: a 19-year epidemiologic study. Medicine (Baltimore) 87(5):272–280

    Article  Google Scholar 

  21. Geirsson AJ, Wollheim FA, Akesson A (2001) Disease severity of 100 patients with systemic sclerosis over a period of 14 years: using a modified Medsger scale. Ann Rheum Dis 60(12):1117–1122

    Article  PubMed  CAS  Google Scholar 

  22. Hesselstrand R, Scheja A, Akesson A (1998) Mortality and causes of death in a Swedish series of systemic sclerosis patients. Ann Rheum Dis 57(11):682–686

    Article  PubMed  CAS  Google Scholar 

  23. Scussel-Lonzetti L, Joyal F, Raynauld JP, Roussin A, Rich E, Goulet JR et al (2002) Predicting mortality in systemic sclerosis: analysis of a cohort of 309 French Canadian patients with emphasis on features at diagnosis as predictive factors for survival. Medicine (Baltimore) 81(2):154–167

    Article  Google Scholar 

  24. Ferri C, Valentini G, Cozzi F, Sebastiani M, Michelassi C, La Montagna G et al (2002) Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients. Medicine (Baltimore) 81(2):139–153

    Article  Google Scholar 

  25. Hashimoto A, Tejima S, Tono T, Suzuki M, Tanaka S, Matsui T et al (2011) Predictors of survival and causes of death in Japanese patients with systemic sclerosis. J Rheumatol 38(9):1931–1939

    Article  PubMed  Google Scholar 

  26. Mok CC, Kwok CL, Ho LY, Chan PT, Yip SF (2011) Life expectancy, standardized mortality ratios, and causes of death in six rheumatic diseases in Hong Kong. China Arthritis Rheum 63(5):1182–1189

    Article  CAS  Google Scholar 

  27. Jacobsen S, Halberg P, Ullman S (1998) Mortality and causes of death of 344 Danish patients with systemic sclerosis (scleroderma). Br J Rheumatol 37(7):750–755

    Article  PubMed  CAS  Google Scholar 

  28. Derk CT, Rasheed M, Artlett CM, Jimenez SA (2006) A cohort study of cancer incidence in systemic sclerosis. J Rheumatol 33(6):1113–1116

    PubMed  Google Scholar 

  29. Hill CL, Nguyen AM, Roder D, Roberts-Thomson P (2003) Risk of cancer in patients with scleroderma: a population based cohort study. Ann Rheum Dis 62(8):728–731

    Article  PubMed  CAS  Google Scholar 

  30. Steen VD, Medsger TA Jr (2000) Long-term outcomes of scleroderma renal crisis. Ann Intern Med 133(8):600–603

    Article  PubMed  CAS  Google Scholar 

  31. Steen VD (2005) Autoantibodies in systemic sclerosis. Semin Arthritis Rheum 35(1):35–42

    Article  PubMed  CAS  Google Scholar 

  32. Penn H, Howie AJ, Kingdon EJ, Bunn CC, Stratton RJ, Black CM et al (2007) Scleroderma renal crisis: patient characteristics and long-term outcomes. QJM 100(8):485–494

    Article  PubMed  CAS  Google Scholar 

  33. Jacobsen S, Ullman S, Shen GQ, Wiik A, Halberg P (2001) Influence of clinical features, serum antinuclear antibodies, and lung function on survival of patients with systemic sclerosis. J Rheumatol 28(11):2454–2459

    PubMed  CAS  Google Scholar 

  34. Perera A, Fertig N, Lucas M, Rodriguez-Reyna TS, Hu P, Steen VD et al (2007) Clinical subsets, skin thickness progression rate, and serum antibody levels in systemic sclerosis patients with anti-topoisomerase I antibody. Arthritis Rheum 56(8):2740–2746

    Article  PubMed  Google Scholar 

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Acknowledgments

Dr Gemma Strickland was funded through the Arthritis Australia Ken Muirden Overseas Training Fellowship. The authors would like to thank Paul Handley for his assistance in using the NHS Strategic Tracing Service.

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Authors and Affiliations

  1. Royal National Hospital for Rheumatic Diseases, Bath, UK

    Gemma Strickland, John Pauling & Neil McHugh

  2. Bath Institute for Rheumatic Diseases, Bath, UK

    Charlotte Cavill & Neil McHugh

  3. Department of Mathematical Sciences, University of Bath, Bath, UK

    Gavin Shaddick

  4. Department of Pharmacy and Pharmacology, University of Bath, Bath, UK

    Neil McHugh

  5. Rheumatology Department, Royal National Hospital for Rheumatic Diseases, Upper Borough Walls, Bath, BA1 1RL, UK

    Gemma Strickland

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  1. Gemma Strickland
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  2. John Pauling
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  3. Charlotte Cavill
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  4. Gavin Shaddick
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  5. Neil McHugh
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Correspondence to Gemma Strickland.

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Strickland, G., Pauling, J., Cavill, C. et al. Mortality in systemic sclerosis—a single centre study from the UK. Clin Rheumatol 32, 1533–1539 (2013). https://doi.org/10.1007/s10067-013-2289-0

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  • DOI: https://doi.org/10.1007/s10067-013-2289-0

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