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Survival and causes of death in systemic sclerosis patients: a single center registry report from Iran

  • Epidemiology of RMD
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Abstract

The aims of the study were to determine prognostic factors for survival and causes of death in a cohort of patients with systemic sclerosis (SSc). This was a cohort study of SSc patients in single rheumatologic center from January 1998 to August 2012. They fulfilled the American College of Rheumatology classification criteria for SSc or had calcinosis Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia or sine sclerosis. Causes of death were classified as SSc related and non-SSc related. Kaplan–Meier and Cox proportional hazard regression models were used in univariate and multivariate analysis to analyse survival in subgroups and determine prognostic factors of survival. The study includes 220 patients (192 female, 28 male). Out of thirty-two (14.5 %) who died, seventeen (53.1 %) deaths were SSc related and in nine (28.1 %) non-SSc-related causes, and in six (18.8 %) of patients causes of death were not defined. Overall survival rate was 92.6 % (95 % CI 87.5–95.7 %) after 5 years and 82.3 % (95 % CI 73.4–88.4 %) after 10 years. Pulmonary involvement was a major SSc-related cause of death, occurred in seven (41.1 %) patients. Cardiovascular events were leading cause of in overall death (11) 34.3 % and 6 in non-SSc-related death. Independent risk factors for mortality were age >50 at diagnosis (HR 5.10) advance pulmonary fibrosis (HR 11.5), tendon friction rub at entry (HR 6.39), arthritis (HR 3.56). In this first Middle Eastern series of SSc registry, pulmonary and cardiac involvements were the leading cause of SSc-related death.

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Authors and Affiliations

  1. Scleroderma Study Group, Firoozgar Hospital, Iran University of Medical Sciences, Tehran, Iran

    Hadi Poormoghim, Afshin Ghaderi & Ali Ghorbannia

  2. Scleroderma Study Group, Firoozgar Hospital, Tehran, Iran

    Elham Andalib

  3. Department of Epidemiology and Biostatistics, Tehran University of Medical Sciences, Tehran, Iran

    Arash Jalali

  4. Department of Immunology, Iran University of Medical Sciences, Tehran, Iran

    Nazanin Mojtabavi

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  1. Hadi Poormoghim
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  2. Elham Andalib
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  4. Afshin Ghaderi
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Correspondence to Hadi Poormoghim.

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Authors declare that they have no conflict of interest.

Ethical standard

After obtaining an informed written consent, patients data have entered Scleroderma Registry, Clinical and Research Rheumatology Information System (CRIS), which is allowed by the Medical Research Ethics Committee of the Iran University Medical Sciences, for clinical study (registration code: 76, No. P/900789).

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Poormoghim, H., Andalib, E., Jalali, A. et al. Survival and causes of death in systemic sclerosis patients: a single center registry report from Iran. Rheumatol Int 36, 925–934 (2016). https://doi.org/10.1007/s00296-016-3475-6

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  • DOI: https://doi.org/10.1007/s00296-016-3475-6

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