title: Survival and causes of death in systemic sclerosis patients: a single center registry report from Iran.
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.
authors.affiliation: Scleroderma Study Group, Firoozgar Hospital, Iran University of Medical Sciences, Tehran, Iran. poormoghim.h@iums.ac.ir.
authors.forename: Hadi
authors.initials: H
authors.lastname: Poormoghim
authors.affiliation: Scleroderma Study Group, Firoozgar Hospital, Tehran, Iran.
authors.forename: Elham
authors.initials: E
authors.lastname: Andalib
authors.affiliation: Department of Epidemiology and Biostatistics, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Arash
authors.initials: A
authors.lastname: Jalali
authors.affiliation: Scleroderma Study Group, Firoozgar Hospital, Iran University of Medical Sciences, Tehran, Iran.
authors.forename: Afshin
authors.initials: A
authors.lastname: Ghaderi
authors.affiliation: Scleroderma Study Group, Firoozgar Hospital, Iran University of Medical Sciences, Tehran, Iran.
authors.forename: Ali
authors.initials: A
authors.lastname: Ghorbannia
authors.affiliation: Department of Immunology, Iran University of Medical Sciences, Tehran, Iran.
authors.forename: Nazanin
authors.initials: N
authors.lastname: Mojtabavi
mesh_terms: D000328:Adult; D002423:Cause of Death; D016009:Chi-Square Distribution; D005260:Female; D006331:Heart Diseases / Q000175:diagnosis / Q000401:mortality*; D006801:Humans; D007492:Iran / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D008171:Lung Diseases / Q000175:diagnosis / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011379:Prognosis; D016016:Proportional Hazards Models; D012042:Registries; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000175:diagnosis / Q000401:mortality*; D013997:Time Factors; D055815:Young Adult
keywords: Causes of death; Survival; Systemic sclerosis