Survival, mortality and causes of death in inflammatory myopathies

Torres, Carmen de; Belmonte, Rebeca; Carmona, Loreto; Gómez-Reino, F; Galindo, María; Ramos, Belén; Cabello, Ana; Carreira, Patrícia

doi:10.1080/08916930600622603

Cited by 98 publications

(71 citation statements)

References 22 publications

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“…However, survival time from diagnosis was significantly worse in non-Jo-1 patients compared with Jo-1, SRP and Mi-2 positive patients, making non-Jo-1 a poor prognosis marker. Although the cumulative survival for the entire anti-synAb cohort was similar to previously published reports,14 24 25 the non-Jo-1 patients had lower 5- and 10-year unadjusted cumulative survival rates of 75% and 47%. Non-Jo-1 patients had a significant delay in diagnosis as compared with Jo-1 patients, a key variable negatively impacting their survival.…”

Section: Discussionsupporting

confidence: 86%

Patients with non-Jo-1 anti-tRNA-synthetase autoantibodies have worse survival than Jo-1 positive patients

et al. 2013

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Objective To compare the cumulative survival and event free survival in patients with Jo-1 versus non-Jo-1 anti-tRNA synthetase autoantibodies (anti-synAb). Methods Anti-synAb positive patients initially evaluated from 1985 to 2009 were included regardless of the connective tissue disease (CTD) diagnosis. Clinical data were extracted from a prospectively collected database and chart review. Survival between Jo-1 and non-Jo-1 was compared by log rank and Cox proportional hazards methods. Results 202 patients possessed anti-synAb: 122 Jo-1 and 80 non-Jo-1 (35 PL-12; 25 PL-7; 9 EJ; 6 KS; 5 OJ). The diagnoses at first visit for Jo-1 and non-Jo-1 patients were myositis in 83% and 40.0%, overlap or undifferentiated CTD in 17% and 47.5%, and systemic sclerosis in 0% and 12.5%, respectively (p<0.001). The median delay in diagnosis was 0.4 years in Jo-1 patients versus 1.0 year in non-Jo-1 patients (p<0.001). The most common causes of death in the overall cohort were pulmonary fibrosis in 49% and pulmonary hypertension in 11%. The 5- and 10-year unadjusted cumulative survival was 90% and 70% for Jo-1 patients, and 75% and 47% for non-Jo-1 patients ( p<0.005). The hazard ratio (HR) of non-Jo-1 patients compared with Jo-1 patients was 1.9 (p=0.01) for cumulative and 1.9 (p=0.008) for event free survival from diagnosis. Age at first diagnosis and diagnosis delay but not gender, ethnicity and CTD diagnosis influenced survival. Conclusions Non-Jo-1 anti-synAb positive patients have decreased survival compared with Jo-1 patients. The difference in survival may be partly attributable to a delay in diagnosis in the non-Jo-1 patients.

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Section: Discussionsupporting

confidence: 86%

Patients with non-Jo-1 anti-tRNA-synthetase autoantibodies have worse survival than Jo-1 positive patients

et al. 2013

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“…Of the mortality features from our univariable analyses, those also described in adult IIM studies included older age at illness onset (4–6, 10–13, 29), delay in diagnosis or treatment (4, 5, 10), ILD (6, 12–14), anti-synthetase autoantibodies (8, 9, 30) and dysphagia (6). Our study in JIIM did not identify other important mortality risk factors that have been seen in adult IIM, such as malignancy (4, 5, 10–13), cardiac involvement and ischemic cardiovascular disease (6, 8, 11), skin ulcers (10, 13) and male gender (6, 11, 29). The presence of anti-SRP autoantibodies has been found to be a risk factor for death by some investigators (30), but not by others (31); anti-SRP autoantibodies did not appear to increase the risk of death in JIIM in our study.…”

Section: Discussioncontrasting

confidence: 56%

“…Only 3 of these patients had an anti-aminoacyl-tRNA synthetase autoantibody, suggesting that in JIIM there are additional factors contributing to the development of pulmonary disease. In contrast, cardiac disease was a contributing factor to death in only 3 JIIM patients, although it is an important factor in adult studies (6, 11). The cardiac disease in our study is similar to that described in adult myositis studies, which includes rhythm disturbances, conduction abnormalities, pericardial effusion, left ventricular dysfunction, myocarditis, cardiomyopathy and congestive failure (6, 11).…”

Section: Discussionmentioning

confidence: 92%

Early Illness Features Associated With Mortality in the Juvenile Idiopathic Inflammatory Myopathies

Huber

Mamyrova

Lachenbruch

et al. 2014

Arthritis Care & Research

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Objectives Because juvenile idiopathic inflammatory myopathies (JIIM) are potentially life-threatening systemic autoimmune diseases, we examined risk factors for JIIM mortality. Methods Mortality status was available for 405 patients (329 juvenile dermatomyositis [JDM], 30 juvenile polymyositis [JPM], 46 juvenile connective tissue disease–associated myositis [JCTM]) enrolled in nationwide protocols. Standardized mortality ratios (SMR) were calculated using United States population statistics. Cox regression was used to assess univariable associations with mortality, and random survival forest (RSF) classification and Cox regression for multivariable associations. Results Of 17 deaths (4.2% overall mortality), 8 (2.4%) were in JDM patients. Death was related to the pulmonary system, primarily interstitial lung disease (ILD), in 7 patients, gastrointestinal in 3, multisystem in 3, and of unknown etiology in 4 patients. The SMR for JIIM overall was 14.4 [95% confidence interval (CI) 12.2, 16.5] and 8.3 [95% CI 6.4, 10.3] for JDM. The top mortality risk factors in the univariable analysis included clinical subgroup (JCTM, JPM), anti-synthetase autoantibodies, older age at diagnosis, ILD and Raynaud’s phenomenon at diagnosis. In multivariable analyses, clinical subgroup, illness severity at onset, age at diagnosis, weight loss and delay to diagnosis were the most important predictors from RSF; clinical subgroup and illness severity at onset were confirmed by multivariable Cox regression. Conclusions Overall mortality was higher in JIIM patients, and several early illness features were identified as risk factors. Clinical subgroup, anti-synthetase autoantibodies, older age at diagnosis, and ILD are also recognized as mortality risk factors in adult myositis.

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“…Later work have confirmed this, but shown large variation in mortality rates, probably due to differences in patient selection, classification, loss to follow-up and treatment-related issues [4,5]. Peter and Bohan criteria were applied in eight studies on mortality [3,4,[24][25][26][27][28][29] but only one of them assessed an unselected population-based cohort [3]. This study, which is the largest mortality study to date, identified 248 PM/DM cases diagnosed in Finland from 1969 to 1985 through hospital discharge searches and subsequent case assignment by chart review.…”

Section: Introductionmentioning

confidence: 89%

Survival and cancer risk in an unselected and complete Norwegian idiopathic inflammatory myopathy cohort

Dobloug

Garen

Brunborg

et al. 2015

Seminars in Arthritis and Rheumatism

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Survival, mortality and causes of death in inflammatory myopathies

Cited by 98 publications

References 22 publications

Patients with non-Jo-1 anti-tRNA-synthetase autoantibodies have worse survival than Jo-1 positive patients

Patients with non-Jo-1 anti-tRNA-synthetase autoantibodies have worse survival than Jo-1 positive patients

Early Illness Features Associated With Mortality in the Juvenile Idiopathic Inflammatory Myopathies

Survival and cancer risk in an unselected and complete Norwegian idiopathic inflammatory myopathy cohort

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