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Published in: Arthritis Research & Therapy 1/2021

01-12-2021 | Systemic Sclerosis | Research article

Sine scleroderma, limited cutaneous, and diffused cutaneous systemic sclerosis survival and predictors of mortality

Authors: Sébastien De Almeida Chaves, Tiphaine Porel, Mickael Mounié, Laurent Alric, Léonardo Astudillo, Antoine Huart, Olivier Lairez, Martin Michaud, Grégoire Prévot, David Ribes, Laurent Sailler, Francis Gaches, Daniel Adoue, Gregory Pugnet

Published in: Arthritis Research & Therapy | Issue 1/2021

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Abstract

Background

Systemic sclerosis (SSc) is associated with a variability of mortality rates in the literature.

Objective

To determine the mortality and its predictors in a long-term follow-up of a bi-centric cohort of SSc patients.

Methods

A retrospective observational study by systematically analyzing the medical records of patients diagnosed with SSc in Toulouse University Hospital and Ducuing Hospital. Standardized Mortality Ratio (SMR), mortality at 1, 3, 5, 10, and 15 years of disease and causes of death were described. Predictors of mortality using Cox regression were assessed.

Results

Three hundred seventy-five patients were included: 63 with diffuse cutaneous SSc, 279 with limited cutaneous SSc, and 33 with sine scleroderma. The SMR ratio was 1.88 (95% CI 1.46–1.97). The overall survival rates were 97.6% at 1 year, 93.4% at 3 years, 87.1% at 5 years, 77.9% at 10 years, and 61.3% at 15 years. Sixty-nine deaths were recorded. 46.4% were SSc related deaths secondary to interstitial lung disease (ILD) (34.4%), pulmonary hypertension (31.2%), and digestive tract involvement (18.8%). 53.6% were non-related to SSc: cardiovascular disorders (37.8%) and various infections (35.1%) largely distanced those from cancer (13.5%). Four significant independent predictive factors were identified: carbon monoxide diffusing capacity (DLCO) < 70% (HR=3.01; p=0.0053), C-reactive protein (CRP) >5 mg/l (HR=2.13; p=0.0174), cardiac involvement (HR=2.86; p=0.0012), and the fact of being male (HR=3.25; p=0.0004).

Conclusion

Long-term data confirmed high mortality of SSc. Male sex, DLCO <70%, cardiac involvement, and CRP> 5mg/l were identified as independent predictors of mortality.
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Metadata
Title
Sine scleroderma, limited cutaneous, and diffused cutaneous systemic sclerosis survival and predictors of mortality
Authors
Sébastien De Almeida Chaves
Tiphaine Porel
Mickael Mounié
Laurent Alric
Léonardo Astudillo
Antoine Huart
Olivier Lairez
Martin Michaud
Grégoire Prévot
David Ribes
Laurent Sailler
Francis Gaches
Daniel Adoue
Gregory Pugnet
Publication date
01-12-2021
Publisher
BioMed Central
Published in
Arthritis Research & Therapy / Issue 1/2021
Electronic ISSN: 1478-6362
DOI
https://doi.org/10.1186/s13075-021-02672-y

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