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Observational Study
. 2021 Dec 7;23(1):295.
doi: 10.1186/s13075-021-02672-y.

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

Sébastien De Almeida Chaves  1 , Tiphaine Porel  2 , Mickael Mounié  3 , Laurent Alric  2   4 , Léonardo Astudillo  5 , Antoine Huart  6 , Olivier Lairez  4   7 , Martin Michaud  8 , Grégoire Prévot  9 , David Ribes  6 , Laurent Sailler  2   4 , Francis Gaches  8 , Daniel Adoue  2   4 , Gregory Pugnet  2   4
Affiliations
Observational Study

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

Sébastien De Almeida Chaves et al. Arthritis Res Ther. .

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.

Keywords: Autoimmune diseases; Mortality; Prognostic factors; Systemic sclerosis.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Flow chart showing selecting patients from Systemic Scleroderma Toulouse Cohort
Fig. 2
Fig. 2
Kaplan-Meier analysis of overall survival following disease onset in the Systemic Scleroderma Toulouse Cohort according to SSc sub-types
Fig. 3
Fig. 3
Primary causes of deaths in Systemic Scleroderma Toulouse Cohort. A Percentages of causes of death related to systemic sclerosis. B Percentages of causes of death non-related to systemic sclerosis. The bars in bold correspond to the groups of causes of death and in the same color are represented the different etiologies. MI myocardial infarction, PH pulmonary hypertension, SSc systemic sclerosis, UTI urinary tract infection
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References

    1. Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis. 2007;66:940–944. doi: 10.1136/ard.2006.066068. - DOI - PMC - PubMed
    1. Bussone G, Bérezné A, Pestre V, Guillevin L, Mouthon L. The scleroderma kidney: progress in risk factors, therapy, and prevention. Curr Rheumatol Rep. 2011;13:37–43. doi: 10.1007/s11926-010-0145-7. - DOI - PubMed
    1. Rubio-Rivas M, Simeón-Aznar CP, Velasco C, Marí-Alfonso B, Espinosa G, Corbella X, et al. Changes in the pattern of death of 987 patients with systemic sclerosis from 1990 to 2009 from the nationwide Spanish Scleroderma Registry (RESCLE) Clin Exp Rheumatol. 2017;35(Suppl 106):40–47. - PubMed
    1. Scussel-Lonzetti L, Joyal F, Raynauld J-P, Roussin A, Rich E, Goulet J-R, et al. 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. 2002;81:154–167. doi: 10.1097/00005792-200203000-00005. - DOI - PubMed
    1. Hissaria P, Lester S, Hakendorf P, Woodman R, Patterson K, Hill C, et al. Survival in scleroderma: results from the population-based South Australian Register. Intern Med J. 2011;41:381–390. doi: 10.1111/j.1445-5994.2010.02281.x. - DOI - PubMed

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