Objectives. This longitudinal study investigated survival, risk factors and causes of death in the multicentre ItinérAIR-Sclérodermie cohort of patients with SSc without severe pulmonary fibrosis or severe left heart disease at baseline

A. Chabrol

Clements

D. Launay

Derk

E. Diot

E. Hachulla

Ferri

Gladwin

Hachulla

Hill

I. Lazareth

J. Cabane

J. Sibilia

L. Guillevin

L. Mouthon

LeRoy

M. Humbert

P. Carpentier

P. Clerson

P. de Groote

P. Jego

Silman

Simeon

Task Force members

V. Gressin

Y. Allanore

English

PubMed

Crossref

Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinerAIR-Sclerodermie study

International audienceOBJECTIVES: This longitudinal study investigated survival, risk factors and causes of death in the multicentre ItinérAIR-Sclérodermie cohort of patients with SSc without severe pulmonary fibrosis or severe left heart disease at baseline. METHODS: At 3-year follow-up, vital status was obtained from investigators or French national death records. Causes of death were classified as SSc-related or otherwise. Data were censored at 37 months, time of death or loss to follow-up, whichever was earlier. Survival was estimated using the Kaplan-Meier method. Multivariate survival analyses were conducted using the Cox model. RESULTS: In total, 546 patients were followed for a median duration of 37 months, representing 1547 patient-years. At baseline, the majority of patients were female, with lcSSc, mean age 54.9 +/- 13.0 years and mean duration of SSc of 8.8 +/- 8.1 years. In total, 47 patients died, giving a 3-year survival of 91.1% and cumulative mortality of 3.04 deaths per 100 patient-years; 17 deaths (32.2%) resulted from pulmonary arterial hypertension (PAH) and eight (17.1%) from cancer. Of the 47 patients with PAH at baseline, 20 died during follow-up, giving a 3-year survival of 56.3%. In a multivariate analysis, PAH [hazard ratio (HR) 7.246], age at first symptom (HR 1.052), duration of SSc (HR 1.047 per year) and Rodnan skin score (per one point) (HR 1.045) were associated with increased mortality. CONCLUSION: This 3-year study observed survival and mortality estimates that were comparable with previous reports. PAH increased the HR for mortality in patients with SSc, justifying yearly echocardiographic screening

Hachulla, Eric

Carpentier, Patrick, H.

Gressin, Virginie

Diot, Elisabeth

Allanore, Yannick

Sibilia, Jean

Launay, David

Mouthon, Luc

Jego, Patrick

Cabane, Jean

de Groote, Pascal

Chabrol, Amélie

Lazareth, Isabelle

Guillevin, Loïc

Clerson, Pierre

Humbert, Marc

Pison, Christophe, M.

HAL-Inserm

Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinérAIR-Sclérodermie study.

Hal - Université Grenoble Alpes

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Ann Rheum Dis

Changes in causes of death in systemic sclerosis,

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Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The task force on diagnosis and treatment of pulmonary arterial hypertension of the European Society of Cardiology.

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Scleroderma and survival: a review. Ann Rheum Dis

Society Guidelines and Medical Practice Committee recommendations on the management of pulmonary hypertension in clinical practice.

Subcommittee For Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 1980;23:

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Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinérAIR-Sclérodermie study

Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinérAIR-Sclérodermie study

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Hal - Université Grenoble Alpes