Pulmonary arterial hypertension is a major mortality factor in diffuse systemic sclerosis, independent of interstitial lung disease.

Details

Serval ID
serval:BIB_2D4FC0178219
Type
Article: article from journal or magazin.
Collection
Publications
Title
Pulmonary arterial hypertension is a major mortality factor in diffuse systemic sclerosis, independent of interstitial lung disease.
Journal
Arthritis and Rheumatism
Author(s)
Trad S., Amoura Z., Beigelman C., Haroche J., Costedoat N., Boutin le TH, Cacoub P., Frances C., Wechsler B., Grenier P., Piette J.C.
ISSN
0004-3591 (Print)
ISSN-L
0004-3591
Publication state
Published
Issued date
2006
Volume
54
Number
1
Pages
184-191
Language
english
Abstract
OBJECTIVE: To determine whether pulmonary arterial hypertension (PAH) is a prognostic factor for mortality in diffuse cutaneous systemic sclerosis (dcSSc), independent of interstitial lung disease (ILD). METHODS: ILD was diagnosed by high-resolution computed tomography and PAH (pulmonary arterial systolic pressure [PASP] > or =45 mm Hg) by echocardiography. All patients with ILD underwent testing for total lung capacity (TLC), forced vital capacity (FVC), and diffusing capacity for carbon monoxide. RESULTS: Eighty-six patients with dcSSc (mean age at diagnosis 44.5 years) were followed up for a median of 72.5 months. ILD was found in 52 patients (60%) and PAH in 18 (21%). ILD was associated with PAH in 15 patients. Seventeen patients died (19.8%), 9 of whom had PAH (P = 0.001) and 10 of whom had ILD (P = 0.99). By multivariate analysis, age at SSc diagnosis and PAH were the only independent predictors of death (hazard ratio [HR] 1.057, 95% confidence interval [95% CI] 1.009-1.109, P = 0.020 and HR 4.09, 95% CI 1.47-11.5, P = 0.007, respectively). Mean TLC and mean FVC were similar in ILD patients with and those without PAH (P = 0.71 and P = 0.40, respectively). Among ILD patients, age at SSc diagnosis and PAH were again the sole predictors of death (HR 1.073, 95% CI 1.003-1.149, P = 0.042 and HR 5.07, 95% CI 1.09-23.8, P = 0.038, respectively). Twenty ILD patients received at least 6 monthly pulses of intravenous cyclophosphamide (CYC). In CYC-treated patients with PAH (n = 8), PASP increased significantly during the CYC regimen (mean +/- SD 55 +/- 14.5 mm Hg; P = 0.015 versus baseline), while TLC remained stable during the same period. CONCLUSION: These results indicate that, independent of ILD, PAH is a major prognostic factor for survival in dcSSc.
Keywords
Adult, Female, Humans, Hypertension, Pulmonary/complications, Hypertension, Pulmonary/mortality, Lung Diseases, Interstitial/complications, Male, Prognosis, Retrospective Studies, Risk Factors, Scleroderma, Diffuse/complications
Pubmed
Create date
31/08/2011 12:46
Last modification date
20/08/2019 13:12
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