Prevalence of Pulmonary Hypertension in Systemic Sclerosis in European Caucasians and Metaanalysis of 5 Studies

• Published in: Journal of rheumatology Vol. 37; no. 11; pp. 2290 - 2298
• Main Authors: AVOUAC, Jérôme, AIRO, Paolo, CRACOWSKI, Jean-Luc, SIBILIA, Jean, KAHAN, André, MATUCCI-CERINIC, Marco, ALLANORE, Yannick, MEUNE, Christophe, BERETTA, Lorenzo, DIEUDE, Philippe, CARAMASCHI, Paola, TIEV, Kiet, CAPPELLI, Susanna, DIOT, Elisabeth, VACCA, Alessandra
• Format: Journal Article
• Language: English
• Published: Toronto, ON Journal of Rheumatology Publishing 01-11-2010
• Subjects: Age Factors; Aged; Biological and medical sciences; Cardiac Catheterization; Comorbidity; Diseases of the osteoarticular system; Echocardiography, Doppler; European Continental Ancestry Group; Female; Humans; Hypertension, Pulmonary - diagnosis; Hypertension, Pulmonary - epidemiology; Logistic Models; Male; Medical sciences; Middle Aged; Pneumology; Prevalence; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Scleroderma, Systemic - epidemiology; Severity of Illness Index; Immunopathology; Connective tissue disease; Skin disease; Prevalence; Catheterization; Respiratory disease; SYSTEMIC SCLEROSIS; Rheumatology; Autoimmune disease; Cardiovascular disease; Epidemiology; Pulmonary hypertension; Metaanalysis; Systemic disease; Scleroderma
• ISSN: 0315-162X; 1499-2752
• DOI: 10.3899/jrheum.100245

To measure the prevalence of different types of pulmonary hypertension (PH) and to identify patients with systemic sclerosis (SSc) at highest risk in a multicenter European sample, with a metaanalysis of relevant studies.
Consecutive patients with SSc recruited at 11 French and Italian centers underwent detailed evaluations, including Doppler echocardiography, chest computed tomography, pulmonary function tests, and right-heart catheterization (RHC), to detect the presence and causes of PH. A metaanalysis was performed, including data from 4 other studies.
Among 206 patients in whom it was suspected, PH was confirmed by RHC in 83 patients (7%). Precapillary PH was found in 64 patients (5%), of whom 42 had pulmonary arterial hypertension (PAH) and 22 had PH secondary to interstitial lung disease (ILD). RHC identified 17 patients (1%) with postcapillary PH secondary to left-heart disease. Patients with DLCO/alveolar volume < 70% were more likely to have precapillary PH (87.5% vs 42%; p < 0.0001). Precapillary and postcapillary PH were associated with advanced age (68 ± 14 vs 59 ± 12 yrs, p < 0.0001, and 74 ± 16 vs 61.5 ± 10 yrs, p < 0.0001, respectively). The metaanalysis of 3818 patients showed a prevalence of precapillary PH of 9% (95% CI 6%-12%) and identified advanced age, longer disease duration, and limited cutaneous disease subset as risk factors for this condition.
The prevalence of precapillary PH in our multicenter study of SSc was 5%, and in the metaanalysis 9%. Our observations support use of RHC to confirm the presence of precapillary PH suspected by noninvasive testing. We also identified patients at high risk who should be carefully monitored.