Arthritis Rheum. 2013 Jun 5

Arthritis Rheum. 2013 Jun 5. doi: 10.1002/art.38029. [Epub ahead of print]

Survival and prognostic factors in systemic sclerosis-associated pulmonary hypertension: A systematic review and meta-analysis.

Lefèvre G, Dauchet L, Hachulla E, Montani D, Sobanski V, Lambert M, Hatron PY, Humbert M, Launay D.

Université Lille Nord de France, Service de Médecine Interne, Centre National de Référence de la Sclérodermie Systémique, Hôpital Claude-Huriez, CHRU Lille, Lille, France; Université Lille Nord de France, Laboratoire d'Immunologie EA2686, IMPRT IFR 114, Faculté de Médecine, Lille, France.

Abstract

Objective: Pulmonary hypertension (PH) is a frequent and life-limiting complication of systemic sclerosis (SSc). However, there are still conflicting data on the survival rates, their evolution over time as well as on prognostic factors in this complication.
Methods: We performed a systematic review and meta-analysis of literature in MEDLINE and Embase from January 1960 to January 2012. All cohort studies reporting survival and/or prognostic factors of SSc-associated PH were analyzed. We calculated pooled survival rates, analyzed their evolution over time and determined the prognostic factors.
Results: Twenty-two studies were included corresponding to a total of 2244 patients. Pooled 1, 2 and 3-yr survival rates were 81% (95% CI: 79-84), 64% (59-69) and 52% (47-58) respectively. Meta-regression did not reveal a significant change over time while baseline hemodynamic severity of PH was significantly correlated with survival. In SSc patients with pulmonary arterial hypertension (PAH), age, male gender, DLCO, pericardial effusion and the parameters classically associated with idiopathic PAH severity, including six-minute walk test, mean pulmonary arterial pressure, cardiac index and right atrial pressure were significant prognostic factors. DLCO and pericardial effusion were the sole prognostic factors in patients with PH associated with interstitial lung disease.
Conclusions: Our meta-analysis confirms a poor pooled 3-yr survival rate of 52% (47-58) in SSc-associated PH. PAH hemodynamic baseline severity but not period of inclusion correlated significantly with survival in SSc-associated PAH. All the usual prognostic factors found in idiopathic PAH, including six-minute walk test and right atrial pressure, were also prognostic factors in SSc-associated PAH. © 2013 American College of Rheumatology.

PMID:23740572