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Systemic sclerosis related pulmonary hypertension associated with interstitial lung disease Impact of pulmonary arterial hypertension therapies



Systemic sclerosis related pulmonary hypertension associated with interstitial lung disease Impact of pulmonary arterial hypertension therapies







Precapillary pulmonary hypertension (PH) is an important cause of mortality in systemic sclerosis (SSc) where it can be isolated (pulmonary arterial hypertension [PAH]) or associated with interstitial lung disease (ILD). Importantly, outcomes and efficacy of PAH therapies in patients with PH complicating SSc-related ILD (PH-ILD) remain unknown. This study was undertaken to evaluate our experience with PH-ILD and the efficacy and safety of PAH therapies in this patient cohort. A retrospective analysis of consecutive SSc patients with PH-ILD confirmed by right heart catheterization from two large referral centers who received targeted PAH therapies was conducted. World Health Organization (WHO) functional class, 6-minute walk test (6-minWT), and hemodynamics were assessed at baseline and after 7.7 6.2 months of PAH treatment. Kaplan Meier and Cox proportional hazards models were used to analyze survival and identify prognostic factors.Seventy patients were included in the study. No significant changes were observed in functional class, 6-minWT or hemodynamic measurements after therapy. The 1-, 2- and 3-year survival estimates were 71%, 39% and 21%, respectively. In the multivariate model, worsening oxygenation during follow-up and reduced renal function were the only significant risk factors for mortality.This series represents the largest to date describing the impact of PAH therapies in SSc related PH-ILD. In this cohort, PAH therapies were associated with no clear benefits. Deterioration in oxygenation was an important determinant of long-term survival. Prospective clinical trials focusing on this group of patients are warranted.

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Accession: 036110181

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PMID: 21538327

DOI: 10.1002/art.30423


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