PULMONARY HYPERTENSION IN PATIENTS WITH IDIOPATHIC PULMONARY FIBROSIS ON ANTI-FIBROTIC TREATMENT WITH PIRFENIDONE OR NINTEDANIB: CLINICAL EVALUATION AND PROGNOSTIC IMPACT

Abstract

Introduction: Pulmonary hypertension (PH) is a common complication in idiopathic pulmonary fibrosis (IPF) and it’s strongly associated with increased morbidity and mortality.

Aim: The aim of our study was to assess the clinical and prognostic impact of PH in patients affected by IPF, already treated with pirfenidone or nintedanib.

Methods: Seventy-four consecutive outpatients with a diagnosis IPF in therapy with pirfenidone or nintedanib, were prospectively enrolled in our study. All patients underwent pulmonary and cardiology assessment by clinical exam, spirometry, DLCO test, chest CT, 6MWT and echocardiography and GAP index has been determinate. During follow-up, all patients were evaluated every six months for a 3-years follow-up.

Results: Of the seventy-four patients enrolled, were divided in four groups, based on absence or presence of mild/moderate/severe PH by echocardiography at baseline. The echocardiographic stratification revealed that the severity of PH was significantly associated with a reduction of DLCO value and with an increase of the GAP index. Survival was positively correlated (p<0.01) with the 6MWT and with the DLCO and negatively with the GAP index, while no correlation emerged with PAPs (p> 0.05).

Conclusions: The development and progression of PH in patients affected by IPF reduces the mean survival rate and the severity of PH is associated with a reduction of DLCO value and an increase of the GAP index. A practical echocardiographic stratification based on PAPs value is able to stratify the survival in IPF patients and it maybe help us in the decision making to add any specific PAH drugs.