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

Silvia Romana Stornelli, Donato Lacedonia, Giulia Scioscia, Filomena Simone, Giorgia Lepore, Roberto Sabato, Giulio Giganti, Gianluca Ciliberti, Maria Pia Foschino Barbaro
European Respiratory Journal 2021 58: PA3746; DOI: 10.1183/13993003.congress-2021.PA3746
Silvia Romana Stornelli
1Department of Respiratory Diseases, University Hospital Policlinico Riuniti of Foggia, Foggia (Italy), Roma, Italy
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  • For correspondence: silviaromanastornelli@gmail.com
Donato Lacedonia
1Department of Respiratory Diseases, University Hospital Policlinico Riuniti of Foggia, Foggia (Italy), Roma, Italy
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Giulia Scioscia
1Department of Respiratory Diseases, University Hospital Policlinico Riuniti of Foggia, Foggia (Italy), Roma, Italy
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Filomena Simone
1Department of Respiratory Diseases, University Hospital Policlinico Riuniti of Foggia, Foggia (Italy), Roma, Italy
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Giorgia Lepore
1Department of Respiratory Diseases, University Hospital Policlinico Riuniti of Foggia, Foggia (Italy), Roma, Italy
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Roberto Sabato
1Department of Respiratory Diseases, University Hospital Policlinico Riuniti of Foggia, Foggia (Italy), Roma, Italy
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Giulio Giganti
1Department of Respiratory Diseases, University Hospital Policlinico Riuniti of Foggia, Foggia (Italy), Roma, Italy
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Gianluca Ciliberti
1Department of Respiratory Diseases, University Hospital Policlinico Riuniti of Foggia, Foggia (Italy), Roma, Italy
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Maria Pia Foschino Barbaro
1Department of Respiratory Diseases, University Hospital Policlinico Riuniti of Foggia, Foggia (Italy), Roma, Italy
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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.

  • Idiopathic pulmonary fibrosis
  • Pulmonary hypertension

Footnotes

Cite this article as: European Respiratory Journal 2021; 58: Suppl. 65, PA3746.

This abstract was presented at the 2021 ERS International Congress, in session “Prediction of exacerbations in patients with COPD”.

This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).

  • Copyright ©the authors 2021
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PULMONARY HYPERTENSION IN PATIENTS WITH IDIOPATHIC PULMONARY FIBROSIS ON ANTI-FIBROTIC TREATMENT WITH PIRFENIDONE OR NINTEDANIB: CLINICAL EVALUATION AND PROGNOSTIC IMPACT
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PULMONARY HYPERTENSION IN PATIENTS WITH IDIOPATHIC PULMONARY FIBROSIS ON ANTI-FIBROTIC TREATMENT WITH PIRFENIDONE OR NINTEDANIB: CLINICAL EVALUATION AND PROGNOSTIC IMPACT
Silvia Romana Stornelli, Donato Lacedonia, Giulia Scioscia, Filomena Simone, Giorgia Lepore, Roberto Sabato, Giulio Giganti, Gianluca Ciliberti, Maria Pia Foschino Barbaro
European Respiratory Journal Sep 2021, 58 (suppl 65) PA3746; DOI: 10.1183/13993003.congress-2021.PA3746

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PULMONARY HYPERTENSION IN PATIENTS WITH IDIOPATHIC PULMONARY FIBROSIS ON ANTI-FIBROTIC TREATMENT WITH PIRFENIDONE OR NINTEDANIB: CLINICAL EVALUATION AND PROGNOSTIC IMPACT
Silvia Romana Stornelli, Donato Lacedonia, Giulia Scioscia, Filomena Simone, Giorgia Lepore, Roberto Sabato, Giulio Giganti, Gianluca Ciliberti, Maria Pia Foschino Barbaro
European Respiratory Journal Sep 2021, 58 (suppl 65) PA3746; DOI: 10.1183/13993003.congress-2021.PA3746
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