Pneumologie 2020; 74(S 01): 99
DOI: 10.1055/s-0039-3403286
Posterbegehung (PO20) – Sektion Klinische Pneumologie
Fortschritte bei Lungenfibrosen 2020
Georg Thieme Verlag KG Stuttgart · New York

Non-interventional study AER plus: Clinical course of mild to moderate IPF during therapy with pirfenidone

C Grohé
1   Evang. Lungenklinik Berlin
,
W Schütte
2   Hospital Martha-Maria Halle-Doelau
,
W Koerber
3   Ev. Krankenhaus Göttingen-Weende gGmbH
,
B Seese
4   Abt. Pneumologie, Thoraxzentrum
,
D Koschel
5   Fachkrankenhaus Coswig
,
K Neuland
6   Roche Pharma AG
,
J Schreiber
7   Universitätsklinikum Magdeburg
› Author Affiliations
Further Information

Publication History

Publication Date:
28 February 2020 (online)

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Background: Idiopathic pulmonary fibrosis (IPF) is characterized by chronic, progressive scarring of the lungs and if left untreated associated with high morbidity, mortality and severe symptoms. Pirfenidone was the first anti-fibrotic drug to be approved 2011 in Europe for the treatment of mild to moderate IPF.

Objective: To investigate the clinical course of mild to moderate IPF in pirfenidone-treated patients in a real-world setting.

Methods: Adult patients with mild to moderate IPF were treated with pirfenidone (escalated from 3x1 to 3x3 capsules Esbriet®/day within 3 weeks) for 12 months. At baseline (BL) and visits 1 – 4 (V1-4, month 3, 6, 9 and 12), disease progression (defined as decrease of 10% in VC or 15% in DLCO and/or ≥ 50 m in 6-minute walking distance vs. previous assessment), pulmonary function (e.g. VC, FVC, DLCO), physical resilience (6-MWT), Leicester Cough Questionnaire (LCQ) and Shortness of Breath Questionnaire (SOBQ) scores and safety were assessed.

Results: 51 patients (80.4% male, mean age 70.6 years) were included in the full analysis set. Comorbidities were documented in 15 patients (29.4%), 5 patients (9.8%) took IPF-related concomitant medication (NAC, glucocorticoids). A declining trend in pulmonary function was observed, with a mean VC, FVC and DLCO of 68.4%, 70.2% and 45.2% at BL and 67.8%, 70.7% and 44.9% at V4, respectively. Consistently, the percentage of patients with disease progression increased from 44.1% at V1 to 50.0% at V4. Only minor fluctuations were observed in LCQ and SOBQ scores. In total, 29 patients (56.9%) experienced at least one adverse drug reaction (ADR) (21.6% discontinued due to ADRs). The most common non-serious ADRs were nausea (9.8%), decreased appetite (9.8%), dizziness (9.8%), and pruritus (7.8%).

Conclusion: The results of this study are in line with the established effectiveness and safety profile of pirfenidone. Therefore, pirfenidone can be considered a valuable treatment option for mild to moderate IPF.