Antifibrotic treatment after multidisciplinary team discussion and with patient consent may be a valuable treatment option in patients with progressive fibrosing non-IPF ILDs that do not benefit from corticosteroids and immunosuppressive treatments if no other treatment options exist. However, prospective, randomized clinical trials are urgently needed to assess the real impact of antifibrotic therapy in these patients.
BAL: Bronchoalveolar lavage; DLCO: Diffusing capacity of the lung for carbon monoxide; FVC: Forced vital capacity; HRCT: High resolution computed tomography; IPAF: Interstitial pneumonia with autoimmune features; IPF: Idiopathic Pulmonary Fibrosis; MDT: Multidisciplinary team; PFILDs: Progressive fibrosing non-IPF interstitial lung diseases; PPFE: Pleuroparenchymal fibroelastosis; SSc-ILD: Systemic sclerosis associated-ILD
Availability of data and materials
The data analyzed in the current study are not publicly available but may be made available from the corresponding authors on reasonable request.
Ethics approval and consent to participate
The ethics committee of the University of Heidelberg approved this retrospective study. Due to the retrospective nature of this analysis and according to the vote of the ethics committee, written informed consent could not be obtained by the patients but patient records/information were anonymized and de-identified prior to analysis.
Consent for publication
BMC Pulm Med. 2019;19(213) © 2019 BioMed Central, Ltd.