Possible Value of Antifibrotic Drugs in Patients With Progressive Fibrosing Non-IPF Interstitial Lung Diseases

Sebastiano Emanuele Torrisi; Nicolas Kahn; Julia Wälscher; Nilab Sarmand; Markus Polke; Kehler Lars; Monika Eichinger; Claus Peter Heussel; Stefano Palmucci; Francesca Maria Sambataro; Gianluca Sambataro; Domenico Sambataro; Carlo Vancheri; Michael Kreuter


BMC Pulm Med. 2019;19(213) 

In This Article


Interstitial lung diseases comprise a heterogeneous group of almost 200 entities characterized by a different amount of inflammation and/or fibrosis.[1,2] Idiopathic Pulmonary Fibrosis (IPF) is the most frequent and aggressive form, representing the prototype of progressive fibrosing interstitial lung diseases.[3,4] However, recent evidence has shown that also other non-IPF fibrosing interstitial lung diseases may, similarly to IPF, reveal a progressive phenotype (PF-ILDs) characterized by a rapid functional decline, worsening of symptoms and a detrimental prognosis.[5–9] According to a recent study by Olson et al., the prevalence of this subset of patients has been estimated at 0.22–2 per 10,000 persons in Europe and 2.8 per 10,000 persons in USA, thus representing a considerable number.[10]

Therapy of PF-ILDs is currently based on corticosteroids and/or immunomodulators. However, response to these therapies is highly variable, sometimes without meaningful improvement.[11] In recent years two antifibrotic drugs, pirfenidone and nintedanib, have been developed and approved for the treatment of IPF.[12–17] Their impact on the course of other fibrosing ILDs is unknown. However, given some pathobiological and clinical similarities between PF-ILDs and IPF, both pirfenidone and nintedanib, may represent an interesting and reasonable approach also for PF-ILDs.[18–21]

We here report our experience with antifibrotic drugs in fibrosing non-IPF ILDs patients with a progressive phenotype despite immunosuppressive therapy.