Treatment with antifibrotic agents in idiopathic pleuroparenchymal fibroelastosis with usual interstitial pneumonia

Abstract

BackgroundThere are no established therapeutic options available for idiopathic pleuroparenchymal fibroelastosis (IPPFE), apart from supportive care and lung transplantation. Furthermore, it is known that IPPFE with a usual interstitial pneumonia (UIP) pattern and lower lobe predominance is a disease entity distinct from idiopathic pulmonary fibrosis (IPF). To our knowledge, few studies are available that report on the efficacy of antifibrotic agents for IPPFE with UIP.AimThe aim of this study was to compare the efficacy of antifibrotic agents between IPPFE with UIP and typical IPF in real-world clinical practice.Patients and MethodsA retrospective analysis was performed on the medical records of all patients at 2 interstitial lung disease centres. Sixty-four patients were diagnosed as having IPPFE with UIP and 195 patients were diagnosed with typical IPF. We compared the efficacy of antifibrotic agents between these 2 groups.ResultsSurvival time was significantly shorter in the patients with IPPFE with UIP. Some 125 patients were administered antifibrotic agents for over 6 months (34 with IPPFE with UIP and 91 with typical IPF). Reduced forced vital capacity (FVC) 6 months after treatment with antifibrotic agents was significantly greater in the IPPFE with UIP group than in those in the typical IPF. Moreover, the change in FVC% predicted was significantly greater during the follow-up in patients with IPPFE with UIP compared with those with typical IPF.ConclusionsThe efficacy of antifibrotic agents was limited in patients with IPPFE with UIP. Thus, IPPFE with UIP remains a fatal and progressive disease.

Effects of pulmonary rehabilitation therapy combined with conventional drugs on BODE and pulmonary function indexes in elderly patients with interstitial pneumonia

“…Conventional drugs mainly include prednisone+azathioprine+acetylcysteine which can inhibit the inflammatory reaction, dissolve and promote the expectoration of sputum. 13,14 Based on the comprehensive assessment of the patient's condition, pulmonary rehabilitation therapy may additionally provide protein supplementation, oxygen inhalation, exercise and respiratory training guidance and psychological intervention. Protein supplementation can promote the improvement of immune function, while oxygen inhalation can improve the symptoms of dyspnea.…”

A proposed prognostic prediction score for pleuroparenchymal fibroelastosis

“…However, the impact of the drug is negligible because the use of nintedanib for progressive fibrosing ILD was started in Japan in 2020. In addition, a retrospective study showed that the efficacy of antifibrotic agents was limited in PPFE patients with UIP [ 42 ]. Third, we have recently reported that high serum LTBP-4 levels may be associated with a poor prognosis in PPFE patients [ 22 ]; however, serum LTBP-4 was not measured in the present study.…”

Pleuroparenchymal fibroelastosis

“…Patients often receive low-dose glucocorticoids, however, immunosuppressive therapy is generally discouraged [54]. The antifibrotic agents pirfenidone (not approved for this indication) [7 ▪ ,12 ▪ ,21,71] and nintedanib [44 ▪▪ ] have been used is few cases, and interpretation of data is often confounded by the association of PPFE and UIP patterns [24]. In the INBUILD study of nintedanib in patients with ILD and a progressive fibrosing phenotype (progressive pulmonary fibrosis) [72], a low number of patients with PPFE were enrolled [73].…”