Rapid decrease in forced vital capacity in patients with idiopathic pulmonary upper lobe fibrosis

A Patient with Idiopathic Pleuroparenchymal Fibroelastosis Showing a Sustained Pulmonary Function due to Treatment with Pirfenidone

“…Watanabe et al reported that the annual decline of FVC in the seven cases of IPUF was remarkable (6). In this report, five of seven patients received steroid treatment, and they conclude that steroid treatment did not appear to slow the decline in FVC.…”

“…Marked apical pleural thickening associated with superior hilar retraction is present on chest X-ray, and HRCT shows pleural thickening, fibrosis, architectural distortion, traction bronchiectasis, and honeycomb lung (7). The annual decline in the respiratory function is marked, and is similar to or more rapid than that observed for chronic fibrosing interstitial pneumonias such as UIP and fibrotic nonspecific interstitial pneumonia (6). The clinical course of this disease is progressive and almost all patients clinically and functionally deteriorate even over a relatively short follow-up period.…”

“…Kobayashi et al also reported the rapid decrease of FVC in a case of IPUF despite the steroid treat- (5). Although the use of FVC to assess the progression of IPPFE has not been validated with a large cohort or in clinical trials, we summarized the annual decline of FVC in patients with IPPFE in previous reports (3,6,(10)(11)(12) in Table 2. The data showed that the decline of FVC in the present case during treatment with pirfenidone could be small as compared to those in other cases (Table 2).…”

“…While the clinical features of IPPFE are not entirely clear because of its extreme rarity, the condition may be divisible into subgroups with or without lesions of usual interstitial pneumonia (UIP) with fibroblastic foci in the lower lobes (5). The clinical course is considered progressive, and almost all patients clinically and functionally deteriorate even over a relatively short follow-up period (5,6). The prognosis has been reported to be poor, and there are no established therapeutic options available for IPPFE except for supportive care and, ultimately, lung transplantation (7).…”

Pleuroparenchymal fibroelastosis from a consecutive database: a rare disease entity?

“…The three IPPFE cases among these four cases did not have radiotherapy, chemotherapy or inhalational injuries, which are known aetiologies of intra-alveolar fibrosis with septal elastosis. Some reports have included PPFE cases without pleural thickening [4,5,8,12,13]. Further studies are required to clarify the significance of pleural thickening in the diagnosis of PPFE.…”