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

Sato, Seidai; Hashimoto, Masaki; Takahashi, Mikiko; Fukuda, Yuh; Morizumi, Shun; Toyoda, Yuko; Goto, Hisatsugu; Nishioka, Yasuhiko

doi:10.2169/internalmedicine.55.5047

Cited by 32 publications

(17 citation statements)

References 12 publications

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“…These treatments were not previously considered as effective for IPPFE patients who reportedly have similar characteristics [ 17 ]. However, pirfenidone was effective for treating IPPFE combined with UIP in the lower lobes [ 20 ]. Pirfenidone may be an effective drug for IPPFE patients with a UIP lesion because it reduces the progression of fibrotic changes.…”

Section: Discussionmentioning

confidence: 99%

Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage

Shioya

Otsuka

Yamada

et al. 2018

Canadian Respiratory Journal

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Objective Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disease characterized by predominant upper lobe pulmonary fibrosis of unknown etiology. However, the prognosis of IPPFE has not been discussed. We investigated the clinical characteristics and prognostic factors of IPPFE and idiopathic pulmonary fibrosis (IPF). Methods We performed a retrospective cohort study on 375 consecutive idiopathic interstitial pneumonia patients between April 2004 and December 2014. Among them, we diagnosed IPPFE and IPF patients using high-resolution computed tomography radiological criteria. Results Twenty-nine IPPFE patients (9 males, 20 females) and 67 IPF patients (54 males, 13 females) were enrolled. IPPFE patients were significantly more likely to be females and nonsmokers and had lower body mass index, lower values of predicted percentage of forced vital capacity (%FVC), and a higher residual volume-to-total lung capacity ratio than IPF patients. Survival analysis revealed that they had significantly poorer prognosis than IPF patients in GAP (gender, age, and physiology) stages II + III. %FVC and GAP index independently predict mortality in patients with IPPFE. Conclusions Patients with IPPFE showed poorer prognosis in the advanced stage than patients with IPF. %FVC and GAP index are independent predictors of survival in patients with IPPFE.

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Section: Discussionmentioning

confidence: 99%

Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage

Shioya

Otsuka

Yamada

et al. 2018

Canadian Respiratory Journal

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Section: The Clinical Spectrummentioning

confidence: 99%

“…Patients have been empirically treated with corticosteroids, immunosuppressive agents, and N-acetyl cysteine, with transient or no benefit [28]. A potential efficacy of pirfenidone in preventing lung function decline has been suggested in a recent case-report [28]. An interesting temporal relationship was observed in this patient between lung function decline and pirfenidone intake, as the disease stabilized while taking the drug, progressed after drug discontinuation due to liver toxicity, and then stabilized again once pirfenidone was reintroduced.…”

Section: The Clinical Spectrummentioning

confidence: 99%

Idiopathic Pleuroparenchymal Fibroelastosis

2017

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Purpose of the reviewIdiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare fibrosing lung disease, affecting the visceral pleura and the subpleural parenchyma with an upper lobe predilection, included as a distinct clinicopathologic entity in the latest international multidisciplinary classification of the idiopathic interstitial pneumonias (IIP). We aim to summarize the current evidence on IPPFE, in terms of clinical features and potential treatments.Recent findingsOverall, there is increasing awareness of PPFE in association with a separate ILD pattern. Although an agreed consensus on diagnosis has yet to be defined, a list of radiological and histopathological criteria has been proposed. Due to the unfavorable risk-benefit profile of surgical lung biopsy in a significant proportion of patients, a potential role for transbronchial lung cryobiopsy has been suggested. At present, lung transplantation remains the only curative option.SummaryThe increasing awareness of this condition among specialists has led to more frequent identification of IPPFE. Large international studies are needed to better characterize pathogenesis and pheno/endotypes of disease, a key step towards the development of effective treatments.

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“…The median follow-up was 10 months 22. Sato et al reported benefit from pirfenidone, however, this has not been corroborated in other reports 23 24. Other authors have also reported disease progression on steroids, cyclosporine and cyclophosphamide 24 25…”

Section: Discussionmentioning

confidence: 94%

Pleuroparenchymal fibroelastosis (PPFE) treated with lung transplantation and review of the literature

Ali

Ramalingam²,

Haasler

et al. 2019

BMJ Case Rep

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A 26-year-old woman presented with a 15-year history of non-progressive dyspnoea. Chest imaging showed bilateral apical pleural and parenchymal scarring, pleural thickening and bronchiectasis. Pulmonary function tests showed a moderate restrictive defect. Non-invasive workup was non-revealing; therefore, the patient was referred for video-assisted thoracic surgery and lung biopsy. Histopathology revealed pleural thickening and, subpleural parenchymal fibrosis and elastic tissue deposition. Lung parenchyma further away from the pleura was well preserved. Based on these findings, the patient was diagnosed with pleuroparenchymal fibroelastosis (PPFE). Since PPFE is a progressive disorder without effective medical therapies, and given our patient’s worsening symptoms, she underwent bilateral lung transplantation. It has been almost 4 years since the lung transplantation, our patient continues to do well. To the best of our knowledge, to date, this is the longest follow-up reported for a PPFE patient undergoing lung transplantation.

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

Cited by 32 publications

References 12 publications

Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage

Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage

Idiopathic Pleuroparenchymal Fibroelastosis

Pleuroparenchymal fibroelastosis (PPFE) treated with lung transplantation and review of the literature

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