BACKGROUND: Pleuroparenchymal fibroelastosis (PPFE) has no currently available specific treatment. Benefits of lung transplantation (LT) for PPFE are poorly documented.
METHODS: We conducted a nation-wide multicentric retrospective study in patients who underwent lung or heart-lung transplantation for chronic end-stage lung disease secondary to PPFE between 2012 and 2022 in France.
RESULTS: Thirty-one patients were included. At transplantation, median age was 48 years [IQR 35 - 55]. 64.5% were women. Twenty-one (67.7%) had idiopathic PFFE. Sixteen (52%) had bilateral LT, 10 (32%) had single LT, 4 (13%) had lobar transplantation and one (3%) had heart-lung transplantation. Operative mortality was 3.2%. Early mortality (< 90 days or during the first hospitalization) was 32%. Eleven patients (35.5%) underwent reoperation for hemostasis. Eight (30.8%) experienced bronchial complications. Mechanical ventilation time was 10 days [IQR 2-55]. Length of stay in intensive care unit and hospital were 34 [IQR 18-73] and 64 [IQR 36-103] days, respectively. Median survival was 21 months. Post-transplant survival rates after 1, 2, and 5 years were 57.9%, 42.6% and 38.3% respectively. Low albuminemia (p=0.046), FVC (p=0.021), FEV1 (p=0.009) and high emergency lung transplantation (p=0.04) were associated with increased early mortality. Oversized graft tended to be correlated to a higher mortality (p=0.07).
CONCLUSIONS: LT for PPFE is associated with high post-operative morbi-mortality rates. Patients requiring high emergency lung transplantation with advanced disease, malnutrition, or critical clinical status experienced worse outcomes.
RESULTS:
UNASSIGNED: NCT05044390.

UNASSIGNED: Given the recently expanded approval of antifibrotics for various fibrotic interstitial lung diseases (ILDs), early and correct recognition of these diseases is imperative for physicians. Because high-resolution chest CT scan forms the backbone of diagnosis for ILD, this review will discuss evidence-based imaging findings of key fibrotic ILDs and an approach for differentiating these diseases.
RESULTS: (1) Imaging findings of nonspecific interstitial pneumonia may evolve over time and become indistinguishable from usual interstitial pneumonia. Therefore, if remote imaging can be reviewed, this would increase the likelihood of an accurate imaging diagnosis, particularly if findings appear to represent a usual interstitial pneumonia pattern on the recent examination. (2) Given the difficulty and lack of objectivity in classifying patients with hypersensitivity pneumonitis into acute, subacute, and chronic categories and that prognosis depends primarily on presence or absence of fibrosis, the new set of guidelines released in 2020 categorizes patients with hypersensitivity pneumonitis as either nonfibrotic (purely inflammatory) or fibrotic (either purely fibrotic or mixed fibrotic/inflammatory) based on imaging and/or histologic findings, and the prior temporal terms are no longer used. (3) Interstitial lung abnormalities are incidental CT scan findings that may suggest early ILD in patients without clinical suspicion for ILD. Patients with high-risk features should undergo clinical evaluation for ILD and be actively monitored for disease progression.
CONCLUSIONS: Fibrotic ILD on high-resolution chest CT scan is a complex topic, but with use of an evidence-based analysis and algorithm as provided in this article, the probability of a correct imaging diagnosis increases.

OBJECTIVE: Pleuroparenchymal Fibroelastosis (PPFE) is a type of pulmonary fibrosis most commonly occurring at the apices. Patients with PPFE have an increased risk of adverse effects from lung biopsy and in the post-surgical setting. Here, we investigated simple and reproducible measurements on chest CT to evaluate their predictive value in diagnosing PPFE.
METHODS: We analyzed a cohort of patients with histologically-proven PPFE and compared them to a cohort of patients diagnosed with \"biapical scarring\" (BAS) on chest CT. We measured plueuroparenchymal thickness using several independent parameters on chest CT. We also assessed other radiologic and clinical characteristics to identify if any were predictive of PPFF.
RESULTS: Our analysis demonstrated the average greatest apical thickness with a cut off of 4.5 mm yielded a sensitivity of 94.4% and a specificity of 88.9%, and an area under the curve of 97.2%. Single greatest apical thickness with a cut off of 7.5 mm had a sensitivity of 100% and a specificity of 88.9%, with the area under the curve of 97.8%. Average greatest upper lobe thickness with a cut off of 8.0 mm had a sensitivity of 88.9% and a specificity of 100%, with an area under the curve of 98.2%. Single greatest upper lobe thickness with a cut off of 8.5 yielded both a sensitivity and specificity of 94.4% and an area under the curve of 94.3%.
CONCLUSIONS: Measurements described above are highly sensitive and specific for the diagnosis of PPFE and warrant investigation with a larger cohort of patients.

Chronic lung allograft dysfunction (CLAD) is a syndrome of progressive lung function decline, subcategorized into obstructive, restrictive, and mixed phenotypes. The trajectory of CLAD is variable depending on the phenotype, with restrictive and mixed phenotypes having more rapid progression and lower survival. The mechanisms driving CLAD development remain unclear, though allograft injury during primary graft dysfunction, acute cellular rejection, antibody-mediated rejection, and infections trigger immune responses with long-lasting effects that can lead to CLAD months or years later. Currently, retransplantation is the only effective treatment.

BACKGROUND: Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia defined by pleural and subpleural parenchymal fibrosis predominantly in the upper lobes. Although the radiological and pathological characteristics of PPFE have become increasingly recognized, its pulmonary physiological features are not well understood.
METHODS: We reviewed nine patients with radiologically and histologically proven PPFE, and evaluated pulmonary physiological data.
RESULTS: Of the nine patients, six were male and three were female. The median age at presentation was 61 years. Common symptoms were dyspnea on exertion, weight loss, and nonproductive cough. Recurrent pneumothorax was found in eight patients and pneumonia in four. Median pulmonary function test results were as follows: forced vital capacity, 55.4% predicted; total lung capacity (TLC), 67.1% predicted; residual volume (RV), 102.3% predicted; and RV/TLC, 143.6% predicted. RV/TLC was increased without evidence of small airway disease according to clinico-radiologic-pathologic evaluation. The median partial pressure of oxygen in arterial blood and the alveolar-arterial gradient of oxygen were within normal limits, although there was a slightly elevated partial pressure of carbon dioxide in arterial blood (PaCO2). PPFE progressed in all patients despite treatment with pirfenidone, corticosteroids, and immunosuppressive agents. Seven patients died during the follow-up, five because of hypercapnic respiratory failure.
CONCLUSIONS: PPFE is characterized by severe mechanical restriction with high RV/TLC, causing increased PaCO2 and eventual hypercapnic respiratory failure. These physiological findings may be useful as an adjunct in the diagnosis of PPFE.

Pleuroparenchymal fibroelastosis (PPFE) is a rare condition currently described as an upper lobe subpleural and interstitial proliferation of predominantly elastic fibers. The etiology is unknown, and no specific diagnostic criteria have been reported. Here we report 5 cases of PPFE, 1 man and 4 women, 3 of them diagnosed at the time autopsy, 1 diagnosed in an explanted lung, and 1 diagnosed on a surgical wedge biopsy. The average age of diagnosis among this series is 73 years, and the duration of pulmonary symptoms ranged from 14 months to at least 9 years. Two patients had been exposed to specific medications (daptomycin and dapsone) preceding the development of pulmonary symptoms, and 1 patient developed eosinophilic pneumonia in the course of the disease. Four patients had clinical evidence of fibrous interstitial pneumonia. We found evidence of diffuse parenchymal fibroelastosis involving both upper and lower lobes in all 5 cases, suggesting that the disease may be a more diffuse condition than previously reported. PPFE may actually represent a pattern of chronic lung injury rather than a specific entity and may be seen in association with a variety of clinicoradiologic conditions. Based on our findings in this series and the most recent publications of the subject, we propose the following set of diagnostic criteria for PPFE: multilobar subpleural and/or centrilobular fibrous interstitial pneumonia characterized by an extensive (>80%) proliferation of elastic fibers in nonatelectatic lung, along with absent to mild chronic inflammation, and absent to rare granulomas.