Background Physicians have few opportunities to perform surgical lung biopsy (SLB) to diagnose idiopathic pleuroparenchymal fibroelastosis (IPPFE). Therefore, diagnostic criteria for IPPFE that do not require SLB must be established. Herein, we propose diagnostic criteria for IPPFE with and without SLB. Methods and results The diagnostic criteria for IPPFE with SLB are histological, based on computed tomography (CT) lesions compatible with PPFE, predominantly in the upper lobes. The three diagnostic criteria for IPPFE without SLB are as follows: (1) radiologically possible IPPFE (a radiological criterion confirming CT lesions in both lung apexes, regardless of the lower lobe lesions); (2) radiologically probable IPPFE (also a radiological criterion, but mandatory to confirm chest radiograph findings of bilateral upward shift of the hilar structures and/or CT findings of volume loss of the upper lobes); (3) radiologically and physiologically probable IPPFE. Our data from 41 patients with IPPFE and 97 with idiopathic pulmonary fibrosis (IPF) showed that the percentage of the predicted values of the ratio of residual volume to total lung capacity (RV/TLC %pred.) ≥115% and body mass index (BMI) ≤20 kg/m2 plus RV/TLC %pred. ≥80% performed well for discriminating IPPFE from IPF. These parameters were thus added to criterion (3). Conclusions We have proposed diagnostic criteria for IPPFE in patients with and without SLB. Both imaging criteria and physiological criteria using RV/TLC and BMI successfully discriminate IPPFE from chronic IIPs when SLB cannot be performed.

Criteria for the diagnosis of idiopathic pleuroparenchymal fibroelastosis: A proposal.

Pleuroparenchymal fibroelastosis diagnosed by multidisciplinary discussions in Japan.

Functional associations of pleuroparenchymal fibroelastosis and emphysema with hypersensitivity pneumonitis.

Idiopathic 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. Overall, 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. The 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.

/pdf/idiopathic-pleuroparenchymal-fibroelastosis-48x1s29ifq.pdf

Idiopathic Pleuroparenchymal Fibroelastosis.

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.

/pdf/poorer-prognosis-of-idiopathic-pleuroparenchymal-s2u716c8zi.pdf

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

Idiopathic pleuroparenchymal fibroelastosis is an acceptable entity among IIPs and not as rare as previously reported http://ow.ly/I5NEz

https://erj.ersjournals.com/content/erj/45/4/1183.full.pdf

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

Malignant pleural mesothelioma (MPM) is an uncommon, fatal neoplasm induced by asbestos exposure. Brain metastases from MPM are extremely rare, with most such cases diagnosed only at the time of autopsy. This report describes what we believe to be the first case of MPM metastasizing to the brain after a subarachnoid hemorrhage, as well as the subsequent surgical removal of the brain metastasis.

Brain Metastasis of Pleural Mesothelioma after a Subarachnoid Hemorrhage.

Background

A previous study showed a survival benefit with maintenance therapy with pemetrexed in patients with advanced non-small cell lung cancer (NSCLC). However, it remains unclear whether continuation maintenance therapy with pemetrexed is beneficial in Japanese patients. Here, we present our phase II study that assessed the efficacy and safety of cisplatin plus pemetrexed as induction chemotherapy, followed by maintenance therapy with pemetrexed in advanced NSCLC patients in Japan.



Methods

Chemotherapy-naive patients received 500 mg/m2 pemetrexed and 75 mg/m2 cisplatin on day one every three weeks for four cycles. In patients who responded to therapy or achieved stable disease, pemetrexed was continued until disease progression. The primary endpoint of this study was the progression-free survival rate at six months (PFS-6).



Results

Of the 35 patients initially enrolled in the study, 18 (51%) received maintenance chemotherapy with pemetrexed. The median PFS was 6.7 months, and the PFS-6 was 60% (95% confidence interval [CI], 42–76%). Median overall survival (OS) was 15.5 months (95% CI, 8.3–22.7 months). The median PFS and OS in patients who received maintenance chemotherapy with pemetrexed were 9.5 months and 25.3 months, respectively. The most frequently noted severe toxicity during induction chemotherapy was neutropenia, which occurred in seven patients. Two patients discontinued maintenance therapy owing to prolonged grade 2 edema in one patient and grade 3 neutropenia in another.



Conclusion

Continuation maintenance chemotherapy with pemetrexed is associated with a survival benefit in patients who have completed induction chemotherapy for non-squamous NSCLC.

/pdf/prospective-phase-ii-study-of-cisplatin-plus-pemetrexed-with-g76hge57th.pdf

Prospective phase II study of cisplatin plus pemetrexed with maintenance of pemetrexed for advanced non-squamous cell non-small cell lung cancer in Japan

Introduction: Lymphangioleiomyomatosis (LAM) is a rare multisystem disease, caused by the proliferation of LAM cells leading to destruction and remodeling of the tissues. Renin–angiotensin system and mast cells have been reported to function on cell proliferation and tissue remodeling in LAM (AJRCMB 2006, AJRCCM 2002, Respir Res 2012). Aim of this study is to clarify the role of measurement of serum angiotensin-converting enzyme (ACE) activity as a biomarker for LAM. Subjects and methods: 102 patients of LAM including 15 tuberlous sclerosis complex (TSC)-LAM, 37 healthy volunteers (HV) and 16 other lung disease controls (OLD) were enrolled. Serum ACE activities were measured by Kasahara9s method. Serum VEGF-D levels and clinical measures were compared with serum ACE. Results: Serum ACE significantly elevated in LAM {14.6 (11.7-19.1) median (IQR) IU/L } compared in HV {10.5 (7.6-12.7) IU/L } or OLD {9.9 (7.4-11.6) IU/L } (p Discussion and conclusion: Serum ACE significantly increased in LAM, which was associated with lymphangioleiomyomas and serum VEGF-D, suggesting lymphatic abnormalities. Serum ACE may have accessory role in LAM which differ from VEGF-D. This study was partially supported by the grant from JMHLW (YI).

Serum angiotensin-converting enzyme activity in lymphangioleiomyomatosis

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Papers

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

Brain Metastasis of Pleural Mesothelioma after a Subarachnoid Hemorrhage.

Prospective phase II study of cisplatin plus pemetrexed with maintenance of pemetrexed for advanced non-squamous cell non-small cell lung cancer in Japan

Serum angiotensin-converting enzyme activity in lymphangioleiomyomatosis