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Using our diagnostic criteria, we could recruit relatively many patients with similar characteristics to those of idiopathic PPFE patients in the literature. The possibility of clinical diagnosis of idiopathic PPFE should be further discussed.

These results confirm that patients with IPF often develop pneumothorax during their clinical course and that the onset of pneumothorax predicts a poor outcome.

A diagnosis of IPAF might predict a favorable prognosis and less risk of AEs in patients with CFIP.

Change in body composition with skeletal muscle wasting, a major component of pulmonary cachexia, is associated with mortality in chronic obstructive pulmonary disease and cancer. However, its relevance in interstitial lung diseases (ILDs) remains unclear. We hypothesized changes in body composition would be associated with mortality in ILDs. We measured the cross-sectional-area (ESMCSA) and muscle attenuation (ESMMA) of erector-spinae muscles, as determined by CT-imaging, in patients with idiopathic pulmonary fibrosis (IPF; n = 131) and idiopathic pleuroparenchymal fibroelastosis (iPPFE; n = 43) and controls. Subsequently, implications with prognosis were evaluated. The ESMCSA of ILD patients, but not ESMMA, was significantly smaller than that in controls. Lower ESMCSA with decreased BMI were recorded in iPPFE patients versus IPF patients, whilst IPF patients had decreased ESMCSA without BMI decline. Lower ESMCSA in IPF patients were associated with poorer prognoses. Conversely, decreased ESMMA were associated with worse survival in iPPFE patients. Multivariate analyses showed that ESMCSA in IPF and ESMMA in iPPFE were independent risk factors for mortality. Distinct changes in body composition had prognostic significance among patients with IPF and iPPFE. Lower ESMCSA and ESMMA were independently associated with poor prognosis in IPF and iPPFE, respectively. These results suggest values to measure body composition changes in managing patients with IPF and iPPFE.

BackgroundRadiologic pleuroparenchymal fibroelastosis (PPFE)-like lesion including pulmonary apical cap can be occasionally observed in clinical settings. However, the significance of radiologic PPFE-like lesion is unclear in connective tissue disease (CTD)-related interstitial lung disease (ILD).Materials and methodsA total of 113 patients with CTD-related ILD were enrolled and assessed for radiologic PPFE-like lesion, which was defined as bilateral, upper lobe, and subpleural dense consolidations with or without pleural thickening on chest high-resolution computed tomography. The clinical, radiologic, and pathologic characteristics were evaluated.ResultsRadiologic PPFE-like lesion was found in 21 patients (19%) and were relatively frequent in those with systemic sclerosis (6/14: 43%) and primary Sjögren's syndrome (4/14: 29%). Patients with PPFE-like lesion were significantly older, had lower body mass index, higher ratio of residual volume to total lung capacity, and higher complication rate of pneumothorax and/or pneumomediastinum than those without. Twelve of the 21 patients were diagnosed radiologically as usual interstitial pneumonia (UIP) or possible UIP pattern. Two of three patients who underwent surgical lung biopsy of the upper lobes showed UIP on histopathology. Another patient was confirmed to have upper lobe PPFE on autopsy. During the clinical course, progression of the radiologic PPFE-like lesions was observed in 13 of 21 patients. Six patients died (mortality rate: 29%) and their PPFE-like lesions were commonly progressive. In the total cohort, our multivariate analysis identified the presence of PPFE-like lesion as a significant risk factor for respiratory death (hazard ratio: 4.10, 95% confidence interval: 1.33–12.65, p = 0.01).ConclusionIn patients with CTD-related ILD, radiologic PPFE-like lesion, which may present as not only PPFE but also apical cap and upper lobe subpleural fibrosis commonly due to UIP, was not uncommon and was associated with poor prognosis. Clinicians should be cautious with this radiologic finding, particularly when it is progressive.

IntroductionInterstitial lung disease associated with primary Sjögren’s syndrome (pSS–ILD) shows several patterns such as nonspecific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP). Although UIP is a well–recognized prognostic determinant in idiopathic interstitial pneumonias, whether this is also the case in pSS–ILD is unclear. The objectives of this study were to evaluate the prognostic effect of UIP, and to identify the prognostic factors in pSS–ILD.MethodsA retrospective review of medical records identified 33 consecutive patients with pathologically–proven pSS–ILD. Each patient was classified into each ILD pattern by multidisciplinary analysis. Baseline clinical–radiologic–pathologic characteristics and survival rates were compared between the ILD patterns. Finally, the prognostic factors in pSS–ILD were assessed by univariate and subsequent multivariate analyses using Cox’s proportional hazards regression model.ResultspSS–ILD patients were diagnosed with NSIP (n = 22) or UIP (n = 11). The median follow–up period was 110 months, and five-year survival rate was 87.3% in the total patient population. The prognosis of the UIP patients was not significantly different from that of the NSIP patients (NSIP to UIP, hazard ratio [HR]: 0.77, 95% confidence interval [CI]: 0.18–3.36, P = 0.73). Multivariate analysis identified PaCO2 (HR: 1.68 per 1 Torr increase, 95% CI: 1.24–2.28, P < 0.01), extent of reticular abnormality on high–resolution CT (HR: 4.17 per 1-grade increment, 95% CI: 1.18–14.73, P = 0.03), and severity of fibroblastic foci (HR: 9.26 per 1-grade increment, 95% CI: 1.74–49.35, P < 0.01) as prognostic factors in pSS–ILD.ConclusionsUIP in pSS–ILD was not related to poorer prognosis than NSIP. Assessment of detailed clinical–radiologic–pathologic findings is more important than distinguishing UIP to evaluate prognosis in this disease.

Homozygous deletion of 9p21, the locus harboring the p16 gene, has been reported as one of the most common genetic alterations in malignant mesotheliomas (MMs). Previous studies showed that this alteration might be useful for differentiating benign from malignant mesothelial tumors in cytology and surgical specimens. Although the diagnostic utility of 9p21 homozygous deletion by fluorescence in situ hybridization (FISH) analysis has been reported only recently, it has not been well demonstrated. The purpose of this study is to evaluate the diagnostic utility of 9p21 homozygous deletion assessed by FISH in mesothelial neoplasm and hyperplasia of Japanese patients using paraffin-embedded tissue. Simultaneously, p16 protein immunoexpression was explored as a potential diagnostic aid. FISH analysis demonstrated 9p21 deletion in 35 of 40 cases with MM (88%) (P < 0.001). In contrast, no cases of adenomatoid tumor, benign mesothelial multicystic tumor, reactive mesothelial hyperplasia or pleuritis showed 9p21 deletion (P < 0.005). 9p21 homozygous deletion correlated well with p16 protein expression in the tumor cells. Our study suggests that 9p21 homozygous deletion assessed by FISH on paraffin-embedded tissue may be very useful for differentiating MM from reactive mesothelial proliferation.