BLC is a new biopsy method that has a meaningful impact on diagnostic confidence in the multidisciplinary diagnosis of interstitial lung disease and may prove useful in the diagnosis of IPF. This study provides a robust rationale for future studies investigating the diagnostic accuracy of BLC compared with SLB.

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Recent evidence suggests that idiopathic nonspecific interstitial pneumonia (iNSIP) is a distinct clinical entity amongst other idiopathic interstitial pneumonias, and some data seem to suggest a possible pathogenetic role of autoimmunity. The aim of the present study was to assess if iNSIP might represent an early lung manifestation of an autoimmune disease.After initial review of cases found in the medical records database by searching for the term ''NSIP'' (n563), 37 iNSIP cases were identified, and were re-evaluated using a dynamic integrated multidisciplinary approach.27 cases with iNSIP were selected for the study. Mean¡SD age at first respiratory symptom was 54.2¡8 yrs, 70% were females, and 59% were never-smokers. At follow-up (mean¡SD 59.7¡29 months, range 12-138 months), autoimmune diseases occurred in 14 (52%) patients, with seven (26%) cases of autoimmune thyroiditis, six (22%) of undifferentiated connective tissue disease and three (11%) of connective tissue disease. Patients developing autoimmune diseases were older and more frequently never-smoking females.In .50% of patients diagnosed with iNSIP, evidence of autoimmune diseases develops within 2 yrs, suggesting a probable link between the clinical entity of iNSIP and autoimmune disorders.

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently described clinical-pathologic entity characterized by pleural and subpleural parenchymal fibrosis, mainly in the upper lobes. As this disease is extremely rare (only 7 cases have been described in the literature to date) poorly defined cases of IPPFE can go unrecognized.The clinical course of disease is progressive and prognosis is poor, with no therapeutic options other than lung transplantation currently available, yet. The aim of this report is to describe two further cases of this rare disease, reviewing CT, clinical and histological features.

The azygos venous system represents an accessory venous pathway supplying an important collateral circulation between the superior and inferior vena cava. The aim of this article is to revise the wide spectrum of changes ranging from normal to pathological conditions involving the azygos system.Teaching points• The azygos vein is a collateral venous pathway, becoming a vital shunt if major pathways of venous return are obstructed.• In azygos continuation, the azygos vein becomes significantly enlarged due to inferior vena cava interruption.• Fibrosing mediastinitis is an underestimated acquired disorder.• Fibrosing mediastinitis induces a variable engorgement of collateral veins.• Fibrosing mediastinitis leads to superior vena cava syndrome.

BHDS is associated with cystic lung disease largely under-recognized by pulmonary physicians and can mimic LAM and may be associated with lung tumor, pulmonary histiocytoma. In one case we found a novel mutation in exon 7, c.771 del (ref.seq. NM_144997.5) never reported before.

CT was the most effective method for evaluating the presence and features of diverticula. A correlation between tracheobronchial diverticula and chronic respiratory disease was documented in 5/16 patients, whereas no respiratory disorder was identified in the remaining 11. Although chronic inflammatory tracheobronchial changes and increased endoluminal pressure may be important causes, we hypothesise that other, as yet unknown, aetiopathological factors could exist.

Birt-Hogg-Dubé syndrome (BHDS) is characterized by a clinical triad including cutaneous hamartomas originating from hair follicles, lung cysts/pneumothorax, and kidney tumors. Inactivating mutations of the tumor suppressor gene FLCN are identified in most families with BHDS. Usually, patients are referred for genetic examination by dermatologists because of the presence of typical multiple skin tumors with or without additional symptoms. However, because of phenotypic variability and incomplete penetrance, the clinical presentation of BHDS is not yet fully defined. Criteria for genetic testing and diagnosis that take into account variable manifestations have recently been proposed by the European BHD Consortium. We sequenced the FLCN gene coding region in a series of 19 patients selected for kidney and/or lung manifestations. Overall, FLCN mutations were found in 9 of 19 (47%) families and were detected only in probands who had either >2 components of the clinical triad or a single component (renal or pulmonary) along with a family history of another main BHDS manifestation. Typical cutaneous lesions were present only in 8 of 21 FLCN mutation carriers aged >20 years identified in the mutation-positive families. In addition, we provide clinical and molecular evidence that parotid oncocytoma, so far reported in six BHDS cases, is associated with this condition, based on the observation of a patient with bilateral parotid involvement and marked reduction of the wild-type FLCN allele signal in tumor DNA. Overall, the results obtained in this study contribute to the definition of the phenotypic characteristics that should be considered for BHDS diagnosis and FLCN mutation testing.