A tricky and rare cause of pulmonary eosinophilia: myeloid/lymphoid neoplasm with eosinophilia and rearrangement of PDGFRA.

Background: Eosinophilic lung diseases represent a heterogeneous group of disorders with prominent infiltrate of eosinophils in lung interstitium and alveolar spaces. Peripheral blood eosinophilia is often present. Infections, drugs, allergens, toxic agents have to be evaluated as possible causes of eosinophilic lung infiltrates.

Angiosarcoma in the chest: radiologic-pathologic correlation: Case report.

Rationale: Angiosarcomas are rare, malignant vascular tumors.

Patient Concerns: They represents about 2% of all soft tissue sarcoma, which can often metastasize through the hematogenous route. The radiological features have been analyzed in 4 patients with metastatic angiosarcoma in the chest.

From "traction bronchiectasis" to honeycombing in idiopathic pulmonary fibrosis: A spectrum of bronchiolar remodeling also in radiology?

Background: The diagnostic and prognostic impact of traction bronchiectasis on high resolution CT scan (HRCT) in patients suspected to have idiopathic pulmonary fibrosis (IPF) is increasing significantly.

Main Body: Recent data demonstrated that cysts in honeycombing areas are covered by epithelium expressing bronchiolar markers. In IPF bronchiolization is the final consequence of a variety of pathogenic events starting from alveolar stem cell exhaustion, and ending in a abnormal/dysplastic proliferation of bronchiolar epithelium.

Bronchoscopic Lung Cryobiopsy Increases Diagnostic Confidence in the Multidisciplinary Diagnosis of Idiopathic Pulmonary Fibrosis.

Rationale: Surgical lung biopsy is often required for a confident multidisciplinary diagnosis of idiopathic pulmonary fibrosis (IPF). Alternative, less-invasive biopsy methods, such as bronchoscopic lung cryobiopsy (BLC), are highly desirable.

Objectives: To address the impact of BLC on diagnostic confidence in the multidisciplinary diagnosis of IPF.

The azygos vein pathway: an overview from anatomical variations to pathological changes.

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.

Features and outcome of familial idiopathic pulmonary fibrosis.

Background: Idiopathicpulmonary fibrosis (IPF) has a sporadic occurrence in most instances,but can also occasionally occur in familial form. While clinicalfeatures of sporadic IPF are well defined, clinical presentation,complications, and outcome of familial IPF are still undefined. Thisretrospective study was undertaken to establish clinical parametersand survival time in a consecutive series of patients with familialIPF and to establish whether the phenomenon of anticipation could beobserved.

Premature lung aging and cellular senescence in the pathogenesis of idiopathic pulmonary fibrosis and COPD/emphysema.

Different anatomic and physiological changes occur in the lung of aging people that can affect pulmonary functions, and different pulmonary diseases, including deadly diseases such as chronic obstructive pulmonary disease (COPD)/emphysema and idiopathic pulmonary fibrosis (IPF), can be related to an acceleration of the aging process. The individual genetic background, as well as exposure to a variety of toxic substances (cigarette smoke in primis) can contribute significantly to accelerating pulmonary senescence. Premature aging can impair lung function by different ways: by interfering specifically with tissue repair mechanisms after damage, thus perturbing the correct crosstalk between mesenchymal and epithelial components; by inducing systemic and/or local alteration of the immune system, thus impairing the complex mechanisms of lung defense against infections; and by stimulating a local and/or systemic inflammatory condition (inflammaging).

Idiopathic pulmonary fibrosis: diagnosis and prognostic evaluation.

Idiopathic pulmonary fibrosis (IPF) is the most common type of idiopathic interstitial pneumonia and has a dismal prognosis. Median age at IPF onset is 60-70 years and it is mainly related to cigarette smoke exposure. Its clinical profile is heterogeneous and different clinical phenotypes are now better defined: familial IPF, slow and rapid progressors, combined pulmonary fibrosis and emphysema, anti-neutrophil cytoplasmic antibodies/microscopic polyangiitis and IPF, and IPF associated with lung cancer.

High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis.

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.

Diffuse alveolar hemorrhage after leflunomide therapy in a patient with rheumatoid arthritis.

We report the case of a young man, affected by rheumatoid arthritis who developed a rapid-onset short-of-breath, hemoptysis, and severe weakness, about 2 weeks after the administration of leflunomide. Chest radiography showed central bilateral opacities and pleural effusion as confirmed by the high-resolution computed tomography that demonstrated diffuse ground-glass and interlobular septal thickening as well. On admission at the Emergency Department, a microhematuria and a severe anemia were also documented.

Successful endoscopic Nd-YAG laser treatment of endobronchial endometriosis.

Catamenial hemoptysis is a rare condition that is associated with the presence of intrapulmonary or endobronchial endometrial tissue. Diagnosis of and therapy for this condition are still a matter of debate. We describe a case of endobronchial endometriosis with catamenial hemoptysis.