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. The category of myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA, PDGFRB, FGFR1 and PCM1-JAK2 represents an uncommon cause of eosinophilic lung infiltrate. CASE PRESENTATION: We report the case of a 70-year old man complaining of dry cough and dyspnea. Ground glass-opacities were seen on imaging studies and peripheral blood eosinophilia was present. A thorough step-wise patient's evaluation led to identify the clonal nature of eosinophilia and the diagnosis of myeloid/lymphoid neoplasm with eosinophilia and rearrangement of PDGFRA was made. CONCLUSIONS: Correlation with clinical history, laboratory tests and imaging studies is essential to achieve the correct diagnosis when facing with eosinophilic lung infiltrates. A prolonged eosinophilia can cause life-threatening organ damage. Identification of PDGFRA rearrangement, as in the present case, is particularly critical given the sensitivity and excellent response to imatinib, which has completely changed the natural history of this neoplasm.

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. DIAGNOSES: The main radiologic findings included nodules, cysts, nodules with halo sign, and vascular tree-in-bud. Morphologic features, as observed in the histologic specimen, have been correlated with radiologic appearance. LESSONS: Metastatic angiosarcomas to the lung are characterized by a wide variety of radiologic appearances that can be very characteristic. Computed tomographic findings observed include bilateral solid nodules, cystic, and bullous lesions sometimes associated with spontaneous hemopneumothoraces.

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. CT scan features of traction bronchiectasis and honeycombing should be interpreted under the light of these new pathogenetic and morphologic considerations. SHORT CONCLUSION: We suggest that in IPF subjects traction bronchiectasis and honeycombing -now defined as distinct entities on HRCT scan- are actually diverse aspects of a continuous spectrum of lung remodeling.

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. METHODS: In this cross-sectional study we selected 117 patients with fibrotic interstitial lung disease without a typical usual interstitial pneumonia pattern on high-resolution computed tomography. All cases underwent lung biopsies: 58 were BLC, and 59 were surgical lung biopsy (SLB). Two clinicians, two radiologists, and two pathologists sequentially reviewed clinical-radiologic findings and biopsy results, recording at each step in the process their diagnostic impressions and confidence levels. MEASUREMENTS AND MAIN RESULTS: We observed a major increase in diagnostic confidence after the addition of BLC, similar to SLB (from 29 to 63%, P = 0.0003 and from 30 to 65%, P = 0.0016 of high confidence IPF diagnosis, in the BLC group and SLB group, respectively). The overall interobserver agreement in IPF diagnosis was similar for both approaches (BLC overall kappa, 0.96; SLB overall kappa, 0.93). IPF was the most frequent diagnosis (50 and 39% in the BLC and SLB group, respectively; P = 0.23). After the addition of histopathologic information, 17% of cases in the BLC group and 19% of cases in the SLB group, mostly idiopathic nonspecific interstitial pneumonia and hypersensitivity pneumonitis, were reclassified as IPF. CONCLUSIONS: 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.

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. • 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.

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. METHODS: 30 patients had received a diagnosis of familial IPF at ourinstitution over the period from January 2005 and December 2011; in7 of them there was a parent-child relation.Clinical features and patient outcome were analyzed and contrasted toa well characterized cohort of 127 patients with non familial IPF. RESULTS: there was no significant difference in presenting symptoms and theoverall outcomes were quite similar in the two groups, but thefamilial group was much more enriched for females and we found astatistically significant lower age at onset in the youngergenerations (mean age 57,8 years versus 74,2 years, p 0,001). Acuteexacerbations, IPF progression and lung cancer were more frequent inthe familial IPF group as a cause of death (p < 0,005). CONCLUSION: familial IPF seems indistinguishable from sporadic IPF with respectto most clinical and physiologic findings; however the age of onsetwas slightly lower among the familial cases than in the sporadiccases of IPF and the phenomenon of anticipation could be observed.

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). According to recently proposed pathogenic models in COPD and IPF, premature cellular senescence likely affects distinct progenitors cells (mesenchymal stem cells in COPD, alveolar epithelial precursors in IPF), leading to stem cell exhaustion. In this review, the large amount of data supporting this pathogenic view are discussed, with emphasis on the possible molecular and cellular mechanisms leading to the severe parenchymal remodeling that characterizes, in different ways, these deadly diseases.

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. Acute exacerbation associated with rapid functional decline is an event that does not happen infrequently and affects survival. Diagnosis requires a typical usual interstitial pneumonia (UIP) pattern on computed tomography in the appropriate clinical setting or morphological confirmation of the UIP pattern when imaging findings are not characteristic enough. Surgical lung biopsy is the gold standard to obtain valuable information for histological analysis. However, less invasive procedures (transbronchial lung biopsy or even improved transbronchial lung biopsy by cryoprobes) are now under consideration. Prognostic indicators are mainly derived by pulmonary function tests. Recently, staging systems have been proposed.

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. The aim of this report is to describe two further cases of this rare disease, reviewing CT, clinical and histological features.

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. On the basis of the clinico-radiologic presentation, a pulmonary hemorrhage was likely to occur; so to clarify the origin of this process, a complete serologic examination was performed but all the antibodies were negative. Finally a renal biopsy was performed and it showed a pauci-immunologic glomerulonephritis and the bronchioloalveolar lavage confirmed the diffuse alveolar hemorrhage. In conclusion, the diagnosis of leflunomide-pulmonary-renal syndrome was rendered. The treatment with leflunomide was suspended; the conditions of the patient gradually improved and he became completely asymptomatic 1 week later.

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. An endobronchial lesion was diagnosed by spiral CT scan, taken at the onset of the menses, and confirmed with flexible bronchoscopy. The patient was successfully treated with endoscopic Nd-YAG laser therapy with a 1-day in-hospital procedure. We suggest that endoscopic laser treatment should be the first line of therapy for central airway endometriosis, provided that the source of bleeding has been conclusively located and all of the lesions can be reached with the bronchoscope.