Epidemiology of Malignant Pleural Mesotheliomas in Croatia in the Period from 1989 to 1998

Malignant pleural mesotheliomas are rare tumors. Their occurrence is often associated with the exposure to asbestos. Asbestos is widely used in various industries as well as for many types of products in everyday use. In Croatia in the period from 1989 to 1998, the rate of incidence was 0.4–1.1 / 100,000. The highest rate of incidence was in the Districts of Istria (2.9) and Split-Dalmatia (2.5). It is more frequent among males than among females with a ratio of 3.2:1. It rarely occurs before the age of 40 and most of the patients suffering from the disease are more than 65 years old. About 12% of mesotheliomas metastasize into regional lymph nodes and 17% of them into distant organs. The disease unavoidably leads to death and, according to the data obtained in Croatia in the period from 1989 to 1998 the mortality and incidence are very close

Lung Lavage Neutrophils, Neutrophil Elastase and Albumin in the Prognosis of Pulmonary Sarcoidosis

The authors measured the bronchoalveolar lavage fluid (BALF) neutrophils, neutrophil elastase and albumin in 60 patients with pulmonary sarcoidosis, 18 patients with other interstitial lung diseases and 14 healthy controls in order to evaluate its significance in the prognosis of sarcoidosis. The concentrations were measured by enzymoimmunoassay and radial immunodiffusion (LC plates). Obtained values were correlated with some clinical indices, radiological stage and lung function values. The patients were followed up for 22.3 6.8 months. At the end of observation period the outcome of sarcoidosis has been evaluated upon the findings of chest radiograms and lung function tests. The length of duration of the disease correlated with the lung lavage neutrophil counts (r = 0.2535; p < 0.05). Significantly higher values of BALF elastase were obtained in III radiologic stage than II and I. Patients with high BALF albumin had more often dispnoa (p < 0.05) and chronic forms of the disease (p < 0.05). The values were high in patients in II and III radiologic stage. Unfavorable outcome of chest radiograms was announced by high BALF albumins (p < 0.05). The measurement of BALF neutrophils, neutrophil elastase and albumin are helpful in clinical management and estimation of the prognosis in patients with pulmonary sarcoidosis

Lung Lavage Neutrophils, Neutrophil Elastase and Albumin in the Prognosis of Pulmonary Sarcoidosis

The authors measured the bronchoalveolar lavage fluid (BALF) neutrophils, neutrophil elastase and albumin in 60 patients with pulmonary sarcoidosis, 18 patients with other interstitial lung diseases and 14 healthy controls in order to evaluate its significance in the prognosis of sarcoidosis. The concentrations were measured by enzymoimmunoassay and radial immunodiffusion (LC plates). Obtained values were correlated with some clinical indices, radiological stage and lung function values. The patients were followed up for 22.3 6.8 months. At the end of observation period the outcome of sarcoidosis has been evaluated upon the findings of chest radiograms and lung function tests. The length of duration of the disease correlated with the lung lavage neutrophil counts (r = 0.2535; p < 0.05). Significantly higher values of BALF elastase were obtained in III radiologic stage than II and I. Patients with high BALF albumin had more often dispnoa (p < 0.05) and chronic forms of the disease (p < 0.05). The values were high in patients in II and III radiologic stage. Unfavorable outcome of chest radiograms was announced by high BALF albumins (p < 0.05). The measurement of BALF neutrophils, neutrophil elastase and albumin are helpful in clinical management and estimation of the prognosis in patients with pulmonary sarcoidosis

Pulmonary embolism versus pulmonary vasculitis in Hughes-Stovin syndrome: Characteristic computed tomography pulmonary angiographic findings and diagnostic and therapeutic implications. HSS International Study Group

Background and aim: Hughes-Stovin syndrome (HSS) is a rare systemic vasculitis with widespread venous/arterial thrombosis and pulmonary vasculitis. Distinguishing between pulmonary embolism (PE) and in-situ thrombosis in the early stages of HSS is challenging. The aim of the study is to compare clinical, laboratory, and computed tomography pulmonary angiography (CTPA) characteristics in patients diagnosed with PE versus those with HSS. Methods: This retrospective study included 40 HSS patients with complete CTPA studies available, previously published by the HSS study group, and 50 patients diagnosed with PE from a single center. Demographics, clinical and laboratory findings, vascular thrombotic events, were compared between both groups. The CTPA findings were reviewed, with emphasis on the distribution, adherence to the mural wall, pulmonary infarction, ground glass opacification, and intra-alveolar hemorrhage. Pulmonary artery aneurysms (PAAs) in HSS were assessed and classified. Results: The mean age of HSS patients was 35 ± 12.3 years, in PE 58.4 ± 17 (p &lt; 0.0001). Among PE 39(78 %) had co-morbidities, among HSS none. In contrast to PE, in HSS both major venous and arterial thrombotic events are seen. Various patterns of PAAs were observed in the HSS group, which were entirely absent in PE. Parenchymal hemorrhage was also more frequent in HSS compared to PE (P &lt; 0.001). Conclusion: Major vascular thrombosis with arterial aneurysms formation are characteristic of HSS. PE typically appear loosely-adherent and mobile whereas “in-situ thrombosis” seen in HSS is tightly-adherent to the mural wall. Mural wall enhancement and PAAs are distinctive pulmonary findings in HSS. The latter findings have significant therapeutic ramifications.</p

Diagnostic accuracy of a clinical diagnosis of idiopathic pulmonary fibrosis: An international case-cohort study

We conducted an international study of idiopathic pulmonary fibrosis (IPF) diagnosis among a large group of physicians and compared their diagnostic performance to a panel of IPF experts. A total of 1141 respiratory physicians and 34 IPF experts participated. Participants evaluated 60 cases of interstitial lung disease (ILD) without interdisciplinary consultation. Diagnostic agreement was measured using the weighted kappa coefficient (\u3baw). Prognostic discrimination between IPF and other ILDs was used to validate diagnostic accuracy for first-choice diagnoses of IPF and were compared using the Cindex. A total of 404 physicians completed the study. Agreement for IPF diagnosis was higher among expert physicians (\u3baw=0.65, IQR 0.53-0.72, p20 years of experience (C-index=0.72, IQR 0.0-0.73, p=0.229) and non-university hospital physicians with more than 20 years of experience, attending weekly MDT meetings (C-index=0.72, IQR 0.70-0.72, p=0.052), did not differ significantly (p=0.229 and p=0.052 respectively) from the expert panel (C-index=0.74 IQR 0.72-0.75). Experienced respiratory physicians at university-based institutions diagnose IPF with similar prognostic accuracy to IPF experts. Regular MDT meeting attendance improves the prognostic accuracy of experienced non-university practitioners to levels achieved by IPF experts

A critical analysis of 57 cases of Hughes-Stovin syndrome (HSS). A report by the HSS International Study Group (HSSISG)

Hughes-Stovin syndrome (HSS) is a systemic disease characterized by widespread vascular thrombosis and pulmonary vasculitis with serious morbidity and mortality. The HSS International Study Group is a multidisciplinary taskforce aiming to study HSS, in order to generate consensus recommendations regarding diagnosis and treatment. We included 57 published cases of HSS (43 males) and collected data regarding: clinical presentation, associated complications, hemoptysis severity, laboratory and computed tomography pulmonary angiography (CTPA) findings, treatment modalities and cause of death. At initial presentation, DVT was observed in 29(33.3 %), thrombophlebitis in 3(5.3%), hemoptysis in 24(42.1%), and diplopia and seizures in 1 patient each. During the course of disease, DVT occurred in 48(84.2%) patients, and superficial thrombophlebitis was observed in 29(50.9%). Hemoptysis occurred in 53(93.0%) patients and was fatal in 12(21.1%). Pulmonary artery (PA) aneurysms (PAAs) were bilateral in 53(93%) patients. PAA were located within the main PA in 11(19.3%), lobar in 50(87.7%), interlobar in 13(22.8%) and segmental in 42(73.7%). Fatal outcomes were more common in patients with inferior vena cava thrombosis (p = 0.039) and ruptured PAAs (p < 0.001). Death was less common in patients treated with corticosteroids (p < 0.001), cyclophosphamide (p < 0.008), azathioprine (p < 0.008), combined immune modulators (p < 0.001). No patients had uveitis; 6(10.5%) had genital ulcers and 11(19.3%) had oral ulcers. HSS may lead to serious morbidity and mortality if left untreated. PAAs, adherent in-situ thrombosis and aneurysmal wall enhancement are characteristic CTPA signs of HSS pulmonary vasculitis. Combined immune modulators contribute to favorable outcomes

Pulmonary vasculitis in Hughes-Stovin syndrome (HSS): a reference atlas and computed tomography pulmonary angiography guide - a report by the HSS International Study Group

Introduction: Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by widespread venous/arterial thrombosis and pulmonary artery aneurysms (PAAs), which is associated with serious morbidity and mortality. All fatalities reported in HSS resulted from unpredictable fatal suffocating hemoptysis. Therefore, it is necessary to recognize pulmonary complications at an early stage of the disease. Objectives: The aims of this study are to develop a reference atlas of images depicting the characteristic features of HSS by computed tomography pulmonary angiography (CTPA). To make a guide for physicians by developing a classification of PAAs according to the severity and risk of complications associated with each distinct lesion type. Methods: The Members of the HSS International Study Group (HSSISG) collected 42 cases, with high-quality CTPA images in one radiology station and made reconstructions from the source images. These detailed CTPA studies were reviewed for final image selection and approved by HSSISG board members. We classified these findings according to the clinical course of the patients. Results: This atlas describes the CTPA images that best define the wide spectrum of pulmonary vasculitis observed in HSS. Pulmonary aneurysms were classified into six radiographic patterns: from true stable PAA with adherent in-situ thrombosis to unstable leaking PAA, BAA and/or PAP with loss of aneurysmal wall definition (most prone to rupture), also CTPA images demonstrating right ventricular strain and intracardiac thrombosis. Conclusion: The HSSISG reference atlas is a guide for physicians regarding the CTPA radiological findings, essential for early diagnosis and management of HSS-related pulmonary vasculitis.Key Points• The Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by extensive vascular thrombosis and pulmonary artery aneurysms (PAAs) that can lead to significant morbidity and mortality.• All fatalities reported in HSS were related to unpredictable massive hemoptysis; therefore, it is critical to recognize pulmonary complications at an early stage of the disease.• The HSS International Study Group reference atlas classifies pulmonary vasculitis in HSS at 6 different stages of the disease process and defines the different radiological patterns of pulmonary vasculitis notably pulmonary artery aneurysms, as detected by computed tomography pulmonary angiography (CTPA).• The main aim of the classification is to make a guide for physicians about this rare syndrome. Such a scheme has never been reached before since the first description of the syndrome by Hughes and Stovin since 1959. This classification will form the basis for future recommendations regarding diagnosis and treatment of this syndrome