Guidelines of the Polish Respiratory Society on the Diagnosis and Treatment of Progressive Fibrosing Interstitial Lung Diseases Other than Idiopathic Pulmonary Fibrosis.

The recommendations were developed as answers to previously formulated questions concerning everyday diagnostic and therapeutic challenges. They were developed based on a review of the current literature using the GRADE methodology. The experts suggest that PF-ILD be diagnosed based on a combination of different criteria, such as the aggravation of symptoms, progression of radiological lesions, and worsening of lung function test parameters. The experts recommend a precise diagnosis of an underlying disease, with serological testing for an autoimmune disease always being included. The final diagnosis should be worked out by a multidisciplinary team (MDT). Patients with an interstitial lung disease other than IPF who do not meet the criteria for the progressive fibrosis phenotype should be monitored for progression, and those with systemic autoimmune diseases should be regularly monitored for signs of interstitial lung disease. In managing patients with interstitial lung disease associated with autoimmune diseases, an opinion of an MDT should be considered. Nintedanib rather than pirfenidon should be introduced in the event of the ineffectiveness of the therapy recommended for the treatment of the underlying disease, but in some instances, it is possible to start antifibrotic treatment without earlier immunomodulatory therapy. It is also admissible to use immunomodulatory and antifibrotic drugs simultaneously. No recommendations were made for or against termination of anti-fibrotic therapy in the case of noted progression during treatment of a PF-ILD other than IPF. The experts recommend that the same principles of non-pharmacological and palliative treatment and eligibility for lung transplantation should be applied to patients with an interstitial lung disease other than IPF with progressive fibrosis as in patients with IPF.

Guidelines of the Polish Respiratory Society on the Diagnosis and Treatment of Progressive Fibrosing Interstitial Lung Diseases Other than Idiopathic Pulmonary Fibrosis

The recommendations were developed as answers to previously formulated questions concerning everyday diagnostic and therapeutic challenges. They were developed based on a review of the current literature using the GRADE methodology. The experts suggest that PF-ILD be diagnosed based on a combination of different criteria, such as the aggravation of symptoms, progression of radiological lesions, and worsening of lung function test parameters. The experts recommend a precise diagnosis of an underlying disease, with serological testing for an autoimmune disease always being included. The final diagnosis should be worked out by a multidisciplinary team (MDT). Patients with an interstitial lung disease other than IPF who do not meet the criteria for the progressive fibrosis phenotype should be monitored for progression, and those with systemic autoimmune diseases should be regularly monitored for signs of interstitial lung disease. In managing patients with interstitial lung disease associated with autoimmune diseases, an opinion of an MDT should be considered. Nintedanib rather than pirfenidon should be introduced in the event of the ineffectiveness of the therapy recommended for the treatment of the underlying disease, but in some instances, it is possible to start antifibrotic treatment without earlier immunomodulatory therapy. It is also admissible to use immunomodulatory and antifibrotic drugs simultaneously. No recommendations were made for or against termination of anti-fibrotic therapy in the case of noted progression during treatment of a PF-ILD other than IPF. The experts recommend that the same principles of non-pharmacological and palliative treatment and eligibility for lung transplantation should be applied to patients with an interstitial lung disease other than IPF with progressive fibrosis as in patients with IPF.

Guidelines of the Polish Respiratory Society for diagnosis and treatment of idiopathic pulmonary fibrosis.

INTRODUCTION: This document presents the guidelines of the Polish Respiratory Society (PTChP, Polskie Towarzystwo Chorób Pluc) for diagnosis and treatment of idiopathic pulmonary fibrosis (IPF), developed by agroup of Polish experts. MATERIAL AND METHODS: The recommendations were developed in the form of answers to previously formulated questions concer-ning everyday diagnostic and therapeutic challenges. They were developed based on acurrent literature review using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. RESULTS: We formulated 28 recommendations for diagnosis (8), pharmacological treatment (12) as well as non-pharma-cological and palliative therapy (8). The experts suggest that surgical lung biopsy (SLB) not be performed in patients with the probable usual interstitial pneumonia (UIP) pattern, with an appropriate clinical context and unanimous opinion of a multidisciplinary team. The experts recommend using antifibrotic agents in IPF patients and suggest their use irrespective of the degree of functional impairment. As regards non-pharmacological and palliative treatment, strong re-commendations were formulated regarding pulmonary rehabilitation, oxygen therapy (in patients with chronic respiratory failure), preventive vaccinations as well as referring IPF patients to transplant centres. Table 1 presents an aggregate list of recommendations. CONCLUSIONS: The Polish Respiratory Society Working Group developed guidelines for IPF diagnosis and treatment.

Baseline new bone formation does not predict bone loss in ankylosing spondylitis as assessed by quantitative computed tomography (QCT): 10-year follow-up.

BACKGROUND: To evaluate the relationship between bone loss and new bone formation in ankylosing spondylitis (AS) using 10-year X-ray, dual-energy x-ray absorptiometry (DXA) and quantitative computed tomography (QCT) follow-up. METHODS: Fifteen AS patients free from medical conditions and drugs affecting bone metabolism underwent X-ray, DXA and QCT in 1999 and 2009. RESULTS: In spine QCT a statistically significant (p = 0,001) decrease of trabecular bone mineral content (BMC) was observed (change ± SD: 18.0 ± 7.3 mg/cm3). In contrast, spine DXA revealed a significant increase of bone mineral density (change ± SD: -0.15 ± 0.14 g/cm2). The mean BMC, both at baseline and follow-up was significantly lower (p = 0.02 and p = 0.005, respectively) in advanced radiological group as compared to early radiological group. However, in multiple regression model after adjustment for baseline BMC, the baseline radiological scoring did not influence the progression of bone loss as assessed with QCT (p = 0.22, p for BMC*X-ray syndesmophyte scoring interaction = 0.65, p for ANOVA-based X-ray syndesmophyte scoring*time interaction = 0.39). Baseline BMC was the only significant determinant of 10-year BMC change, to date the longest QCT follow-up data in AS. CONCLUSIONS: In AS patients who were not using antiosteoporotic therapy spine trabecular bone density evaluated by QCT decreased over 10-year follow-up and was not related to baseline radiological severity of spine involvement.

Usefulness of transbronchial needle aspiration for initial lung cancer staging.

INTRODUCTION: Besides radio logical methods (especially positron emission tomography combined with computed tomography), endoscopic techniques including transbronchial needle aspiration (TBNA) of mediastinal lymph nodes play an important role in lung cancer staging, thus having a significant effect on further patient management. OBJECTIVES: The aim of the study was to investigate the diagnostic value of blind TBNA in staging of lung cancer, using systematic mediastinal lymph node dissection (SLND) at thoracotomy as a confirmatory test. PATIENTS AND METHODS: Patients with lung cancer and enlarged mediastinal lymph nodes on computed tomography scans underwent TBNA. Non-small cell lung cancer (NSCLC) patients with negative TBNA or with single-level N2 disease underwent thoracotomy with appropriate pulmonary resection and with SLND. RESULTS: In 84 lung cancer patients, 166 TBNA were performed. Metastatic lymph node involvement was identified in 57 patients (67.9%). There were 10 patients (11.9%) with small cell lung cancer. Of the 74 NSCLC patients, TBNA revealed meta stases in 48 (64.9%). Twenty-four TBNA-negative patients (32.4%) and 4 patients (5.4%) with single-level N2 disease underwent pulmonary resection with SLND. In 8 of 28 operated patients (28.6%), N2 meta static nodes were identified. The per-patient analysis showed the sensitivity of TBNA to be 81.5%, specificity - 100%, accuracy - 86.5%, and negative predictive value (NPV) - 66.7%. CONCLUSIONS: Our results suggest that TBNA may be a useful method for initial NSCLC staging in patients suspected of N2-3 disease. Positive TBNA in 1 station only should not be considered as a true single-level N2 disease, because of a relatively low NPV for TBNA.

TGF-beta1 in bronchoalveolar lavage fluid in diffuse parenchymal lung diseases and high-resolution computed tomography score.

INTRODUCTION: In the pathogenesis of diffuse parenchymal lung diseases (DPLDs), growth factors, including transforming growth factor beta1 (TGF-beta1), are responsible for cell proliferation, apoptosis, chemotaxis, and angiogenesis, and also for the production and secretion of some components of the extracellular matrix. OBJECTIVES: The aim of the study was to evaluate correlations in DPLDs between TGF-beta1 levels in bronchoalveolar lavage (BAL) fluid and high-resolution computed tomography (HRCT) score. PATIENTS AND METHODS: The study was performed in 31 DPLD patients in whom a selection of lung segments with high and low intensity of abnormalities was estimated by HRCT score. All patients underwent BAL with TGF-beta1 measured by an enzyme immunoassay in BAL fluid and video-assisted thoracic surgery lung biopsy from both selected segments. RESULTS: All 31 patients were diagnosed, and based on histopathology, they were classified into 2 groups: idiopathic interstitial pneumonia (usual interstitial pneumonia - 12, nonspecific inter stitial pneumonia - 2, cryptogenic organizing pneumonia - 2, and desquamative interstitial pneumonia - 1) and granulomatous disease (sarcoidosis - 7, extrinsic allergic alveolitis - 5, and histiocytosis X - 2). The final analysis was performed in 28 patients who showed nonhomogenous distribution on HRCT. TGF-beta1 levels in BAL fluid were significantly higher in the areas with high intensity of abnormalities assessed by HRCT score (P = 0.018, analysis of variance). These levels were not different between the groups, but a trend towards higher levels in idiopathic inter stitial pneumonia was observed. CONCLUSIONS: The results confirm that TGF-beta1 may be a good but not specific marker of fibrosis in DPLDs. A significant positive correlation between TGF-beta1 levels in BAL fluid and the HRCT score was observed.

Pulmonary findings in Churg-Strauss syndrome in chest X-rays and high resolution computed tomography at the time of initial diagnosis.

Churg-Strauss syndrome (CSS) is a rare, systemic, necrotizing, small- and middle-sized vessel vasculitis which is accompanied by blood eosinophilia, eosinophil infiltration of various tissues, and bronchial asthma. The lungs are the organs most often involved in CSS. The aim of this study was a retrospective evaluation of the pulmonary findings in chest X-rays and high resolution computed tomography (HRCT) in CSS patients at the time of initial diagnosis and to determine their frequency, character, and location. Seventeen CSS patients were studied (12 women; 5 men; aged 29-56 years). In all patients at the time of initial diagnosis, chest X-rays were performed, and in 15 patients, HRCT was performed additionally. The radiological images were evaluated independently by two radiologists who reached a decision by consensus. Out of 17 patients studied, chest X-rays revealed parenchymal abnormalities in 11, pleural effusion in three, and bronchial wall thickening in one. In five patients, no abnormalities in chest X-rays were found. In HRCT, abnormalities were found in all patients (15 patients, 100%). Predominant HRCT findings consisted of: ground-glass opacities and consolidations found in 13 patients (86.7%). Additionally, in four patients, pulmonary micronodules were described; in ten, interlobular septal thickening; in three, linear opacities; in ten, bronchial wall thickening and/or bronchial dilatation; and in three, pleural effusions. Ground-glass opacities and consolidation abnormalities distribution pattern were peripheral in seven and random in six patients. In patients with CSS, the most common pulmonary radiological findings are parenchymal opacities, which may be peripheral or random in distribution. Pathologic changes were found in 70.6% of patient in chest X-rays, and in 100%, when HRCT was performed. These changes are nonspecific; however, they should be not overlooked, as they may help in establishing the diagnosis and suggest the prognosis.

Rhabdomyoma as a tumor of the posterior mediastinum.

Rhabdomyoma is a rare tumor in the posterior mediastinum. We present a case of adult rhabdomyoma that occurred multifocally in the head, neck and mediastinum. There have been as few as four previous reports on rhabdomyoma in the posterior mediastinum. An 80-year-old white man was admitted to the Division of Pulmonary Diseases because of dyspnea, hemoptysis and fatigue. Physical examination revealed a tumor in the left submandibular area. Computed tomography (CT) of the head and neck showed a mass which caused a marked prominence of the left posterolateral wall of the pharynx. Chest CT revealed a contrast-enhancing tumor. The mass was 20 x 19 x 55 mm in size and was in contact with the lateral wall of the esophagus. Histological examination showed that the tumor was composed of cells typical of adult rhabdomyoma. The patient has been followed in our outpatient clinic for more than the last 7 years. To our knowledge, this is the fifth case of adult mediastinal rhabdomyoma. We believe that rhabdomyoma is a rare tumor which should be considered in a differential diagnosis of tumors detected in the posterior mediastinum.

The use of endobronchial ultrasonography in assessment of bronchial wall remodeling in patients with asthma.

BACKGROUND: Endobronchial ultrasound (EBUS) is a new technique that enables the assessment of bronchial wall layers. The aim of the study was to verify the utility of EBUS for the assessment of bronchial wall remodeling in patients with asthma. METHODS: In 35 patients with asthma and 23 control subjects, high-resolution CT (HRCT) scanning and EBUS were used to measure bronchial wall thickness in the 10th segment of the right lung. With a radial 20-MHz probe, EBUS identified the 5-laminar structure of the bronchial wall. Layer 1 (L(1)) and layer 2 (L(2)) were analyzed separately, and layers 3 through 5 (L(3-5)), which corresponded to cartilage, were analyzed jointly. Digitalized EBUS images were used for the quantitative assessment of bronchial wall thickness and the wall area (WA) of the layers. Finally, bronchial biopsy specimens were taken for measuring the thickness of the reticular basement membrane (RBM). The thickness and WA of the bronchial wall layers, which were assessed using EBUS, were correlated with FEV(1) and RBM. RESULTS: There was no significant difference in the measurements of total bronchial wall thickness using EBUS and HRCT scanning. The thickness and WA of the bronchial wall and its layers were significantly greater in patients with asthma than in the control subjects. A negative correlation among the thicknesses of L(1), L(2), and L(3-5) and FEV(1), and a positive correlation with RBM were observed only in the patients with asthma. CONCLUSIONS: EBUS allows precise measurement of the thickness and WA of bronchial wall layers. The correlation of these parameters with asthma severity suggests implementation of EBUS in the assessment of bronchial wall remodeling in patients with asthma.

High-resolution computed tomography evaluation of peripheral airways in asthma patients: comparison of focal and diffuse air trapping.

BACKGROUND: Air trapping evaluated in high-resolution computed tomography (HRCT) reflects changes in small bronchi. We simultaneously evaluated focal and diffuse air trapping in asthmatic patients. OBJECTIVES: (1) To evaluate air trapping and bronchial wall thickness in asthmatics. (2) To estimate the relationship between air trapping and bronchial wall thickness, pulmonary function tests (PFTs), age, gender and asthma severity. (3) To compare air trapping between subgroups of asthmatic patients with normal FEV(1) % pred. and FEV(1)/FVC % and controls. (4) To compare air trapping and bronchial wall thickness between aspirin-induced asthmatics (AIA) and aspirin-tolerant asthmatics (ATA). METHODS: Both groups (asthmatics and controls) included 30 patients. All patients underwent HRCT and PFTs. RESULTS: Focal (p < 0.0001) and diffuse (p = 0.0004) air trappings and bronchial wall thickness (T: p < 0.0001; T/D: p < 0.0001; WA%: p < 0.0001) were significantly greater in asthmatics. Focal and diffuse air trappings were inversely correlated (p = 0.021). Diffuse air trapping correlated with bronchial wall thickness: T/D (p = 0.047), T (p = 0.037), and WA% (p = 0.048). There was a significant difference in the extent of focal air trapping between a subgroup of asthmatics with normal FEV(1) % pred. and FEV(1)/FVC % and controls (p < 0.0001). There were no significant differences in focal (p = 0.095) and diffuse air trapping (p = 0.186) and bronchial wall thickness (T: p = 0.086; T/D: p = 0.428; WA%: p = 0.428) between AIA and ATA patients. CONCLUSIONS: Both focal and diffuse air trappings provide valuable diagnostic information and therefore deserve to be estimated. The lack of significant differences in air trapping and bronchial wall thickness between AIA and ATA patients needs further investigation.

Actinomycosis mimicing advanced cancer.

Actinomycosis is a rare infectious inflammatory disease caused by the bacteria of the Actinomyces species. Infection occurs following damage to the skin or mucous membrane. This report presents a case of a 50-year-old female patient with subfebrile temperature, weight loss and pain, who used to have an intrauterine device. Masses suggestive of an advanced cancer were detected in her pelvis and abdominal cavity. A diagnosis of actinomycosis was made after histopathological examination of the tissue sample.

The role of zinc in thrombosis and pulmonary embolism in the course of antiphospholipid syndrome (APS)--short review.

Antiphospholipid antibodies may occur in the course of various diseases, but its presence is not necessarily associated with clinical symptoms. Zinc has multiple biological roles. For example, it stabilizes the cell's membrane and regulates its functions by influencing the synthesis of phospholipids and its distribution. The present review focuses on the possible associations between zinc and antiphospholipid antibodies and with the symptoms of antiphospholipid syndrome.

Role of zinc in hemostasis: a review.

Zinc is a multi-functional element that is found in almost 300 enzymes where it performs catalytic, co-catalytic, and/or structural functions. In 1982, Gordon et al. (Am J Clin Ntr 35:849-857, 1982) found that a low zinc diet caused poor platelet aggregation and increased bleeding tendency in adult males. This fact drew interest to the role of zinc in blood clotting. It has been shown that hyperzincemia predisposes to increased coagulability, and hypozincemia to poor platelet aggregation and increased bleeding time. The blood clotting disturbances can be regressed by appropriate zinc intake management. Considering the importance of zinc as an essential element, its participation in regulation of the equilibrium between pro- and anti-thrombotic factors originating in platelets and endothelium prompted further investigations.

Assessment of airway caliber in quantitative videobronchoscopy.

BACKGROUND: Quantitative assessment of airway caliber is generally confined to indirect methods. Fiberoptic bronchoscopy provides a direct view of the airways, but measurement of the internal size of bronchi in a standard examination is not possible. Using a special image analysis program, we developed a method allowing quantitative assessment of airway caliber by means of videobronchoscopic (VB) examination. OBJECTIVES: The purpose of the study was toshow that quantitative videobronchoscopy (VB coupled with a computer image analysis) allows direct and accurate measurement of the bronchi diameter. METHODS: To test our hypothesis, we measured the same areas of a bronchial tree in CT and in VB in 40 patients with diagnostic indications for both the procedures. RESULTS: We measured the diameters of 149 bronchi. The mean value of the difference between VB and CT measurements was equal to -0.071 mm and was not significantly different from 0 (p = 0.086). There was no obvious relation between the difference and the mean (r = 0.026, p = 0.745). The Bland Altman limits of agreement were L = -1.071 mm and U = 0.929 mm. We also assessed the bronchial diameter after endobronchial challenge and in patients with tracheobronchomalacia to show the application of this method for dynamic measurements. CONCLUSIONS: Quantitative videobronchoscopy allows the accurate and direct measurement of an airway caliber. It may be useful in clinical setting to quantify changes in a bronchial caliber (endobronchial masses, tracheobronchomalacia). Dynamic visualization of changes in airways may be useful in research, especially to explore the mechanics of airway narrowing.