Evaluation of prognostic factors for patients with eosinophilic granulomatosis with polyangiitis recruited at the pneumonological centre and mainly ANCA negativity: A retrospective analysis of a single cohort in Poland.

OBJECTIVES: The aim was to investigate the risk factors for relapse and death in patients with eosinophilic granulomatosis with polyangiitis (EGPA) recruited at the pneumonological centre and mainly antineutrophil cytoplasmic antibody negativity. METHODS: We retrospectively recruited 86 patients. Relapse was defined as the recurrence or appearance of new organ symptoms. The study end-point included the final examination. RESULTS: Relapses occurred in 34.9% of the patients, while 9.3% died. Immunosuppressive therapy (P = 0.042), prolonged low-dose corticosteroid treatments (mainly for asthma) (P = 0.006), and longer follow-up duration (P = 0.004) were associated with a higher relapse risk, while advanced EGPA severity (P = 0.0015) and activity (P = 0.044), older age of onset (P = 0.030), symptomatic cardiac involvement (P = 0.007), and postinflammatory cardiac fibrosis (P = 0.038) were associated with a higher risk of death. Sinusitis (P = 0.028) and prolonged low-dose corticosteroid treatments (P = 0.025) correlated with a better prognosis. Relapses did not have an impact on the mortality (P = 0.693). CONCLUSIONS: Relapses in EGPA remain frequent, although they do not impact mortality. Cardiac involvement is common, but clinically symptomatic cardiomyopathy is associated with a higher risk of death. Asthma requiring chronic corticosteroid treatments is associated with a lower risk of death, although the risk of EGPA recurrence is significantly higher.

A multicentre retrospective observational study on Polish experience of pirfenidone therapy in patients with idiopathic pulmonary fibrosis: the PolExPIR study.

BACKGROUND: Pirfenidone is an antifibrotic agent approved for the treatment of idiopathic pulmonary fibrosis (IPF). The drug is available for Polish patients with IPF since 2017. The PolExPIR study aimed to describe the real-world data (RWD) on the Polish experience of pirfenidone therapy in IPF with respect to safety and efficacy profiles. METHODS: This was a multicentre, retrospective, observational study collecting clinical data of patients with IPF receiving pirfenidone from January 2017 to September 2019 across 10 specialized pulmonary centres in Poland. Data collection included baseline characteristics, pulmonary function tests (PFTs) results and six-minute walk test (6MWT). Longitudinal data on PFTs, 6MWT, adverse drug reactions (ADRs), treatment persistence, and survival were also collected up to 24 months post-inclusion. RESULTS: A total of 307 patients receiving pirfenidone were identified for analysis. The mean age was 68.83 (8.13) years and 77% were males. The median time from the first symptoms to IPF diagnosis was 15.5 (9.75-30) months and from diagnosis to start of pirfenidone treatment was 6 (2-23) months. Patients were followed on treatment for a median of 17 (12-22.75) months. Seventy-four patients (24.1%) required dose adjustments and 35 (11.4%) were chronically treated with different than the full recommended dose. A total of 141 patients (45.92%) discontinued therapy due to different reasons including ADRs (16.61%), death (8.79%), disease progression (6.51%), patient's own request (5.54%), neoplastic disease (3.91%) and lung transplantation (0.33%). Over up to 24 months of follow-up, the pulmonary function remained largely stable. The median annual decline in forced vital capacity (FVC) during the first year of pirfenidone therapy was -20 ml (-200-100) and during the second year was -120 ml (-340-30). Over a study period, 33 patients (10.75%) died. CONCLUSIONS: The PolExPIR study is a source of longitudinal RWD on pirfenidone therapy in the Polish cohort of patients with IPF supporting its long-term acceptable safety and efficacy profiles and reinforce findings from the previous randomised clinical trials and observational studies.

Lung Function in Pregnancy in Langerhans Cell Histiocytosis.

Pulmonary Langerhans cell histiocytosis (LCH) is a rare disease, affecting usually young people. The course of the disease is variable. In some pulmonary LCH patients a severe lung destruction and progression in spite of chemotherapy is observed, but in others just a cessation of smoking induces a regression of the disease. In the present study we seek to determine the influence of pregnancy on pulmonary function in LCH patients, an unchartered area of research. We addressed the issue by investigating eight pregnant women out of the 45 women hospitalized with the diagnosis of pulmonary LCH in the period from 2000 to 2015. For five of the eight pregnant women it was the second gestation. The median follow-up period was 120 months (range 72-175 months). Ten healthy children were born by a C-section. Two spontaneous miscarriages in the seventh week of gestation, and one tubal ectopic pregnancy were recorded. We found that pregnancy did not significantly influence pulmonary function assessed by the following indices: forced expiratory volume in 1 s (FEV1), lung vital capacity (VC), total lung capacity (TLC), residual volume (RV), diffusing capacity of the lungs for carbon monoxide (DLCO), and the distance and arterial oxygen saturation in 6-min walk test. Only one patient in the third trimester of pregnancy experienced bilateral pneumothorax, with persistent air leak. In all patients, delivery and postpartum period were uneventful. We conclude that pregnancy in pulmonary LCH patients is safe and not associated with deterioration of pulmonary function or blood oxygenation.

Cardiac Sarcoidosis: Is it More Common in Men?

INTRODUCTION: Sarcoidosis is a systemic granulomatous disease which predominantly affects the lungs, although granulomas can also involve all other organs, including the heart. Cardiac sarcoidosis (CS) may occur at any stage of the disease and may be the cause of sudden cardiac death, even in a previously asymptomatic patient. The aim of this study was to evaluate the incidence of CS in a large group of patients diagnosed or followed up due to sarcoidosis. METHODS: We performed a retrospective analysis of patients at our institution discharged with the final diagnosis "sarcoidosis" (ICD-10: D86) from January 2008 to October 2012. Only those with biopsy (from respiratory tract or lymph nodes) confirmed diagnosis of sarcoidosis were included. We then selected the subset of patients with cardiac involvement due to sarcoidosis confirmed by positive magnetic resonance imaging. RESULTS: The study covered 1375 consecutive sarcoidosis patients (51 % men), who were hospitalized during 5 years. Multiorgan disease was detected in 160 cases (11.7 %), and cardiac involvement was found in 64 patients (4.7 % of all), 70.3 % of whom were men. Twelve of those with CS were in stage I, 48 in stage II, and four in stage III. The odds ratio for having cardiac involvement in men compared to women was 2.3 (95 % CI 1.36-4.0, p = 0.002). CONCLUSIONS: Cardiac involvement in sarcoidosis was diagnosed in the similar percentage as in previously published data but was significantly more frequently in men.

Interstitial lung disease associated with docetaxel in a patient treated for breast cancer - a case report.

Docetaxel is a semisynthetic cytostatic drug that belongs to the family of taxoids. Docetaxel inhibits normal interphase and mitotic cellular function, causing cell death. Docetaxel is indicated for the treatment of breast, lung and prostate cancers, head and neck cancer and gastric adenocarcinoma. Interstitial pneumonitis is an uncommon side effect of docetaxel. We report a case of docetaxel induced interstitial lung disease (ILD) in a patient with breast cancer.

Organizing pneumonia appearing in B-cell chronic leukemia malignancy progression - a case report.

Patients with chronic lymphocytic leukemia or non-Hodgkin's lymphoma are at risk of infectious diseases of respiratory system because of immunodeficiency. Occurrence of organizing pneumonia in leukemic patients is most commonly correlated with bone marrow transplant or treatment with antimitotic agents. There have been only four reported cases of organizing pneumonia related solitarily to leukemia or lymphoma. We present a case of 65-year old gentlemen, diagnosed 8 months earlier with B-cell chronic lymphocytic leukemia with no previous hematologic treatment, who presented symptoms of persistent pneumonia with no significant reaction to antibiotics. Chest computed tomography scans showed well-localized consolidation with ground glass opacities and some air bronchogram, suggesting infectious disease. All results of microbiological examinations were negative. Due to radiological progression of parenchymal consolidation despite two intravenous courses of antibiotics open lung biopsy was performed. The histologic examination of lung specimen revealed structures typical for organizing pneumonia pattern. There was no evidence for leukemic involvement in lung tissue, as no sign for infectious factors from histological staining was observed. In the inferior mediastinal lymph node sample progression of chronic lymphatic leukemia to mixed cell lymphoma was diagnosed. Patient was commenced on prednisone 60 mg/daily with fast improvement. We believe that this is the first case of organizing pneumonia as a reaction to the conversion of B-cell chronic lymphocytic leukemia progression to more malignant stage.

Atypical image of pulmonary alveolar proteinosis - a case report.

Pulmonary alveolar proteinosis is a very rare interstitial lung disease caused by abnormal intra-alveolar surfactant accumulation. Usually, it appears as a "crazy-paving" pattern on high-resolution computed tomography. The image is so typical, that together with the characteristic bronchoalveolar lavage examination with presence of Periodic Acid Schiff positive substance is sufficient for establishing diagnosis, without histological confirmation. We present the case of the young woman with severe dyspnoea suspected of acute hypersensitivity pneumonia. The computed tomography showed numerous intralobular nodules uniformly distributed troughout the lungs. Treatment by corticosteroids had no clinical effect and next computed tomography showed progression. Despite the high risk of complications (patient had a respiratory failure), a surgical lung biopsy was performed and the histopathological diagnosis of pulmonary alveolar proteinosis was made. The whole lung lavage procedure performed twice caused regression of radiological lesions and respiratory failure.

[Obliterative bronchiolitis due to inhalation of toxic gases and fumes--case report]. / Zarostowe zapalenie oskrzelików w wyniku inhalacji toksycznych gazów i oparów--opis przypadku.

Obliterative bronchiolitis is a rare pulmonary disease, characterised by narrowing and eventual obliteration of bronchioles by peribronchial and submucosal fibrosis. One of the identified causes of bronchiolitis is acute injury due to inhalation of toxic gases and fumes. Physiological criteria, essential in preliminary diagnostics, include irreversible airflow limitation, forced expiratory volume in 1 second (FEV1) < 60%, and exclusion of other causes of airflow obstruction. Surgical lung biopsy with histologic examination confirms diagnosis definitely. Prognosis of obliterative bronchiolitis, irrespective of aetiology, is rather poor, and treatment is rarely efficacious. We present a young chemist exposed to inhalation of toxic gases and fumes due to lack of usage of any personal protective equipment. He was referred to our lung disease department because of shortness of breath on exertion and irreversible airflow limitation. Definitive diagnosis of obliterative bronchiolitis was established by histological examination of specimen from open lung biopsy.

The epidemiological situation of tuberculosis in Poland: Part I. According to notification rates, the incidence of tuberculosis varies in different regions of Poland: is this true?

INTRODUCTION: In 2012 the incidence rate of tuberculosis in Poland was 19.6/100,000 but these was great variability between regions concerning notification rates (from 10.9/100,000 to 30.2/100,000). The aim of the study was to assess whether there are elements that might confirm that these differences are true. To answer this question, we compared the population of TB patients from regions with higher notification rates to the population of patients from regions with lower notifications rates. The data collected during three consecutive years were analysed. We selected for comparison the regions with the lowest and highest notification rates and those in which the notification rates for 3 years (2010-2012) were relatively stable. MATERIAL AND METHODS: Eight regions were chosen: three regions (Group I) with high notification rates (from 23.7 to 32.3/100,000 - mean rates in the analysed period of time) and five (Group II) with low notification rates (mean rates from 12.2 to 18.6/100,000). RESULTS: It was found that the proportion of sputum culture-positive patients was significantly higher in Group II. Thus, the difference in the notification rate of cases with culture-confirmed tuberculosis was smaller than the difference in the whole notification rate. Nevertheless, it was still significant. Tubercle bacilli in patients from Group I were significantly more often resistant to one drug. The incidence of chronic fibro-cavernous disease and of tuberculous pneumonia was significantly higher in Group I. The proportion of patients with symptoms was higher in Group I than in Group II. In addition, patients in Group I had the so-called primary tuberculosis (tuberculous pleuritis and tuberculous lymphadenopathy in the chest) significantly more often. It was also found that among patients from Group I there were significantly more children, more (though not significantly) youngsters and significantly fewer elderly patients. CONCLUSIONS: Based on these observations, it was concluded that there is a real difference in the epidemiological situation of tuberculosis in the selected regions of Poland with high and low rates of notification. Possible causes of this situation will be presented in a following publication.

Epidemiological situation of tuberculosis in Poland: part II. What are the causes of the different epidemiological situation in various regions of Poland?

INTRODUCTION: The different epidemiological situation of tuberculosis in various regions of Poland (higher and lower notification rates) was described previously by our group. The patients diagnosed with tuberculosis in the higher notification rate areas were younger and there were more cases of primary tuberculosis (tuberculous pleurisy and tuberculosis of chest lymph nodes) than in the patients diagnosed in the lower notification areas. The aim of the present study was to assess the possible causes of the different epidemiological situation of tuberculosis in various regions of Poland. MATERIAL AND METHODS: Analysis was done at the same regions as in the previous paper. A comparison was made between two groups: Group I, which included three voivodeships with higher rates of notification, from 23.7 to 32.3/100,000 (mean rates in the analysed period of time); and Group II, which included five voivodeships with lower notification rates (mean rates from 12.2 to 18.6/100,000). The wealth of the regions (GDP, gross domestic product per capita), the level of unemployment, and social status of the patients were analysed. We compared the population density in both regions. The results of treatment in both regions were also analysed. RESULTS: We did not find any differences in GDP and unemployment rates between the compared regions. The results of treatment were different in particular regions, but there was no clear tendency for worse results in voivodeships in Group I compared to voivodeships in Group II. However, the number of patients lost from observation was significantly higher in the regions from Group I than in those from Group II. There was also a significantly higher death rate from tuberculosis in younger patients (£ 59 years) from Group I than from Group II. This is additional proof that the epidemiological situation in the two regions was different. Finally, we found that the mean density of population in the regions from Group I was higher than that from Group II. The density of population may influence transmission of tuberculosis. There is also the possibility that the differences in the epidemiological situation in various regions of Poland are caused by historical events. In the past the epidemiological situation of tuberculosis was much worse in the east of Europe than in the west. Just after the Second World War, according to the changes of the Polish territory, many Polish citizens (mainly ancestors of those from Group I) were displaced from the east to the west. CONCLUSIONS: In conclusion, the greater number of patients lost from observation, together with the higher density of population in the regions from Group I in comparison with those from Group II, seems to be partly responsible for the difference in the epidemiological situation in the two regions. It is also possible that some patients from Group I are more susceptible to infection and disease caused by Mycobacterium tuberculosis due to their ancestors, who lived in the east of Europe.

[Liposarcoma--a rare primary neoplasm of the thorax]. / Tluszczakomiesak--rzadki pierwotny nowotwór w klatce piersiowej.

Liposarcoma is a rare tumour localised within the thorax. It can originate from a different thoracic structures (for example: lung parenchyma, mediastinum, pleura) or thoracic wall. We present a case of a 35-year-old woman with a giant tumour of the left hemithorax, who had two weeks history of non-productive cough and progressive dyspnoea from eight months. Chest radiography showed a large, round opacity in the left hemithorax, which displaced the cardiac silhouette to the right. Contrast-enhanced computed tomography showed a huge, heterogeneous, well-circumscribed mass in the left pleural cavity. The tumour expanded locally towards the thoracic wall and left lung, and displaced mediastinal structures to the right. There was no lymphadenopathy on the physical examination and CT scan. Abdominal ultrasonography was normal. There was no proof of tumour in the extremities and trunk. The patient underwent surgical excision of the tumour via thoracotomy, but because of tumour infiltration, part of the third rib and surrounding wall were resected. There was no evidence of lung and mediastinum involvement. The mass measured 17.5 × 18 × 10 cm and weighed 1690 g. A final diagnosis of a well-differentiated liposarcoma - sclerosing subtype - was established after histological and immunohistochemical staining. In our opinion, the liposarcoma in this case originated from the chest wall. Surgical resection was the only treatment. During six months after surgery the patient had no evidence of disease progression.

[Pneumocystis pneumonia in HIV-infected patients with cytomegalovirus co-infection. Two case reports and a literature review]. / Pneumocystozowe zapalenie pluc u zakazonych HIV przy wspólistniejacym zakazeniu cytomegalowirusem. Opis dwóch przypadków i przeglad literatury.

Pneumocystis pneumonia (PCP) is one of the infectious lung diseases diagnosed in HIV-infected patients. The pathogen responsible for the development of this opportunistic infection is an atypical fungus called Pneumocystis jiroveci. PCP remains the most common disorder diagnosed at the onset of acquired immunodeficiency syndrome (AIDS), especially in individuals not aware of their HIV infection. The most important risk factor of PCP development in HIV-infected person is the decrease of T CD4+ cell number below 200/mcL. Clinical symptoms consist of: chronic cough, dyspnoea and weakness. Arterial blood gas analysis often reveals the presence of hypoxaemia, and high-resolution computed tomography imaging shows diffuse ground glass opacities. Treatment is based on intravenous administration of trimetoprim-sulfamethoxasole. In patients with moderate and severe symptoms of PCP it is recommended that corticosteroids are used. Co-infection with cytomegalovirus (CMV) is a poor prognostic sign in PCP. CMV-related organ disease should be suspected in patients with T CD4+ cell number lower than 50/mcL. It is arguable whether CMV infection in symptomatic PCP patients should be treated. There are suggestions that corticosteroids used in PCP patients with CMV co-infection could promote the development of CMV pneumonia. In the present paper we present two patients with PCP, unaware of their HIV infection. In both cases a CMV co-infection was found.

[Interferon-Gamma Release Assays in the diagnosis of latent tuberculosis infection in clinical situations]. / Testy IGRA w diagnostyce zakazenia pratkami gruzlicy w wybranych sytuacjach klinicznych.

Until recently, the basic test to identify latent tuberculosis infection (LTBI) was the tuberculin skin test, despite its limitations in the form of low sensitivity and specificity. Currently, Interferon Gamma Release Assays from peripheral blood are used for a rapid diagnosis of LTBI and measurement of the interferon gamma (IFN-g) levels secreted by specific T cells stimulated with Mycobacterium tuberculosis antigens. Detection of LTBI is important in the control of people potentially at risk of TB disease, such as people remaining in close contact with BK (+) tb patient and for patients evaluated for biological treatment. The paper presents the value of IGRA in three selected clinical situations: in two cases of latent tuberculosis infection and in one case of active tuberculosis.

Accuracy of FDG PET/CT in the evaluation of solitary pulmonary lesions - own experience.

INTRODUCTION: In recent years, positron emission tomography (PET) has been increasingly applied in the diagnosis of neoplastic lung diseases. In contrast to conventional imaging studies, PET-CT enables the visualisation of not only the morphology of the suspicious lesion, but also its metabolism. The aim of the present study was to investigate the role of PET-CT in the initial assessment of patients with indeterminate solitary pulmonary lesions. MATERIAL AND METHODS: The study was conducted on a group of 82 patients with indeterminate lung nodule diagnosed at the National Institute of Tuberculosis and Lung Diseases in the period from January 2008 to May 2011. CT and PET-CT were performed in all of the patients. Histological or cytological examination of the biopsy specimens obtained from bronchoscopy, mediastinoscopy and intraoperatively were the reference tests. RESULTS: Malignancy was documented in 40 patients (48.8%). Histopathological analysis of all tumours revealed 12 cases of squamous cell carcinoma, 18 cases of adenocarcinoma and 1 case of carcinoid, whereas in 9 patients the diagnosis of "non-small cell cancer not otherwise specified" was made. All lesions except one were of solid character on chest CT. SUV(max) values exceeding 2.5 were found in 38 cancer patients (true positives, TP). The mean value of SUV(max) was 9.1 (1-26.8). Forty-two lesions were documented as benign (51.2%). SUV(max) values equal to or less than 2.5 were found in 37 patients (true negatives, TN). The mean value of SUV(max) in this group was 1.9 (0.5-8.6). The diagnostic value of PET-CT SUV(max) exceeding 2.5 in the prediction of neoplastic origin of solitary pulmonary lesions was: sensitivity - 95% (95% CI 84-99%), specificity - 88% (95% CI 75-95%) and accuracy - 91.5% (95% CI 83-96%). Positive predictive value (PPV) was 88.4% (95% CI 76-95%), and negative predictive value (NPV) was 94.8% (95% CI 83-99%). False negative results concerned two patients, with final diagnosis of carcinoid and adenocarcinoma; false positive results were obtained in 5 patients with various inflammatory lesions. CONCLUSIONS: In the present study, PET-CT appeared to have high sensitivity (95%), but lower specificity (88%) for predicting the malignant character of solitary pulmonary lesions. Overall diagnostic value of PET-CT SUV(max) > 2.5 was high - PPV was 88.4%, NPV was 94.8%. In the authors' opinion, the PET-CT value may increase when clinical data as well as other radiological documentation (with retrospective assessment) are taken into consideration.

Pulmonary alveolar proteinosis during a 30-year observation. Diagnosis and treatment.

INTRODUCTION: Pulmonary alveolar proteinosis (PAP) is a rare disease characterised by the abnormal accumulation of surfactant-like material in macrophages within the alveolar spaces and distal bronchioles. The course of the disease is variable and the prognosis is often good. However, progressive disease in some patients can cause respiratory dysfunction and can be life threatening. In this situation, the only effective treatment is whole lung lavage. The objective of the study was to present the characteristics and the course of pulmonary alveolar proteinosis in our own material, the diagnostic methods used, the indications for treatment and the treatment efficacy. MATERIAL AND METHODS: Retrospective analysis included 17 patients: 6 women and 11 men, aged from 32 to 56 years, who were observed in the Third Lung Department of Pneumonology at the National Institute of Tuberculosis and Lung Diseases between 1984 and 2013. In all patients chest X-ray, pulmonary function test and blood gases were performed. In 15 patients, high-resolution computed tomography (HRCT) was obtained. Bronchoscopy was performed in all of the patients, and in 7/17, bronchoalveolar lavage (BAL) was carried out. Fourteen patients underwent open lung biopsy. The indications for whole lung lavage (WLL) were progression of dyspnoea with restriction of daily activity and/or hypoxaemia. RESULTS: In most of the patients (13/17) the diagnosis was established outside our institute. Patients were referred to our department to establish further procedures. The criteria of diagnosis of PAP in most patients (16/17) was the histological examination of lung tissue, obtained by open lung biopsy (14 cases) and transbronchial lung biopsy (TBLB) (2 cases). Only in one patient the diagnosis was established on the basis of BAL. HRCT imaging was characteristic of proteinosis in 11/15 patients, and BAL examination in 6/7 patients, in whom BAL was performed. In four patients, who had been exposed to injurious factors for many years, secondary proteinosis was recognised; in other patients, no exposure or no other disease was found, and primary alveolar proteinosis was diagnosed. In one patient granulocyte macrophage colony stimulating factor autoantibody was detected. The majority of patients (10/17) had clinical symptoms at the diagnosis. The most commonly reported was dyspnoea, followed by respiratory tract infections. The most common abnormality (12/17) in pulmonary lung test was a decrease of diffusing capacity of the lung for carbon monoxide (DLCO). Respiratory distress at rest was found in two patients. Patients were observed for the period of 6 months to 19 years. Spontaneous partial remission was observed in 10 out of 13 untreated patients, including one complete remission; in 3 cases stabilisation was found in radiological examinations; and in other 4 patients, whole lung lavagewas used, resulting in clinical improvement with partial resolution of lesions in radiological examinations in 3 patients. In one patient, despite WLL being repeated three times, improvement was not achieved. CONCLUSIONS: Pulmonary alveolar proteinosis is a rare interstitial disease with a mild course in most cases. In 13/17 patients diagnosis was based on histological examination of samples from open lung biopsy. The presented patients were observed in the years 1984-2004, and at that time histologic examination was the main diagnostic method. The most common abnormality in pulmonary function tests was decrease of DLCO. In most cases, spontaneous remission of the disease was observed. In four patients with severe course of PAP, WLL was performed with subjective, functional and radiological improvement in 3 of them.

Severe congestive heart failure as the main symptom of eosinophilic granulomatosis and polyangiitis (Churg-Strauss syndrome).

Patients with cardiovascular symptoms are mainly diagnosed in cardiological wards. However, sometimes the other reasons for acute coronary syndrome and heart failure are found. One of such reasons is hypereosinophilia which can be recognized if number of blood eosinophils exceeds 1500/mm3. High eosinophilia is connected with production of cytotoxic eosinophilic proteins which can cause eosinophilic vasculitis or eosinophilic myocarditis. One of the better known hypereosinophilic syndromes is EGPA described by the pathomorphologists Churg and Strauss. The further research works allowed for the clinical characteristics of patients with EGPA. In the course of this disease the following three phases were recognized : prodromal-allergic, eosinophilic, vasculitic. The definitive diagnosis can be established only in the third phase, when vasculitis causes organ involvement. Besides symptoms of the respiratory tract (asthma, nasal polyps, eosinophilic lung infiltrations) also cardiovascular symptoms, gastrointestinal tract symptoms, as well as skin lesions and kidneys involvement can appear. The most dangerous for patients is involvement of the nervous and cardiovascular systems. We present a patient with asthma and eosinophilia in whom EGPA was diagnosed in the course of acute recurrent substernal chest pain, with subsequent signs of cardiac insufficiency.

[Extrapulmonary tuberculosis in Poland in the years 1974-2010]. / Gruzlica pozaplucna w Polsce w latach 1974­2010.

INTRODUCTION: The incidence of tuberculosis in Poland decreased from 128.5 in 100 000 in 1970 to 19.1 in 100 000 in 2010. In many countries, but not in Poland, according to the improvement of the epidemiological situation of tuberculosis (TB), the proportion of the extrapulmonary form of this disease is increasing. The aim of this study was to describe changes in extrapulmonary TB epidemiology in Poland from 1974 to 2010. MATERIAL AND METHODS: Retrospective analysis of data from National TB Register on tuberculosis in Poland in the years 1974-2010. The percentage of extrapulmonary tuberculosis among all cases of tuberculosis, the differences in the proportion of extrapulmonary tuberculosis, and differences in various locations of lesions in this form of disease in relation to sex and age groups were assessed. Information was collected from about 626,093 cases of tuberculosis reported to the Register during the period 1972-2010. In 62,251 cases extrapulmonary tuberculosis was the only form of the disease (9.9% of all tuberculosis cases). The study material consisted of 396,344 male and 196,184 female cases; 30,885 subjects were 0-19 years of age, 191,542 were 20-39 years old, 237,256 were 40-59 years old, and 166,410 subjects were ≥ 60 years old. We compared data from the years 1974-1982 with more recent data (2002-2010). The test of proportions for two independent samples was used to assess the significance of differences in proportions. RESULTS: The proportion of extrapulmonary tuberculosis (EPTB) among all TB cases was 11.2% in the years 1974-1982 and only 8.2% in the years 2002-2010. This difference was significant. The proportion of EPTB among all TB cases was higher in women than in men and was higher in people aged 0-19 years than in other age groups. The location of extrapulmonary tuberculosis was different in women and in men. Pleural TB was the most common form of extrapulmonary tuberculosis in both sexes with a predominance of males. Peripheral lymph nodes, bones and joints, urinary, genital, and skin TB were more common in women (these differences were significant). In the youngest age group intrathoracic lymph node TB dominated, and in the older age groups it was pleural TB. In accordance with the increase in age, the proportion of urinary tract, peripheral node, and pericardium TB increased. Central nervous system TB showed no predilection for a particular age group. CONCLUSIONS: The proportion of EPTB to all cases of TB is lower in Poland than in other countries. In addition, the proportion of EPTB had significantly decreased from 1974-1982 to 2002-2010. It may be due to difficulties in diagnosis of those forms of TB in Poland. Alternatively it is possible that those cases diagnosed in non-respiratory centres are not reported to the National TB Register.

[Diffuse alveolar hemorrhage as the only manifestation of antiphospholipid syndrome]. / Rozlane krwawienie pecherzykowe jako jedyna manifestacja zespolu antyfosfolipidowego.

The antiphospholipid syndrome is characterized by the presence of antiphospholipid antibodies and the association of protean clinical manifestations as a result of both venous and arterial thrombosis. While pulmonary embolism (secondary to deep vein thrombosis) is common and well-known disturbance in antiphospholipid syndrome, recently there are growing number of case reports describing nonthrombotic lung pathologies in APS. We present here a young male with antiphospholipid syndrome, whose the only manifestation was diffuse alveolar hemorrhage.

[Fibrosing mediastinitis with pulmonary hypertension as a rare complication of sarcoidosis]. / Wlóknienie sródpiersia z nadcisnieniem plucnym jako rzadkie powiklanie sarkoidozy.

Fibrosing mediastinitis is a rare disease, mostly associated with previous granulomatous diseases, such as histoplasmosis, tuberculosis, or sarcoidosis. We present a case of 42-year-old woman with sarcoidosis, who had developed fibrosing mediastinitis and pulmonary hypertension. Contrast-enhanced computer tomography showed abnormal, bilateral, solid tissues surrounding the hila and mediastinum. Magnetic resonance imaging showed: abnormal, intermediate signal tissue in the mediastinum, surrounding hila, narrowing both lower lobe arteries and both lobe bronchi, left upper lobe atelectasis, and contrast enhancement of mediastinal infiltration. The patient was treated with steroids for 14 months (initial dose of prednisone was 1 mg/kg/day, then tapered) with clinical and radiological improvement. We reviewed the literature concerning fibrosing mediastinitis. The various causes of that disorder, the radiological manifestation, and possible treatment modalities are discussed. The causes of pulmonary hypertension in our patient are another aspect of the article. It could be associated with both the fibrosing mediastinitis and the sarcoidosis.

Association of genetic dependences between lung cancer and chronic obstructive pulmonary disease.

INTRODUCTION: Recent studies have shown an increased risk of lung cancer in patients with bronchial obstructive changes, including patients with COPD. It seems that there are common factors of pathogenesis of both diseases associated with oxidative stress. In the present paper the genes linked to the repair of oxidative damage of DNA, associated with cancer, of iron metabolism and coding proteolytic enzymes were assessed. MATERIAL AND METHODS: The study was conducted in two groups of patients: 53 patients with non-small cell lung cancer and chronic obstructive pulmonary disease, and 54 patients only with chronic obstructive pulmonary disease. The polymorphisms of the single nucleotide were determined in the case of the majority of genes using the PCR-RFLP method. The statistical analysis of quantitative variables was executed using the Mann-Withney U-test and the test of medians; the analysis of genetic variables was executed using the chi² test. RESULTS: Regarding the polymorphisms of genes involved in iron metabolism, statistically significant differences between the two groups have been demonstrated only in the case of haptoglobin gene HP1/2. A higher incidence of form 1/1 was found in patients with COPD and a higher incidence of form 1/2 in patients with lung cancer and COPD. Analysis of gene polymorphisms of proteolytic enzymes and inhibitors of the enzyme gene showed statistically significant differences between the two groups only for the MMP3 gene 6A/5A. In the case of the MMP12 gene polymorphism (A-82G) a tendency toward differences in the occurrence of specific alleles was identified. CONCLUSIONS: These results indicate that patients with coincidence of COPD and lung cancer have disorders of the genes involved in iron metabolism, and they have different genetic polymorphisms of proteolytic enzymes comparing to COPD patients.