Chronic hypersensitivity pneumonitis is associated with an increased risk of venous thromboembolism: a retrospective cohort study.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) and chronic hypersensitivity pneumonitis share commonalities in pathogenesis shifting haemostasis balance towards the procoagulant and antifibrinolytic activity. Several studies have suggested an increased risk of venous thromboembolism in IPF. The association between venous thromboembolism and chronic hypersensitivity pneumonitis has not been studied yet. METHODS: A retrospective cohort study of IPF and chronic hypersensitivity pneumonitis patients diagnosed in single tertiary referral center between 2005 and 2018 was conducted. The incidence of symptomatic venous thromboembolism was evaluated. Risk factors for venous thromboembolism and survival among those with and without venous thromboembolism were assessed. RESULTS: A total of 411 (259 IPF and 152 chronic hypersensitivity) patients were included (mean age 66.7 ± 8.4 vs 51.0 ± 13.3 years, respectively). There were 12 (4.6%) incident cases of venous thromboembolism in IPF and 5 (3.3%) in chronic hypersensitivity pneumonitis cohort. The relative risk (RR) of venous thromboembolism in chronic hypersensitivity pneumonitis was not significantly different to that found in patients with IPF (7.1 vs 11.8/1000 person-years, RR 1.661 95% CI 0.545-6.019, respectively). The treatment with systemic steroids (OR 5.38; 95% CI 1.65-18.8, p = 0.006) and GAP stage 3 (OR 7.85; 95% CI 1.49-34.9; p = 0.037) were significant risk factors for venous thromboembolism in IPF. Arterial hypertension and pulmonary hypertension significantly increased risk of venous thromboembolism in chronic hypersensitivity pneumonitis. There were no significant differences in survival between patients with and without venous thromboembolism. CONCLUSIONS: The patients with chronic hypersensitivity pneumonitis have a marked increase in the risk of venous thromboembolism, similar to the patients with IPF. Venous thromboembolism does not affect the survival of patients with IPF and chronic hypersensitivity pneumonitis.

Can a New Scoring System Improve Prediction of Pulmonary Hypertension in Newly Recognised Interstitial Lung Diseases?

INTRODUCTION: Pulmonary hypertension (PH) is a well-recognised complication of interstitial lung diseases (ILD), which worsens prognosis and impairs exercise capacity. Echocardiography is the most widely used, non-invasive method for PH assessment. The aim of our study was to identify the factors predictive for echocardiographic signs of PH in newly recognised ILD patients. METHODS: Ninety-three consecutive patients (28F/65M) with different ILD were prospectively evaluated from January 2009 to March 2014. Pulmonary function testing, 6-min walk distance (6MWD), initial and sixth minute room air oxygen saturation, NT-proBNP and echocardiography were assessed in each patient. Echocardiographic PH probability was determined according to the 2009 ESC/ERS guidelines. RESULTS: In 41 patients (Group B) increased PH possibility has been diagnosed on echocardiography, in 52 patients (Group A)-low PH probability. Most pronounced differences (p ≤ 0.0005) between groups B and A concerned: age, 6MWD, room air oxygen saturation at 6 min, DLCO and TLC/DLCO index (57.6 vs 43.8 years; 478 vs 583 m; 89.1% vs 93.4%; 54.8% predicted vs 70.5% predicted and 1.86 vs 1.44; respectively). Univariate analysis showed four-fold increased probability of PH when TLC/DLCO exceeded 1.67. A scoring system incorporating age, TLC/DLCO index, 6MWD and room air oxygen saturation at 6 min provided high diagnostic utility, AUC 0.867 (95% CI 0.792-0.867). CONCLUSION: ILD patients with TLC/DLCO index > 1.67 have a high likelihood of PH and should undergo further evaluation. The composite model of PH prediction, including age, 6-min walk test and TLC/DLCO was highly specific for recognition of PH on echocardiography.

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.

[Disturbances of calcium metabolism and vitamin D supplementation in sarcoidosis - two-way street]. / Zaburzenia gospodarki wapniowej a suplementacja witaminy D u chorych na sarkoidoze ­ dwie strony medalu.

The role of vitamin D in the human body is not limited only to the regulation of calcium metabolism and secondary to the impact on bones. Recent studies have shown the influence of vitamin D level on muscles, on the risk of cancer, diabetes, hypertension and pulmonary diseases, including granulomatous diseases. Sarcoidosis is a granulomatous disease of unknown etiology. Hypercalcemia in the course of the disease occurs in up to 10% of cases in the consequence of autonomous overproduction of 1,25-dihydroxyvitamin D by macrophages of sarcoid granulomas. Hypercalciuria occurs 3-fold more frequent. On the other hand, treatment with corticosteroids increases the risk of osteoporosis. Vitamin D intake is recommended for prevention of osteoporosis. Such management, in sarcoidosis patients, is not so clear because of risk of hypercalcemia. Vitamin D supplementation, according to current recommendations for general population, is based solely on 25-hydroxyvitamin D level testing. This seems to be not safe in the group of sarcoidosis patients. This article discusses the role of vitamin D in sarcoidosis patients and current opinion on vitamin D supplementation in this group.

[Hypersensitivity pneumonitis: Diagnostic criteria, treatment, prognosis and prevention]. / Alergiczne zapalenie pecherzyków plucnych - kryteria rozpoznania, leczenie, rokowanie i zapobieganie.

Hypersensitivity pneumonitis (HP) is caused by inhalation of environmental antigens. Farmers and bird keepers are most frequently affected by this desease. The HP diagnosis is based on clinical symptoms (cough, dyspnea) in a person exposed to environmental antigens, and the presence of characteristic changes in high resolution chest computed tomography (HRCT) (bilateral, mosaic, ground glass opacities in the middle and lower lung zones, ill-defined centrilobular nodules and the sign of air-trapping on expiration). This type of HRCT pattern is most frequently found in the patients with subacute HP. Bronchioloalveolar lavage fluid (BALF) examination is helpful in establishing the HP diagnosis, when the increased total number of cells, with the predominance of T lymphocytes (> 50%), and the increased number of neutrophils (> 3%) and mastocytes (> 1%) are found. The presence of specific serum precipitins increases the likelihood of HP. In case of atypical clinical presentation, lung biopsy is recommended. The diagnostic criterion of HP is the presence of ill-defined non-necrotising granulomas, after excluding other granulomatous lung diseases. The prevention and treatment of HP is based on the elimination of the antigen from the environment. Corticosteroids may contribute to the improvement in the acute and sub-acute form of the disease but their long term effectiveness is uncertain. The prognosis of HP patients is generally perceived as good, especially in those patients in whom antigen avoidance is possible. Nevertheless, in some patients progressive pulmonary fibrosis and development of severe respiratory insufficiency is observed. Med Pr 2016;67(4):517-527.

[Community acquired pneumonia - treatment options according to the international recommendations]. / Zasady leczenia pozaszpitalnego zapalenia pluc zgodnie z aktualnymi wytycxnymi.

Pneumonia remains one of the main reasons of heath care system utilization. Quick diagnosis and prompt treatment initiation determine favorable outcome. Empirical antibiotic treatment allows to achieve treatment success in most patients. Treatment recommendations are based on big epidemiological trials. Nevertheless, it is sometimes necessary to know the definite etiologic factor of pneumonia. In these cases microbiological diagnostics is useful, i.e. sputum microscopy and culture, blood culture, bronchial lavage culture, bacterial antigen tests in urine, molecular tests. Serological tests do not help much in everyday clinical practice. The most common microorganisms causing community acquired pneumonia (CAP) are: Streptococcus pneumoniae, atypical bacteria (Mycoplasma pneumoniae, Chlamydophila pneumoniae, Legionella pneumophila), Haemophilus influenzae, influenza virus. Staphylococcus aureus and Pseudomonas aeruginosa rarely are etiologic factors of CAP. First line antibiotics in pneumonia treatment are beta - lactams. In case of allergy or intolerance of beta - lactams, new fluorochinolones should be used. Macrolides are useful if the atypical etiology is suspected. Duration of treatment in most cases should not exceed 7 days, sometimes it may be even shorter.

Fungal infection of cystic fibrosis patients - single center experience.

INTRODUCTION: Cystic fibrosis (CF) is the most common monogenetic autosomal recessive disease in the human population. This systemic disease is characterized by changes in multiple organs, mainly in the lung tissue and digestive tract. More than 59% of CF patients become sensitized to fungal spores, mostly Aspergillus fumigatus. 5-15% of CF patients develop allergic bronchopulmonary aspergillosis. The aim of the study was to analyse the occurrence of yeast and filamentous fungi of the respiratory infections in CF patients and evaluation of drug resistance. MATERIAL AND METHODS: Between 2006 and 2014, mycological evaluation of 42 patients hospitalized at the National Institute of Tuberculosis and Lung Diseases was carried out. RESULTS: 217 specimens from pulmonary tract were collected from 42 patients with cystic fibrosis. 205 (68%) strains of yeast and 96 (32%) filamentous fungi strains were cultured. The most common mould strain was A. fumigatus - 22,2% (67 species). All isolates of filamentous fungi were in vitro 100% susceptible to itraconazole, voriconazole, posaconazole and amphotericin B. CONCLUSIONS: A. fumigatus and C. albicans were the most common etiological agents of fungal respiratory pathogens associated with CF patients. A. fumigatus strains were in vitro 100% susceptible to azole and amphotericin B. Two strains of C. albicans and one strain of C. tropicalis were non-susceptible to azole (fluconazole, itraconazole and voriconazole). Scedosporium apiospermum was resistant to amphotericin B (MIC > 32 mg/l) and susceptible to voriconazole (MIC 0.094 mg/l).

Centrilobular nodules in high resolution computed tomography of the lung in IPAH patients - preliminary data concerning clinico-radiological correlates.

INTRODUCTION: Inhomogeneity of lung attenuation pattern is observed in high resolution chest computed tomography (HRCT) in some IPAH patients despite lack of interstitial lung disease. Such radiological changes are described either as ill-defined centrilobular nodules (CN) or as focal ground glass opacities (FGGO). There is no consensus in the literature, whether they indicate the distinct type of IPAH, or pulmonary venoocclusive disease (PVOD) with subtle radiological changes. Thus the aim of the present pilot study was to assess the frequency and clinical significance of inhomogenic lung attenuation pattern in IPAH. MATERIAL AND METHODS: 52 IPAH patients (38 females, 14 males, mean age 41 years ± 15 years), entered the study. All available chest CT scans were reviewed retrospectively by the experienced radiologist, not aware about the clinical data of the patients. RESULTS: CN were found in 10 patients (19%), FGGO - in 12 patients (23%). No lymphadenopathy or interlobular septal thickening suggestive of PVOD were found. The significant differences between CN and the remaining patients included: lower mean age - 31 and 43.5 years, (p = 0.02), lack of persistent foramen ovale (PFO) - 0% and 43% (p = 0.03), and higher mean right atrial pressure (mRAP) - 12.5 mm Hg and 7.94 mm Hg (p = 0.01). No significant survival differences were observed between the groups of CN, FGGO and the remaining patients. CONCLUSION: Centrilobular nodules in IPAH were combined with lack of PFO, higher mRAP and younger age of patients.

Low DLCO in idiopathic pulmonary arterial hypertension - clinical correlates and prognostic significance.

INTRODUCTION: Decreased diffusing capacity of the lung for carbon monoxide (DLCO) is observed in some idiopathic pulmonary arterial hypertension (IPAH) patients, but its clinical significance is uncertain. We aimed to assess clinical correlates and prognostic significance of low DLCO in IPAH patients. MATERIAL AND METHODS: In the group of 65 IPAH patients the cut off value for low DLCO was set up based on histogram as < 55% of predicted value. Demographic data, exercise capacity, lung function tests, hemodynamic parameters and survival of the patients were compared depending on DLCO value. RESULTS: Low DLCO was found in 18% of the patients, and it was associated with male sex, older age, worse functional status and exercise capacity, and higher prevalence of coronary artery disease. Low DLCO carried a 4-fold increase of death risk in 5-year perspective. CONCLUSIONS: Low DLCO was a marker of worse functional capacity and increased risk of death in studied IPAH patients.

Tuberculosis in homeless persons in Poland.

UNLABELLED: The fall in rates of tuberculosis (TB) in many countries has been accompanied by the concentration of cases in the social risk groups including homeless persons. AIM: Comparison of TB features in homeless persons and in non-homeless patients. METHODS: TB cases reported to National TB Register in Poland in whom information about the social status was available (the data about the social status were collected obligatorily in the years 2004-2013 only) were analysed. The results of DSTs were obtained from laboratory records and were available for the cases reported since 2010. Treatment outcome after 12 months was analysed for the cases registered between 2004-2012. The significance (Si) of the differences in proportions was assessed with chi-square test. P<0.05 was regarded as statistically significant. Test F was used to evaluate the significance of differences of the means of age. The multivariate logistic regression models were applied to find out the independently operating determinants of not achieving of success of treatment. RESULTS: 2,349 homeless persons (HP) and 72,989 other patients (OP) with TB were included. In the group of HP, there was a greater proportion of males in comparison with OP (90.5% vs. 66.3%) (Si). The mean age of HP was 49.8 years (SD±10.9); of OP-52.9 years (SD±17.5) (Si). 16.6% of HP and 10.4% of OP were previously treated for TB (Si). The previous treatment was adequate in 62.2% of HP and in 85.8% of OP (Si). Pulmonary TB was in 98.0%, extrapulmonary TB in 2.0% of HP and, respectively, in 92.5% and 7.5% of OP (Si). Pulmonary TB was confirmed by culture in 76.3% of HP and in 64.5% of OP (Si). Sputum smears were positive in 70.7% of HP and in 62.5% of OP (Si). Caseous pneumonia occurred in 2.7% of homeless subjects and in 1.1% of OP (Si); infiltrative TB in 95.5% of HP and in 97.5% of OP (Si). Resistance to isoniazid was observed in 2.9% of HP and in 3.1% of OP; to rifampicin in 0.0% of HP and in 0.2% of OP; to isoniazid and rifampicin in 0.4% of HP and in 0.8% of OP. These differences were not Si. Treatment success rate among HP was 44.1%; default rate 24.8%; 4.0% of HP died from tuberculosis; 3.2% died from other causes; 5.2% were transferred and their outcomes were unknown; 0.4% were still on treatment; 0.4% had treatment failure; in 17.9% of HP results of treatment were not available. Among other TB patients the rate of success was 66.8%; of default--8.8%; 1.9% died from TB; 3.1% died from other causes; 2.6% were transferred; 0.5% were still treated; 0.2% had treatment failure; in 16.1% the results were not reported. Differences between both groups were Si, except for the category "died from other causes", "still on treatment" and "treatment failure". CONCLUSIONS: In the group of homeless persons with tuberculosis phenomena which indicate a delay in diagnosis of disease and in some cases the possibility of a recent infection and also unfavorable treatment outcomes occur in a greater proportion than among other patients. Targeted screening for TB and directly observed treatment could likely improve the epidemiological situation of tuberculosis in the population of homeless.

Pulmonary hypertension in the course of diffuse parenchymal lung diseases - state of art and future considerations.

Lung diseases are one of the most frequent causes of pulmonary hypertension (PH). The development of PH influences the course of lung disease, worsening the clinical symptoms and prognosis. According to the most recent publications, PH in the course of lung diseases develops as a result of both "parenchymal" and vascular pathology, in the patients with genetic predisposition. Prolonged infection (especially viral one) may be an additional promoting factor. Right heart catheterization (RHC), which is an invasive procedure, is the only objective method of diagnosing PH. According to the latest recommendations, the management algorithm of PH and coexisting interstitial lung disease is based on RHC and the results of pulmonary function tests. Majority of the patients develop mild PH in the course of advanced lung disease. Best treatment of underlying lung pathology combined with long term oxygen treatment is recommended in this group. In case of severe PH (mean resting pulmonary artery pressure (mPAP) ≥ 35 mm Hg) the alternate cause of PH has to be sought. PAH-specific drugs use should be limited to patients with severe PH participating in clinical trials. In this review, the value of various non-invasive methods (echocardiography, radiological examination, exercise capacity and brain natriuretic peptides assessment) in the process of screening for PH is presented, and the results of recent randomized clinical trials with PAH-specific drugs in patients with diffuse parenchymal lung diseases are discussed.

Interferon gamma release assays based on M. tuberculosis-specific antigens in sarcoidosis patients.

INTRODUCTION: This study is a part of the project on interferon gamma release assays performed in the group of untreated sarcoidosis patients formerly BCG vaccinated. The aim of the study was to assess the rate of positive commercial interferon g release assays in sarcoidosis patients. We discussed the results in the context of hypothesis that M. tuberculosis antigens may play a role in the pathogenesis of sarcoidosis. MATERIAL AND METHODS: 151 patients, mean age 38 ± 10.3, treatment naive, with newly diagnosed pulmonary sarcoidosis were enrolled into the study. All participants underwent QFT-GIT assay. A subgroup of 81 patients underwent also T-SPOT.TB assay. RESULTS: QFT-GIT was positive in 7/151. T-SPOT.TB was positive in 3/81. There were no indeterminate results in both IGRAs. There was no statistically significant relationship between IGRAs results and sarcoidosis parameters such as the radiologic stage, disease duration and the presence of Löfgren's syndrome. CONCLUSIONS: In sarcoidosis patients formerly BCG vaccinated, positive rate of IGRAs was 4.6% for QFT-GIT and 3.7% for T-SPOT. TB. We did not find the influence of the selected parameters of sarcoidosis on IGRAs results.

Non-high risk PE in the patients with acute or exacerbated respiratory disease: the value of the algorithm based on D-dimer evaluation and Revised Geneva Score.

INTRODUCTION: The diagnostic algorithm of non-high risk pulmonary embolism (PE) is based on probability scoring systems and plasma D-dimer (DD) assessment. The aim of the present study was to investigate the efficacy of Revised Geneva Scoring (RGS) and DD testing for the excluding of non-high risk PE, in the patients admitted to the hospital due to acute respiratory diseases. MATERIAL AND METHODS: The consecutive patients, above 18 years of age, referred to the department of lung diseases, entered the study. The exclusion criteria were: the pregnancy and the suspicion of high risk PE. Plasma DD was measured with quick ELISA test, VIDAS D-dimer New, bioMerieux, France. Multislice computed tomography angiography was performed in all of the patients. RESULTS: 153 patients, median age 65 (19-88) years entered the study. The probability of PE was: low - in 58 patients (38%), intermediate - in 90 (59%), high - in 5 (3%). DD < 500 ng/ml was found in 12% of patients with low and intermediate probability of PE. PE was recognized in 10 out of 153 patients (7%). None of the patients with DD < 500 ng/ml was diagnosed with PE (NPV 100%). Median DD value was significantly higher in PE patients comparing to non-PE (4500 ng/ml and 1356 ng/ml respectively, p = 0.006). CONCLUSION: In the group of the patients with acute respiratory symptoms, low or intermediate clinical probability scoring combined with normal DD had a high NPV in excluding PE. Nevertheless, such approach was not very effective, as the increased DD was noted in 88% of the examined population.

[Mycobacterial lung disease in patients with cystic fibrosis--report of three cases]. / Mikobakterioza u chorych na mukowiscydoze--opis trzech przypadków.

Mycobacterial lung disease is caused by nontuberculous mycobacteria (NTM), also known as atypical mycobacteria. NTM are widely distributed in the environment, particularly in soil and water. Although generally of low pathogenicity to humans, NTM can affect patients with underlying chronic lung diseases, such as cystic fibrosis, bronchiectasis, pneumoconiosis, or healed tuberculosis. Some patients with cystic fibrosis (CF) have disease progression due to NTM, others can have NTM cultured intermittently from respiratory specimens without a significant decline in lung function. Identifying which patients will worsen from NTM and therefore need treatment remains difficult because of the similarity of symptoms in CF and NTM lung disease. The most common species of NTM isolated in CF patients are Mycobacterium avium complex (MAC) and Mycobacterium abscessus. In this paper, we present three different cases of mycobacterial lung disease in patients with cystic fibrosis.

[A 24-year-old male patient with pulmonary veno-occlusive disease - case report]. / 24-letni pacjent z choroba zarostowa zyl plucnych - opis przypadku.

Pulmonary veno-occlusive disease (PVOD) is a rare cause of severe pulmonary hypertension characterised by poor prognosis. We report the case of a 24-year-old male patient with increasing dyspnea and exercise intolerance treated with calcium channel blockers and glucocorticosteroids, due to suspicion of pulmonary hypertension and interstitial lung disease, until lung biopsy was performed and a diagnosis of PVOD was established on the basis of the histological analysis of the lung biopsy sample. This case highlights that pulmonary veno-occlusive disease is a disorder that is difficult to diagnose and resistant to medical treatment, which is particularly poor prognostic factor. Due to poor response to medical therapy and high mortality in patients with PVOD, understanding the pathogenesis, differentiation with pulmonary arterial hypertension and the search for a new methods of treatment should be the key challenges for modern medicine.

[Acute interstitial pneumonia in patient with rheumatoid arthritis treated with leflunomide]. / Ostre sródmiazszowe zapalenie pluc u chorego na reumatoidalne zapalenie stawów leczonego leflunomidem.

Leflunomide (LEF) is an isoxazole derivative used as disease-modifying anti-rheumatic drug (DMARD) in the treatment of rheumatoid arthritis (RA). It is effective and safe in patients with active RA, in whom standard treatment is insufficient or contraindicated, but it can cause interstitial lung disease (ILD). Identified risk factors for LEF-induced ILD include pre-existing ILD, cigarette smoking, low body weight, and use of loading dose. LEF should be avoided in patients with pre-existing ILD. We present a case of 59-year-old male with RA and a history of smoking and methotrexate (MTX) treatment, who developed dyspnoea, non-productive cough, and fever about two months after the administration of LEF. The clinical and radiological presentation was of acute pneumonia. The patient was treated with methylprednisolone pulse, prednisone, and cyclophosphamide, but he died of respiratory failure.

Pulmonary pathology in patients with ulcerative colitis treated with mesalazine--a challenging and complex diagnostic problem. Case series and literature review.

Pulmonary involvement in the course of inflammatory bowel disease has been a subject of interest to clinicians for long time, but despite this, its epidemiology and potential pathomechanisms remain obscured. Equally unclear is the role of medications used for bowel disease treatment in lung disease development. We present three patients with ulcerative colitis, all treated with mesalazine, in whom unexplained lung disease developed. Due to different clinical and radiological presentation, different conditions were initially placed on the top of the differential list in each of them. The outcome was favourable in all patients despite differences in management. We compared our patients with similar cases from literature. We show the level of difficulty and complexity in the issue of lung disease in patients with inflammatory bowel disease.

Non-tuberculous mycobacterial lung disease (NT MLD ) in patients with chronic thromboembolic pulmonary hypertension and idiopathic pulmonary arterial hypertension.

INTRODUCTION: Non-tuberculous mycobacterial lung diseases (NTMLD) occur rarely and are diagnosed mainly in patients belonging to risk groups. Pulmonary hypertension (PH) has not been recognised as a risk factor for NTMLD yet. The aim of the study was to analyse the clinical course and predisposing factors of NTMLD recognised in our centre between 2002 and 2012 in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and idiopathic pulmonary arterial hypertension (IPAH). MATERIAL AND METHODS: Thirteen patients (10 - CTEPH, 3 - IPAH) entered the study. PH was recognised during right heart catheterisation. Median value of mean pulmonary artery pressure (mPAP) was 49 mm Hg (39-65 mm Hg). NTMLD was diagnosed according to ATS guidelines (2007). RESULTS: M. kansasii was the most frequent pathogen. Most patients complained of the exaggeration of dyspnoea and productive cough. Computed tomography of the chest with angiography revealed infiltrations with cavitation in seven patients and cavities surrounded by micronodules in six patients. In all CTEPH patients, NTMLD developed in the hypoperfused lung areas. No parenchymal abnormalities preceded the development of NTMLD. After diagnosis all of the patients received antituberculous treatment; in 12/13 improvement was achieved. By the end of March 2014 seven patients died due to right heart insufficiency, no deaths due to NTMLD were noted. CONCLUSIONS: NTMLD should be suspected in patients with CTEPH or IPAH, presenting with productive cough and a new pulmonary infiltrate with cavitation. In patients with CTEPH, special attention should be paid to a new cavitary lesions without accompanying thrombus in the artery supplying the area. High mPAP (CTEPH/IPAH) and hypoperfusion (CTEPH) are predisposing to NTMLD.

[Interstitial pneumonia connected with rituximab therapy--case report]. / Sródmiazszowe zapalenie pluc zwiazane z leczeniem rytuksymabem--opis przypadku.

Rituximab (RTX) is a monoclonal antibody against the CD20 antigen found on the surface of B cells. RTX causes cell lysis and is therefore used to treat lymphomas, leukaemias, transplant rejection and certain autoimmune disorders. Pulmonary adverse events associated with RTX have been reported in literature. We describe a patient with follicular lymphoma treated with R-CHOP scheme (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) who had pulmonary symptoms during chemotherapy which were diagnosed as rituximab-induced interstitial pneumonia. Symptoms and radiological changes resolved with prednisone therapy.