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Background: Pirfenidone and nintedanib are considered as the standard of care in idiopathic pulmonary fibrosis (IPF), but there is no consensus as to which of these two agents should be regarded as first-line treatment. Objective: To provide real-world data on therapeutic decisions of pulmonary specialists, particularly the choice of the antifibrotic drug in patients with IPF. Methods: This was a multicenter, prospective survey collecting clinical data of patients with IPF considered as candidates for antifibrotic treatment between September 2019 and December 2020. Clinical characteristics and information on the therapeutic approach were retrieved. Statistical evaluation included multiple logistic regression analysis with stepwise model selection. Results: Data on 188 patients [74.5% male, median age 73 (interquartile range, 68–78) years] considered for antifibrotic therapy were collected. Treatment was initiated in 138 patients, while 50 patients did not receive an antifibrotic, mainly due to the lack of consent for treatment and IPF severity. Seventy-two patients received pirfenidone and 66 received nintedanib. Dosing protocol ( p

Abstract 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.

Granulomas formations are present in many lung diseases. Coexistence of one or more of these diseases is very rare. Diagnostics of such cases always poses a challenge. We present a case of coexistence of chronic granulomatous disease (CGD) and sarcoidosis.

Idiopathic pulmonary fibrosis (IPF) is a rare disease with progressive course and a very unfavourable prognosis. Antifibrotic drugs are a chance to reduce the rate of disease progression and extend the life of IPF patients. One of these drugs is nintedanib, an oral tyrosine kinase inhibitor. In the following article, the reader will find a summary of current knowledge on the efficacy and safety of nintedanib treatment of IPF patients. This study uses data from pivotal studies and experience from everyday clinical practice indicating a wide range of possible applications of the drug in IPF patients.

Hypersensitivity pneumonitis (HP) is a complex syndrome caused by exaggerated immune response to inhalation of a variety of organic particles in susceptible individuals. In this study we assessed the relationship between age at the time of diagnosis and the degree of functional and radiological changes in HP. The diagnosis of HP was made on the basis of a combination of clinical symptoms, medical history, serological tests, radiologic evidence of diffuse lung disease, and absence of other identifiable causes of lung disease. We reviewed the records of 111 patients (68 women) diagnosed with HP over a period of 18 years (1995-2013). The patients were stratified into 3 age-groups: <30, 30-49, and ≥50 years old. The commonest cause of HP was avian antigens (56.8 %). Dyspnea was present in 97.3 % of patients, weight loss in 54.7 % of patients, and respiratory insufficiency in 24.3 % of patients. Lung fibrosis in chest computed tomography was found in 35.1 % of patients. Lung function was impaired more seriously in the youngest age-group, with lung diffusing capacity for carbon monoxide (DLCO) <40 % in 69.2 % of these patients. Restrictive pattern was present in 92.3 % of patients in this group, as compared with the 41.0 % in the whole cohort. In this group, desaturation in the six minute walk test also was most notable, amounting to a median of 11 %. In conclusion, diagnosis of HP at young age is predictive of a more severe clinical course of disease, with lung fibrosis and higher disturbances in pulmonary function.

Cardiac involvement in sarcoidosis may be incidentally discovered without any symptoms of the disease. When undiagnosed and untreated it is potentially fatal. Although there is no recommended strategy for the diagnosis, the introduction of newer technology is promising and may be useful for early diagnosis of sarcoid heart disease and for the evaluation of response to therapy. We present a case of 53-years old woman, with asymptomatic cardiac arrhythmias and x-ray chest picture changes without any respiratory symptoms. Further assessment and the use of cardiac magnetic resonance imaging allowed a diagnosis of cardiac sarcoidosis to be made.

Various types of non-tuberculous mycobacteria can be the aetiologic factors of chronic lung infections especially in patients with underlying chronic lung diseases. The aim of this study is to present the cases of pulmonary mycobacterioses observed in Institute of Tuberculosis and Lung Diseases in the years 1995-2001. There were 23 patients, 12 men and 11 women in the age between 35-77 years, mean 56 years. 16 out of 23 patients had underlying respiratory problems, mainly healed tuberculosis (7) and COPD (6). Two additional patients suffered from other diseases with potential immunosuppression (leukopenia). In 5 patients no disease other than mycobacteriosis was found, but they were chronic smokers. In 19 cases cough and expectoration of purulent sputum lasting from several months to several years was observed. In 5 patients onset of disease was acute or subacute with high fever. Eight patients had haemoptysis. In chest X-ray pathological lesions including (18 cases) lung cirrhosis (10) and cavities (15) were found. In 4 cases disseminated bronchiectases with small nodules were the main radiologic feature. Mycobacteriosis was caused by M. kansasii in 11 cases, by M. intracellularae in 6, by M. xenopi in 5 and by M. scrofulaceum in 1 case.

Pulmonary disorders in systemic lupus erythematosus are frequent and sometimes they are the first symptoms of the disease. SLE may cause a variety of clinical presentations and pathologic patterns, which can be difficult to diagnose. We observed 11 patients (9 women and 2 men) with pulmonary manifestations of SLE during last 18 years in our department. Mean age of patients was 47.7 +/- 13.4 years. There were no patients with drug induced SLE. Interstitial lung diseases (7/11) acute or chronic and pleural involvement (5/11) were the most frequent clinical presentation. In three cases airway disease presented as reduction of FEV1%VC index was detected. In one case "shrinking lung" syndrome was confirmed by muscle function (diaphragm relaxation time) and lung function tests. Pulmonary hypertension confirmed by echocardiography, was associated with interstitial lung disease or vasculitis. Six cases representing different lung involvement pattern of SLE were described in details.

Sarcoidosis is a multisystem disorder most frequently presenting with hilar lymphadenopathy, pulmonary infiltrations, ocular and skin lesions. However pulmonary manifestations typically dominate, any organ can be affected. Sometimes leading symptoms are caused by extrapulmonary manifestation of the disease, and together with the absence of typical picture in chest radiographs may be confusing for the physicians. We present 4 cases of proven sarcoidosis in which leading symptoms were caused by sarcoidal involvement of different organs (liver, spleen, heart and skin) without typical changes in the lung. In all cases multiorgan involvement was documented and disease was successfully treated.

Work place environment plays an important role in development diseases because of the time spend in and different toxic factors placed in it. The aim of study was to assess the ventilatory efficiency in individuals employed in Generating Plant (GP) in relation to the pollution of the work place and the tobacco smoking habit during 3 years. The study covered a group of 144 male individuals employed at GP. (age 39.3 +/- 8.7 yrs, period of employment 20.3 +/- 8.6 yrs, smokers 53.5%. Lung function tests consisted of VC, FVC, FEV1, FEV1%VC, PEF, FEF50 counted from flow-volume curve and TGV, Rt measured using pletysmographic method. All tests were performed using pletysmograph (Masterlab) "Jaeger" placed on the ambulance near to the work place. The measurements were taken in May 1993 and 1996. Results were compared to with normal values (acc. ECSC). The anamnesis was obtained from all workers in the form of questionnaire projected for this study. All measurements were done during work time (9.00 a.m. to 2.00 p.m.). Mean values of the ventilation indices remained within the normal range but comparing results in smokers and non-smokers group significant differences in FEV1 and TGV were found. The symptoms of chronic bronchitis were present in 20.8% of persons. Spirometric criteria for the COPD diagnosis were found in 16 persons (11%). Only 5 persons (31%) were symptomatic. The mean decrease of FEV1 was 16.2 ml/yr, in the COPD group it was 82.5 ml/yr. The greater annual loss of FEV1 was found in the smokers and symptoms groups.