Angiogenesis is an important process involved in the pathogenesis of diffuse parenchymal lung diseases. The aim of the study was to compare the angiogenic profile of patients with sarcoidosis and idiopathic pulmonary fibrosis (IPF) based on analysis of circulating factors. Serum concentrations of angiopoietin-2 (Ang-2), follistatin, granulocyte-macrophage-colony stimulating factor (GM-CSF), interleukin-8 (IL-8), platelet derived growth factor-BB (PDGF-BB), platelet endothelial cellular adhesion molecule-1 (PECAM-1) and vascular endothelial growth factors (VEGF) were measured in the patients and the healthy subjects. Serum concentrations of G-CSF, follistatin, PECAM-1 and IL-8 were significantly higher in the IPF patients in comparison with the control group and the sarcoid patients. PDGF-BB concentrations were also significantly higher in serum of IPF patients than in sarcoid patients, but not than in the controls. In contrast, Ang-2 and VEGF concentrations did not differ significantly between the three groups. In the sarcoid patients, irrespective of the disease activity or the radiological stage, serum concentrations of these cytokines were similar to the control group. These results indicate that differences may exist in angiogenic activity between patients with parenchymal lung diseases. In contrast to sarcoidosis, IPF is characterized by a higher serum concentration of different molecules involved in the angiogenic processes .

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.

INTRODUCTION: This report introduces two cases presenting absorption of considerable radiological changes in the course of the coronavirus pneumonia in patients treated with prolonged oral steroids.CASES: The first case concerns a male receiving steroids only during hospitalisation in the Infectious Disease Hospital. After discharge, the patient experienced increasing dyspnoea resulting in hospitalisation in our Department of Lung Diseases. HRCT revealed progression of a bilateral, middle, and basal ground-glass opacity when compared to the examination performed at the early stage of the disease. The supplementary oxygen therapy and steroids were administered, followed by extended prednisone consumption up to 2 months after discharge. Follow-up HRCT revealed an almost complete absorption of the ground-glass opacity. The second case concerns a male treated with steroids only during hospitalisation in the Infectious Disease Hospital. Chest CT revealed widespread bilateral ground-glass opacities with consolidations. After discharge with no treatment, he suffered from severe dyspnoea and exercise intolerance, resulting in hospitalisation on the 7^th day of home stay. Since then, a continued steroid treatment was administered resulting in a clinical, spirometric, and radiological improvement.CONCLUSIONS: Based on these observations, patients after the COVID-pneumonia may derive benefits from a prolonged steroid treatment. Therefore, this class of medications should be considered in SARS-CoV-2 patients, especially in patients with persistent radiological changes and dyspnoea requiring the supplementary oxygen therapy. However, randomised controlled trials are required to establish guidelines for the steroid treatment in this group of patients.

Introduction: Fatigue is one of many symptoms reported by patients with sarcoidosis. It is believed that fatigue may be the cause of exercise intolerance and reduced quality of life in patients with sarcoidosis. The purpose of the work was to present the frequency of fatigue prevalence in patients with sarcoidosis and to investigate the correlation between fatigue and the results of pulmonary function tests and walking distance. Materials and Methods: A total of 74 patients with sarcoidosis in a stable phase of the disease, not treated in the past with glucocorticoids or immunosuppressive drugs, and without indications for treatment at the time of the study were examined. In all patients fatigue evaluation was carried out with the use of the Fatigue Assessment Scale questionnaire (FAS); dyspnoea was assessed with the use of the Medical Research Council scale (MRC). Body Mass Index (BMI), spirometry, and a 6-minute walk test were additionally performed. The control group included 30 healthy volunteers who completed the FAS. Results: In the examined group of patients fatigue was diagnosed in 36 patients (50%), and in 5 (6.94%)-strong fatigue was observed. The remaining 31 (43.06%) patients felt no fatigue. The average value of points obtained by FAS questionnaire in sarcoidosis patients was significantly higher than the respective value in the control group (p = 0.02). A significantly higher number of points by FAS questionnaire was observed in female patients with sarcoidosis (p = 0.04) in comparison to men. No significant statistical correlation between fatigue index FAS and BMI (r = 0.22, p = 0.11), FEV₁ (r = –0.11, p = 0.3), FEV₁% pred. (r = 0.01, p = 0.9), FVC (r = −0.03, p = 0.77), FEF₂₅₋₇₅ (r = −0.23, p = 0.1) and the distance in the six-minute walk test (6MWT) (r = −0.01, p = 0.9) was observed. However, there was a weak negative correlation between the age of the patients and the FAS index (r = −0.29, p = 0.01). Conclusions: Fatigue in patients with sarcoidosis does not correlate with the results of lung function tests or with walking distance in 6MWT.

Nintedanib is a disease-modifying agent licensed for the treatment of IPF. Data on Polish experience with nintedanib in IPF are lacking. The present study aimed to describe the safety and efficacy profiles of nintedanib in a large real-world cohort of Polish patients with IPF. This was a multicenter, retrospective, observational study of IPF patients treated with nintedanib between March 2018 and October 2021. Data collection included baseline clinical characteristics, results of pulmonary function tests (PFTs), and a six-minute walk test (6MWT). Longitudinal data on PFTs, 6MWT, adverse drug reactions (ADRs), and treatment persistence were also retrieved. A total of 501 patients (70% male) with a median age of 70.9 years (IQR 65–75.7) were included in this study. Patients were followed on treatment for a median of 15 months (7–25.5). The majority of patients (66.7%) were treated with the full recommended dose of nintedanib and 33.3% of patients were treated with a reduced dose of a drug. Intermittent dose reductions or drug interruptions were needed in 20% of patients. Over up to 3 years of follow-up, pulmonary function remained largely stable with the minority experiencing disease progression. The most frequent ADRs included diarrhea (45.3%), decreased appetite (29.9%), abdominal discomfort (29.5%), weight loss (32.1%), nausea (20.8%), fatigue (19.2%), increased liver aminotransferases (15.4%), and vomiting (8.2%). A total of 203 patients (40.5%) discontinued nintedanib treatment due to diverse reasons including ADRs (10.2%), death (11.6%), disease progression (4.6%), patient’s request (6.6%), and neoplastic disease (2.2%). This real-world study of a large cohort of Polish patients with IPF demonstrates that nintedanib therapy is safe, and is associated with acceptable tolerance and disease stabilization. These data support the findings of previously conducted clinical trials and observational studies on the safety and efficacy profiles of nintedanib in IPF.

Background and Aims: Antifibrotic therapies reduce lung function decline in patients with idiopathic pulmonary fibrosis (IPF). This single‐arm, open‐label, nonrandomized study aimed to determine the influence of antifibrotic treatment on patients' reported symptoms and expectations of the therapy.Methods: Fifty‐two patients with confirmed IPF at a mean age of 65 ± 8.63 years (73% male) completed the following surveys at baseline and after 12 months of Pirfenidone treatment: Short Form Healthy Survey (SF‐36), St. George's Respiratory Questionnaire (SGRQ), Baseline Dyspnea Index (BDI), Fatigue Assessment Scale (FAS), Leicester Cough Questionnaire (LCQ), and Patient's Needs and Expectations Authors' Survey.Results: The most important patients' needs were access to novel therapy, fast and easy access to health centers specializing in IPF treatment, and the improvement of the general condition or the maintenance of its level. These needs did not change with time, except for the significantly more important right of deciding on disease management after 12 months of treatment (p = 0.014). The quality of life per SF‐36, after 1 year of Pirfenidone treatment, significantly improved in the physical cumulative score (p = 0.004) and mental cumulative score (p = 0.003). Significant deteriorations were observed in bodily pain and vitality. For the remaining questionnaires (SGRQ, BDI, FAS, and LCQ), no significant changes in the course of the study were noticed. Around one in 10 patients subjected to Pirfenidone therapy had achieved general symptom improvement in all areas; that is, quality of life improvement as well as cough and dyspnea reduction.Conclusions: One year of antifibrotic treatment resulted in a general improvement in the quality of life per the SF‐36 questionnaire. Patients' expectations of disease management did not change; also, access to novel therapies and easy access to health centers specializing in IPF management remained their top needs.