AIM: Transbronchial cryobiopsies are increasingly used for the diagnosis of interstitial lung disease (ILD), but there is a lack of published information on the features of specific ILD in cryobiopsies. Here we attempt to provide pathological guidelines for separating usual interstitial pneumonia (UIP) of idiopathic pulmonary fibrosis (IPF), fibrotic hypersensitivity pneumonitis (FHP) and connective tissue disease-associated ILD (CTD-ILD) in cryobiopsies. METHODS: We examined 120 cryobiopsies from patients with multidisciplinary discussion (MDD)-established CTD-ILD and compared them to a prior series of 121 biopsies from patients with MDD-established IPF or FHP. RESULTS: A non-specific interstitial pneumonia (NSIP) pattern alone was seen in 36 of 120 (30%) CTD-ILD, three of 83 (3.6%) FHP and two of 38 (5.2%) IPF cases, statistically favouring a diagnosis of CTD-ILD. The combination of NSIP + OP was present in 29 of 120 (24%) CTD-ILD, two of 83 (2.4%) FHP and none of 38 (0%) IPF cases, favouring a diagnosis of CTD-ILD. A UIP pattern, defined as fibroblast foci plus any of patchy old fibrosis/fibrosis with architectural distortion/honeycombing, was identified in 28 of 120 (23%) CTD-ILD, 45 of 83 (54%) FHP and 27 of 38 (71%) IPF cases and supported a diagnosis of FHP or IPF. The number of lymphoid aggregates/mm2 and fibroblast foci/mm2 was not different in IPF, CTD-ILD or FHP cases with a UIP pattern. Interstitial giant cells supported a diagnosis of FHP or CTD-ILD over IPF, but were infrequent. CONCLUSIONS: In the correct clinical/radiological context the pathological findings of NSIP, and particularly NSIP plus OP, favour a diagnosis of CTD-ILD in a cryobiopsy, but CTD-ILD with a UIP pattern, FHP with a UIP pattern and IPF generally cannot be distinguished.

• Klíčová slova
• connective tissue disease‐associated interstitial lung disease, cryobiopsy, hypersensitivity pneumonitis, usual interstitial pneumonia,
• MeSH
• biopsie metody   MeSH
• dospělí MeSH
• idiopatická plicní fibróza patologie   komplikace   diagnóza   MeSH
• intersticiální plicní nemoci * patologie   diagnóza   komplikace   MeSH
• lidé středního věku MeSH
• lidé MeSH
• nemoci pojiva * komplikace   patologie   diagnóza   MeSH
• plíce patologie   MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

Anti-melanoma differentiation-associated gene-5 (MDA-5) antibody is an autoantibody found in patients with dermatomyositis. These antibody-positive patients are clinically characterized by complications of rapidly progressive interstitial pneumonia resistant to treatment and with poor prognosis. We describe herein a patient with MDA-5 antibody-positive interstitial lung disease, which progressed rapidly to death after a period of slow progress. Recently, attention has been paid to the similarities in clinical courses and CT images between MDA-5 antibody-positive interstitial lung disease and coronavirus disease 2019 (COVID-19)-associated pneumonia. Patients with MDA-5 antibody do not always have diffuse and evenly distributed bilateral opacities at the time of first presentation. This patient had significant laterality of such opacities. It should be considered that MDA-5 antibody-positive patients with such laterality in opacities might progress rapidly. Chest physicians, dermatologists, and dermatologists need to be aware of the characteristics of the disease for optimal treatment choices.

• Klíčová slova
• anti-melanoma differentiation-associated gene-5 antibody: smoking, interstitial lung disease, prognosis,
• MeSH
• autoprotilátky MeSH
• COVID-19 * komplikace   MeSH
• dermatomyozitida * komplikace   MeSH
• intersticiální plicní nemoci * komplikace   MeSH
• lidé MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• autoprotilátky MeSH

The use of pulmonary function tests (PFTs) has been widely described in airway diseases like asthma and cystic fibrosis, but for children's interstitial lung disease (chILD), which encompasses a broad spectrum of pathologies, the usefulness of PFTs is still undetermined, despite widespread use in adult interstitial lung disease. A literature review was initiated by the COST/Enter chILD working group aiming to describe published studies, to identify gaps in knowledge and to propose future research goals in regard to spirometry, whole-body plethysmography, infant and pre-school PFTs, measurement of diffusing capacity, multiple breath washout and cardiopulmonary exercise tests in chILD. The search revealed a limited number of papers published in the past three decades, of which the majority were descriptive and did not report pulmonary function as the main outcome.PFTs may be useful in different stages of management of children with suspected or confirmed chILD, but the chILD spectrum is diverse and includes a heterogeneous patient group in all ages. Research studies in well-defined patient cohorts are needed to establish which PFT and outcomes are most relevant for diagnosis, evaluation of disease severity and course, and monitoring individual conditions both for improvement in clinical care and as end-points in future randomised controlled trials.

• MeSH
• dítě MeSH
• intersticiální plicní nemoci diagnóza   patofyziologie   MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• předškolní dítě MeSH
• respirační funkční testy * MeSH
• věkové faktory MeSH
• Check Tag
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH

Successful rituximab treatment of granulomatous/lymphocytic interstitial lung disease in common variable immunodeficiency Common variable immunodeficiency, a heterogeneous group of diseases, represents a clinically relevant form of antibody immunodeficiency. Granulomatous/lymphocytic interstitial lung disease is among the most serious complications. A case report is presented of a young women with granulomatous/lymphocytic interstitial lung disease and splenomegaly accompanied by pancytopenia. Intravenous rituximab treatment in monotherapy (at a weekly dose of 375 mg/m2 for four consecutive weeks, repeated six months later) not only led to a significant improvement in clinical symptoms but also to positive morphological and functional lung changes, mitigation of pancytopenia, considerable reduction of alkaline phosphatase level, and disappearance of splenic granulomas. The treatment was well tolerated without any side effects. The case report presented suggests possible efficacy and safety of rituximab monotherapy in patients with a complicated form of common variable immunodeficiency. KEYWORDS Rituximab - antibody immunodeficiency - lung disease - treatment Epidemiol. Mikrobiol. Imunol., 67, 2018, č. 3, s. 142-148.

• Klíčová slova
• 2018, 67, Rituximab - antibody immunodeficiency - lung disease - treatment Epidemiol. Mikrobiol. Imunol., s. 142-148, č. 3,
• MeSH
• běžná variabilní imunodeficience * komplikace   MeSH
• imunologické faktory terapeutické užití   MeSH
• intersticiální plicní nemoci * farmakoterapie   etiologie   MeSH
• lidé MeSH
• rituximab * terapeutické užití   MeSH
• výsledek terapie MeSH
• Check Tag
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• imunologické faktory MeSH
• rituximab * MeSH

BACKGROUND: Interstitial lung disease is rarer in children than adults, but, with increasing diagnostic awareness, more cases are being discovered. The prognosis of childhood interstitial lung disease is often poor, but increasing numbers are now surviving into adulthood. AIM: To characterise childhood interstitial lung disease survivors and identify their impact on adult interstitial lung disease centres. METHODS: This was a European study (34 adult and childhood interstitial lung disease centres) reporting incident/prevalent cases of childhood interstitial lung disease survivors from January to July 2023. Epidemiological, clinical, physiological and genetic data were collected. RESULTS: 244 patients were identified with a median (interquartile range) age at diagnosis of 12.5 years (6-16 years) and age at study inclusion of 25 years (22-33 years), with 51% male, 86% nonsmokers and a median (interquartile range) % predicted forced vital capacity of 70% (47-89%) and diffusing capacity of the lungs for carbon monoxide of 48% (32-75%). 32% were prescribed long-term oxygen and 227 (93%) were followed up in adult centres whereas 17 (7%) never transitioned. The commonest diagnoses (82%) were childhood interstitial lung disease category B1 (sarcoidosis, hemosiderosis, connective tissue disorders, vasculitis) at 35%, A4 (surfactant-related) at 21%, B2 (bronchiolitis obliterans, hypersensitivity pneumonitis) at 14% and Bz (unclassified interstitial lung disease) at 13%. Bz patients had the worst functional status. 60% of all patients were still being prescribed corticosteroids. Re-specification of diagnosis and treatment were made after transition for 9.8% and 16% of patients, respectively. Not all childhood interstitial lung disease diagnoses were recognised in adult interstitial lung disease classifications. CONCLUSION: Childhood interstitial lung disease survivors are seen in most adult interstitial lung disease centres and only a minority continue follow-up in paediatric centres. Survivors have a significant loss of lung function. The heterogeneity of their aetiologies and therapeutic requirements has a real impact on adult interstitial lung disease centres. Re-specification of diagnosis and treatment may contribute to precision and personalisation of management.

• MeSH
• dítě MeSH
• dospělí MeSH
• intersticiální plicní nemoci * diagnóza   patofyziologie   epidemiologie   MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• přežívající * statistika a číselné údaje   MeSH
• prognóza MeSH
• vitální kapacita MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• Geografické názvy
• Evropa epidemiologie   MeSH

OBJECTIVES: Physical activity is reduced in patients with interstitial lung disease (ILD) and physical inactivity is related to poor health outcomes. We investigated the effect of a telecoaching intervention to improve physical activity in patients with ILD. METHODS: Eighty patients with ILD were randomized into the intervention or control group. Patients in the intervention group received a 12-week telecoaching program including a step counter, a patient-tailored smartphone application, and coaching calls. Patients in the control group received usual care. Physical activity (primary outcome), physical fitness and quality of life were measured at baseline and 12 weeks later with an accelerometer, 6-min walking test and quadriceps muscle force and the King's Brief Interstitial Lung Disease questionnaire (K-BILD). RESULTS: Participation in telecoaching did not improve physical activity: between-group differences for step count: 386 ± 590 steps/day, p = .52; sedentary time: 4 ± 18 min/day, p = .81; movement intensity: 0.04 ± 0.05 m/s2, p = .45). Between-group differences for the 6-min walking test, quadriceps muscle force and K-BILD were 14 ± 10 m, p = .16; 2 ± 3% predicted, p = .61; 0.8 ± 1.7 points, p = .62 respectively. CONCLUSIONS: Twelve weeks of telecoaching did not improve physical activity, physical fitness or quality of life in patients with ILD. Future physical or behavioural interventions are needed for these patients to improve physical activity.

• Klíčová slova
• Physical activity, interstitial lung disease, telecoaching,
• MeSH
• cvičení MeSH
• intersticiální plicní nemoci * terapie   MeSH
• kvalita života MeSH
• lidé MeSH
• mentoring * MeSH
• průzkumy a dotazníky MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• randomizované kontrolované studie MeSH

Interstitial lung diseases very often do not only affect the lung tissue, but are part of multisystem diseases. Awareness of their classification and differential diagnosis therefore does not belong only to respiratory departments, but shall be acknowledged by all specialisations. It is obvious that the approach to a patient with life-threatening disease or acute onset of symptoms differently then patients with slow disease onset or "accidentally" detected lung abnormalities. The presented manuscript brings a differential diagnostic approach to facilitate orientation in the field of interstitial ling diseases.

• Klíčová slova
• Interstitial lung diseases, classification, differential diagnosis, examination methods, interstitial lung disease,
• MeSH
• diferenciální diagnóza MeSH
• intersticiální plicní nemoci * diagnóza   MeSH
• lidé MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

We evaluated patterns of normal and abnormal lung parenchyma on standard 8-mm computed tomography (CT) scans, and 1-mm or 2-mm high-resolution CT (HRCT) scans in 22 subjects. There were five control subjects with no lung symptoms, and 17 patients with proved diagnosis of diffuse interstitial diseases consisting of sarcoidosis (n = 7), bronchioloalveolar carcinoma (n = 3), extrinsic allergic alveolitis (n = 2), asbestosis (n = 2), scleroderma (n = 2), and drug toxicity (n = 1). CT and HRCT scans were evaluated for specific parenchymal features particularly the distribution in secondary pulmonary lobule; conditions of pleura, mediastinum, and thoracic wall were appreciated. CT and HRCT findings were described in individual disease. We believe that CT and HRCT are useful investigations in patients with suspected or known diffuse interstitial lung diseases. At present time CT, and particularly HRCT seem to be the best available method to image the lung parenchyma.

• MeSH
• dospělí MeSH
• intersticiální plicní nemoci diagnostické zobrazování   MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádory plic diagnostické zobrazování   MeSH
• plíce anatomie a histologie   diagnostické zobrazování   MeSH
• počítačová rentgenová tomografie * MeSH
• referenční hodnoty MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• srovnávací studie MeSH

Malignant mesothelioma typically encases lungs as a thick rind, while relatively sparing lung parenchyma. We describe an unusual presentation of mesothelioma characterized by diffuse intrapulmonary growth, with absent or inconspicuous pleural involvement, clinically simulating interstitial lung disease (ILD). We identified 5 patients (median age 56 y, all men) with diffuse intrapulmonary malignant mesothelioma in our pathology consultation practice from 2009 to 2012. Clinical history, imaging, and pathology materials were reviewed. Symptoms included chronic dyspnea (4 cases), cough (3), and acute dyspnea with bilateral pneumothorax (1). Chest imaging showed irregular opacities (5), reticulation (4), pleural effusions (2), and subpleural nodular densities (1), without radiologic evidence of pleural disease or masses. A clinicoradiologic diagnosis of ILD was made in all cases, and wedge biopsies were performed. Histologic evaluation revealed a neoplastic proliferation of bland epithelioid or spindled cells, showing various growth patterns simulating silicotic nodules, desquamative interstitial pneumonia, organizing pneumonia, and Langerhans cell histiocytosis. Some areas mimicked adenocarcinoma, with lepidic, acinar, micropapillary, and solid patterns. Initial diagnoses by referring pathologists included reactive changes (1), hypersensitivity pneumonitis versus drug reaction (1), desquamative interstitial pneumonia versus neoplasm (1), and mesothelioma (2). Microscopic pleural involvement was identified in 4 cases. Immunohistochemistry confirmed the characteristic immunophenotype of mesothelioma in all cases. Median survival of 3 patients treated with chemotherapy was 28 months. Two patients received no therapy and survived 3 and 4 weeks, respectively. "Diffuse intrapulmonary malignant mesothelioma" is a rare variant with a distinctive presentation that clinically mimics ILD. Recognition is essential to avoid misdiagnosis.

• MeSH
• diferenciální diagnóza MeSH
• imunohistochemie MeSH
• intersticiální plicní nemoci diagnóza   MeSH
• lidé středního věku MeSH
• lidé MeSH
• mezoteliom diagnóza   MeSH
• nádory plic diagnóza   MeSH
• senioři MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

Diffuse interstitial lung disorders include more than 200 different syndroms affecting the space between epithelial basal membrane and endothelial cells. Histopathological investigation of the lung tissue is one of the crucial parts of the multidisciplinary team approach for the investigation of these disorders. The aim of this review is a brief characterization of the pattern of the main subtypes of lung tissue damage.

• Klíčová slova
• interstitial - pulmonary - fibrosis - histopathology.,
• MeSH
• intersticiální plicní nemoci patologie   MeSH
• lidé MeSH
• plíce patologie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH