Despite the considerable progress in the classification of the idiopathic interstitial pneumonias (IIPs), the lack of an international standard has resulted in variable and confusing diagnostic criteria and terminology. The advent of high-resolution computerized tomography, the narrowed pathologic definition of usual interstitial pneumonia (UIP) and recognition of the prognostic importance of separating UIP from other IIP patterns have profoundly changed the approach to the IIPs. This is an international Consensus Statement defining the clinical manifestations, pathology, and radiologic features of patients with IIP. The major objectives of this statement are to standardize the classification of the idiopathic interstitial pneumonias (IIPs) and to establish a uniform set of definitions and criteria for the diagnosis of IIPs. The targeted specialties are pulmonologists, radiologists, and pathologists. A multidisciplinary core panel was responsible for review of background articles and writing of the document. In addition, this group reviewed the clinical, radiologic, and pathologic aspects of a wide spectrum of cases of diffuse parenchymal interstitial lung diseases to establish a uniform and consistent approach to these diseases and to clarify the terminology, definitions, and descriptions used in routine clinical practice. The final statement was drafted after a series of meetings of the entire committee. The level of evidence for the recommendations made in this statement is largely that of expert opinion developed by consensus. This classification of IIPs includes seven clinico-radiologic-pathologic entities: idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and lymphoid interstitial pneumonia. The need for dynamic interaction between pathologists, radiologists, and pulmonologists to accurately diagnose these disorders is emphasized. The level of evidence for the recommendations made in this Statement is largely that of expert opinion developed by consensus. This Statement is an integrated clinical, radiologic, and pathologic approach to the classification of the IIPs. Use of this international multidisciplinary classification will provide a standardized nomenclature and diagnostic criteria for IIP. This Statement provides a framework for the future study of these entities. Key Messages * Unclassifiable interstitial pneumonia : Some cases are unclassifiable for a variety of reasons (see text). † This group represents a heterogeneous group with poorly characterized clinical and radiologic features that needs further study. ‡ COP is the preferred term, but it is synonymous with idiopathic bronchiolitis obliterans organizing pneumonia.

SummaryThis article contains clinical guidelines and current approaches to diagnosis and treatment of idiopathic pulmonary fibrosis (IPF). The aims of devel opment this guidelines were to improve early detection and efficacy of pharmacological and non pharmacological therapy of IPF. Achieving these goals indicates improvement in medical care quality for these patients. These guidelines are intended to pulmonologists, therapeutists and other medical specialists, healthcare managers and other healthcare providers.

Background: Autopsies on COVID-19 deceased patients have many limitations due to necessary epidemiologic and preventative measures. The ongoing pandemic has caused a significant strain on healthcare systems and is being extensively studied around the world. Clinical data does not always corelate with post-mortem findings. The goal of our study was to find pathognomonic factors associated with COVID-19 mortality in 100 post-mortem full body autopsies.Materials and Methods: Following necessary safety protocol, we performed 100 autopsies on patients who were diagnosed with COVID-19 related death. The macroscopic and microscopic pathologies were evaluated along with clinical and laboratory findings.Results: Extensive coagulopathic changes are seen throughout the bodies of diseased patients. Diffuse alveolar damage is pathognomonic of COVID-19 viral pneumonia, and is the leading cause of lethal outcome in younger patients. Extrapulmonary pathology is predominantly seen in the liver and spleen. Intravascular thrombosis is often widespread and signs of septic shock are often present.Conclusion: The described pathological manifestations of COVID-19 in deceased patients are an insight into the main mechanisms of SARS-CoV-2 associated lethal outcome. The disease bears no obvious bias in severity, but seems to be more severe in some patients, hinting at genetic or epigenetic factors at play.

В настоящей статье рассматривается современная концепция диффузного альвеолярного повреждения (ДАП)достаточно часто встречающегося состояния, требующего проведения интенсивных терапевтических мероприя тий. Клиническим проявлением морфологии ДАП является респираторный дистресс синдром взрослых. Приведе ны причины возникновения ДАП-на фоне инфекционного процесса, включая септические состояния; ингаляций токсичных газов и аэрозолей; при шоке; действии радиации; лекарственной терапии и др. Подробно описан пато генез развития ДАП и патологическая анатомия, при этом характеризуются патологические изменения в легких на разных сроках от начала развития заболевания. The paper considers the current concept of diffuse alveolar lesion (DAL), a rather rare condition that requires intensive care measures. The clinicomorphological manifestation of DAL is adult respiratory distress syndrome. The causes of DAL occurring with an infectious process, including septic conditions, as well as with inhalations of toxic gases and aerosols; shock, radiation, drug therapy, etc. are presented. The pathogenesis of DAL and its pathological anatomy is described in detail, pathological lung alterations in different periods after the onset of the disease being characterized.

Aim. Compare radiological patterns of COVID-19 pneumonia with pulmonary histology in deceased patients.Materials and methods. The analysis of recent lifetime CT studies of deceased patients was performed with the identification of all existing and leading CT symptoms, including “ground glass”, “crazy paving”, consolidation, as well as the symptom complex (pattern) of organizing pneumonia. Based on the CT symptoms, we selected the target points for taking the specimens by 3-D reconstructions. At the autopsy the lungs were entirely fixed into the front and then marked on CT sections cut from 1 to 3 pieces that were placed in paraffin and processed according to the standard technique, stained with hematoxylin and eosin and fuchsin-facelina. The specimens were analyzed by identifying all available histology changes and selecting the leading one.Results. 45 targeted pieces of lung tissue were obtained from 14 deceased COVID-19 patients (7 men/ 7 women), with an average age of 77.1 ± 12.9 (49–90 years). In deceased patients with the presence of the "ground glass" symptom, in most cases (57.1%) revealed an increase in intra-alveolar cellularity, hyaline membranes, desquamation of the alveolar epithelium and infiltration of the interalveolar septum by lymphocytes, which corresponds to the exudative phase of diffuse alveolar damage (DAP). Mosaic histological changes with alternation of filled alveoli (intraalveolar edema, clusters of red blood cells, macrophages, lymphocytes) and air alveoli were detected from the areas of “crazy paving” zones. Several cases demonstrated interstitial edema and lymphoid infiltration of interalveolar partitions of different severity without their thickening. Areas of consolidation were histologically represented by extensive intraalveolar hemorrhages and / or typical zones of hemorrhagic infarcts in 45.5% of cases. Perilobular consolidation, subpleural cords, symptoms of “halo” and “reverse halo”, which we considered as part of the symptom complex of organizing pneumonia in 43% of cases, morphologically corresponded to organizing pneumonia (the proliferative phase of DAP), as well as to distelectases.Conclusion. Comparison of CT patters and post-mortem pulmonary histology in COVID-19 deceased patients demonstrated that CT symptoms and patterns correspond to certain morphological changes of different phases of DAP.

Гистиоцитоз из клеток Лангерганса (ГКЛ) -гетеро генная группа заболеваний, характеризующихся на коплением клеток Лангерганса в различных органах и тканях с формированием гранулем с эозинофиль ной инфильтрацией.ГКЛ относится к гистиоцитарным болезням. По классификации Гистиоцитарного общества 1997 г., гистиоцитарные заболевания делятся на 3 группы [1]: 1 я группа -ГКЛ; 2 я группа -нелангергансоклеточ ный гистиоцитоз, гистиоцитоз из мононуклеарных фагоцитов -болезнь Эрдгейма-Честера, болезнь Розаи-Дорфмана; 3 я группа -злокачественные гистиоцитарные заболевания. ГКЛ, в свою очередь, классифицируется по распространенности пора жения и клиническим проявлениям. Поражение одного органа (кости, мозга или легкого) обычно наблюдается у молодых взрослых. Мультисистемное поражение с острым началом (болезнь Леттерера-Сиве) встречается преимущественно у детей и имеет относительно неблагоприятный прогноз. Синдром Ханда-Шулера-Крисчена наблюдается у детей и под ростков и также имеет полиорганное поражение, но более благоприятный прогноз.Таким образом, легочный ГКЛ может развивать ся либо как самостоятельное заболевание, либо как проявление мультисистемного заболевания, при этом легочные симптомы обычно отходят на второй план. У взрослых развивается преимущественно изолированный легочный ГКЛ, однако в 15 % случа ев имеет место мультисистемное поражение [2]. ЭпидемиологияГКЛ относится к редким заболеваниям. Ежегодно выявляют 3-5 случаев ГКЛ на 1 млн детей. У взрос лых легочный ГКЛ встречается у 3-5 % пациентов с диффузными заболеваниями легких. Гистиоцитоз легких чаще выявляют в возрасте 20-40 лет, преиму щественно у курящих пациентов (> 90 %) [3]. Прек ращение курения приводит к частичной или полной ремиссии болезни [4]. Первоначально считалось, что ГКЛ с поражением легких чаще встречается у мужчин, но позднее выяснилось, что мужчины и женщины болеют с одинаковой частотой. Возмож но, это связано с изменением в эпидемиологии ку рения, произошедшем за последние 20 лет [5]. Наи более частыми внелегочными проявлениями ГКЛ являются кистозное поражение костей, поражения кожи и несахарный диабет, развивающийся в резуль тате поражения задней доли гипофиза.Этиология ГКЛ неизвестна. Вопрос о том, явля ется ли ЛКЛ реактивным процессом или злокачест венным опухолевым заболеванием, в настоящее вре мя является предметом дискуссий [6]. Патогенез ГКЛВ отличие от легочного альвеолярного протеиноза, экспериментальной модели ГКЛ легких не сущест вует.Дендритные клетки развиваются в косном мозге и мигрируют в ткани -дерму, паренхиму легкого, где они поглощают антигены, поступающие в ткани из внешней среды, путем макропиноцитоза и эндоци тоза, опосредованного рецепторами [7]. В отсутствие дополнительных стимулов дендритные клетки боль ше участвуют в реакциях иммунной толерантности, чем в инициации иммунного ответа. Провоспали тельные цитокины, патогены и фрагменты молекул, выделяющихся при повреждении ткани (например, липополисахарид, вирусная РНК или эндогенные белки некротизированных клеток) связываются с ре цепторами узнавания на поверхности ден...

Granulomatous lung disease is a heterogeneous group of diseases with different etiology, clinical symptoms and tissue damage, and different response to therapy. The prevalent histological sign of granulomatous lung disease is granuloma which is the clinical and morphological entity of this disease. The aim of this review was to describe a diversity of granulomatous diseases, key morphological features of infectious and non-infectious granulomatosis, and a diagnostic approach. Granuloma is a result of chronic inflammation involving cells of macrophage system and other inflammatory cells. Antigens activate T-lymphocytes, macrophages, epithelioid cells and polynuclear giant cells which form granuloma. Also, granuloma contains the extracellular matrix that is produced by fibroblasts and is intended to isolate the antigen. Granulomas could be infectious and non-infectious but, according to recent findings, microorganisms could cause granuloma formation in cases considered as non-infectious diseases. Therefore, sometimes it is difficult to estimate infectious or non-infectious origin of the disease.

World literature is paying increasing attention to the long-term course of COVID-19 and symptoms that appear after the acute coronavirus infection. The symptoms, functional state of the lungs, and the X-ray changes are assessed. Nevertheless, post-COVID lung histology has not been described yet.The aim of this article is to study the long-term pathological changes in the lungs after acute COVID-19. Methods. We analyzed autopsy lung material from 19 deceased who had COVID-19. The average age of the deceased was 68.82 ± 14.6 years. All patients were found to have IgG to SARS-CoV-2. The median time to death following viral interstitial pneumonia was 72 days. Results. The causes of death and thrombotic complications (infarctions of various organs and venous thrombosis) were analyzed. Histological examination revealed thrombosis of small pulmonary arteries and capillaries of interalveolar septa, microinfarctions, hemorrhages, foci of organizing pneumonia, and nonspecific interstitial pneumonia.Conclusion. The first assessment of histological changes in human lungs showed that the most common post-COVID pathologic changes are microcirculation disorders combined with small areas of acute lung damage. The obtained data are essential for understanding the pathogenesis of post-COVID syndrome, necessitate diagnostic of microvasculature disorders using laboratory tests, scintigraphy, and CT imaging, as well as search for the therapeutic strategies.