Практические возможности ПЭТ / КТ в оценке активности и распространенности саркоидоза

Real-life PET / CT abilities to assess activity and extension of sarcoidosis.Практические возможности ПЭТ / КТ в оценке активности и распространенности саркоидоза

Макрофагальный и цитокиновый спектры бронхоальвеолярного смыва при впервые выявленном и рецидивирующем саркоидозе органов дыхания

Goal of the study: to investigate structural and functional specific features of macrophages and cytokine profiles of bronchoalveolar lavage in new and relapsing respiratory sarcoidosis (RS).Materials and methods. The study included comparative light optical and electronic microscopic examinations of macrophages, macrophage formula and cytokine profile of bronchoalveolar lavage in 120 patients suffering from different forms of RS. It was found that in case of new RS mostly macrophages of M1 phenotype were detected in the lavage. They had high level of IL-8, IL-2, IL-1β production and ultrastructural signs of hypersecretion which was especially typical of platycytes. High level of IL-4, IL-5 content is typical of relapsing sarcoidosis: additionally to macrophages of M1 phenotype, there are macrophages of M2 phenotype with ultrastructural signs of phagocytic function which can be used for diagnostics of this form of RS.Цель исследования: изучить структурно-функциональные особенности макрофагов и цитокиновый спектр бронхоальвеолярного лаважа при впервые выявленном и рецидивирующем саркоидозе органов дыхания (СОД).Материалы и методы. Проведено сравнительное светооптическое и электронно-микроскопическое изучение макрофагов, определены макрофагальная формула и цитокиновый спектр бронхоальвеолярного смыва у 120 больных с различными вариантами СОД. Показано, что при впервые выявленном СОД в лаваже определяются преимущественно макрофаги М1-фенотипа. Они отличаются высоким уровнем продукции IL-8, IL-2, IL-1β и ультраструктурными признаками гиперсекреции, что особенно характерно для эпителиоидных клеток. Для рецидивирующего саркоидоза характерно повышенное содержание IL-4, IL-5: помимо макрофагов с М1-фенотипом, появляются макрофаги М2-фенотипа с ультраструктурными признаками фагоцитарной функции, что может быть использовано в диагностике этой формы СОД

Клинические и морфологические особенности лимфаденопатии средостения при гранулематозных заболеваниях легких

The purpose of this study was to determine detection rate and clinical and morphological characteristics of mediastinal lymphadenopathy (ML) in patients with respiratory system sarcoidosis (RSS), disseminated pulmonary tuberculosis (DPT) and exogenic allergic alveolitis (EAA).Materials and Methods. Patients (n = 278) with established diagnosis RSS, DPT, EAA were investigated. The cumulative index parameters were determined, hematology test, assessments of pulmonary function, diffuse lung capacity, diffusion coefficient were performed in all patients. Computed tomography of chest organs and morphologic examination of mediastinal lymph node (LN) biopsy were performed.Results. Subjects with newly diagnosed (n = 72) and recurrent (n = 104) disease were observed among patients with RSS (n = 176). ML was observed in 95% of cases, involved bronchopulmonary, bifurcation, paratracheal and paraaortal groups; dimensions of intrathoracic LN (ITLN) were 20.0 ± 1.9 mm. Epitheliocellular granulomas (EG) without necrosis, not fusing with each other, were established morphologically; they were determined in recurrent disease course in association with background fibrosis. In patients with DPT (n = 41), subacute (n = 28) and chronic (n = 13) disease course was noted. With subacute course of DPT in 28.6% of cases, ML of paratracheal and bifurcation groups (dimensions of ITLN – 13.1 ± 0.3 mm) was observed, with chronic course – the enlargement of paratracheal and bronchopulmonary LN (7.7% of cases). EG with necrosis and tendency to fusion was verified histologically; inflammatory process involved LN capsule and could be spread to fatty tissue. In patients with EAA (n = 76), acute (n = 10), subacute (n = 38) and chronic (n = 28) disease courses were observed. With acute EAA course, hyperplasia of ITLN of bifurcation and tracheo-bronchial groups up to 13.5 ± 0.6 mm was observed in 20% of cases. Follicular hyperplasia with widening and edema of hermintative centers was observed at morphological investigation. With subacute EAA course, increased ITLN in bifurcation and broncho-pulmonary groups up to 13.6 ± 0.6 mm were observed in 46% of cases. Formation of histiocytic-macrophagal granulomas without epithelioid cells and deposit precipitation in the plasma cell aggregation areas was found at morphological evaluation. With chronic EAA, ML in broncho-pulmonary and paratracheal groups was detected in 17.8% of cases (enlargement of LN up to 11 ± 0.9 mm). The formation of diffuse and focal fibrosis and hyalinosis was noted morphologically.Conclusion. ML is observed in all granulomatous pulmonary diseases studied, however its rate and severity, composition of involved ITLN groups and character of morphological changes is different which is possible to use in diagnostics.Целью данного исследования явилось определение частоты выявления, клинических и морфологических особенностей лимфаденопатии средостения (ЛС) у больных саркоидозом органов дыхания (СОД), диссеминированным туберкулезом легких (ДТЛ) и экзогенным аллергическим альвеолитом (ЭАА).Материалы и методы. Обследованы пациенты (n = 278) с установленными диагнозами СОД, ДТЛ и ЭАА. У всех больных определялись показатели кумулятивного индекса, проводились исследование клинического анализа крови, функции внешнего дыхания, диффузионной способности легких, коэффициента диффузии. Выполнялись компьютерная томография органов грудной клетки и морфологическое исследование биопсии лимфатических узлов (ЛУ) средостения.Результаты. Среди больных СОД (n = 176) наблюдались лица с впервые выявленным (n = 72) и рецидивирующим (n = 104) заболеванием. ЛС наблюдалась в 95 % случаев, затрагивала бронхопульмональные, бифуркационные, паратрахеальные и парааортальные группы; размеры внутригрудных ЛУ (ВГЛУ) составляли 20,0 ± 1,9 мм. Морфологически установлены эпителиоидноклеточные гранулемы (ЭГ) без некроза, не сливающиеся между собой, при рецидивирующем течении определялись на фоне фиброза. У пациентов с ДТЛ (n = 41) отмечалось подострое (n = 28) и хроническое (n = 13) течение заболевания. При подостром течении ДТЛ в 28,6 % случаев наблюдалась ЛС паратрахеальной и бифуркационной групп (размеры ВГЛУ – 13,1 ± 0,3 мм), при хроническом – увеличение паратрахеальных и бронхопульмональных ЛУ (7,7 % случаев). Гистологически верифицирована ЭГ с некрозом и тенденцией к слиянию; воспалительный процесс затрагивал капсулу ЛУ и мог переходить на жировую клетчатку. У пациентов с ЭАА (n = 76) отмечено острое (n = 10), подострое (n = 38) и хроническое (n = 28) течение заболевания. При остром течении ЭАА в 20 % случаев наблюдалась гиперплазия до 13,5 ± 0,6 мм ВГЛУ бифуркационной и трахеобронхиальной групп. Морфологически выявлялась фолликулярная гиперплазия с расширением и отеком герминтативных центров. При подостром течении ЭАА в 46 % случаев отмечалось увеличение до 13,6 ± 0,6 мм ВГЛУ бифуркационной и бронхопульмональной групп. Морфологически выявлялось формирование гистиоцитарно-макрофагальных гранулем без эпителиоидных клеток и отложения депозитов в зонах скопления плазматических клеток. При хроническом ЭАА ЛС бронхопульмональной и паратрахеальной групп выявлена в 17,8 % случаев (увеличение ЛУ до 11 ± 0,9 мм). Морфологически отмечено формирование диффузного и очагового фиброза и гиалиноза.Заключение. ЛС наблюдается при всех исследованных гранулематозных заболеваниях легких, однако частота и степень ее выраженности, состав вовлекаемых групп ВГЛУ и характер морфологических изменений различен, что возможно использовать в диагностике

Diagnostic accuracy of a clinical diagnosis of idiopathic pulmonary fibrosis: An international case-cohort study

We conducted an international study of idiopathic pulmonary fibrosis (IPF) diagnosis among a large group of physicians and compared their diagnostic performance to a panel of IPF experts. A total of 1141 respiratory physicians and 34 IPF experts participated. Participants evaluated 60 cases of interstitial lung disease (ILD) without interdisciplinary consultation. Diagnostic agreement was measured using the weighted kappa coefficient (\u3baw). Prognostic discrimination between IPF and other ILDs was used to validate diagnostic accuracy for first-choice diagnoses of IPF and were compared using the Cindex. A total of 404 physicians completed the study. Agreement for IPF diagnosis was higher among expert physicians (\u3baw=0.65, IQR 0.53-0.72, p20 years of experience (C-index=0.72, IQR 0.0-0.73, p=0.229) and non-university hospital physicians with more than 20 years of experience, attending weekly MDT meetings (C-index=0.72, IQR 0.70-0.72, p=0.052), did not differ significantly (p=0.229 and p=0.052 respectively) from the expert panel (C-index=0.74 IQR 0.72-0.75). Experienced respiratory physicians at university-based institutions diagnose IPF with similar prognostic accuracy to IPF experts. Regular MDT meeting attendance improves the prognostic accuracy of experienced non-university practitioners to levels achieved by IPF experts

Саркоидоз: федеральные клинические рекомендации по диагностике и лечению

Sarcoidosis is a multi-organ granulomatosis  of unknown  origin. Modern  diagnostic methods  allow detecting  this disease at an early stage. The absence of specific markers requires a comprehensive  approach  to diagnosis based on comparison  of radiation,  clinical, morphological  and functional data. The course of sarcoidosis without damage to the respiratory system presents significant difficulties. It is extremely important  to understand the time and means of starting Sarcoidosis’s treatment to avoid the early initiation  of hormones  and cytostatics and, on the other hand,  to timely respond to progression and threatening  conditions.  Methods. Clinical recommendations are based on the analysis of Russian and English publications of the latest sarcoidosis research. The target audience of these clinical guidelines are therapists, general practitioners, pulmonologists, TB doctors, rheumatologists,  dermatologists,  radiation diagnosticians,  immunologists,  and clinical pharmacologists.  Each thesis-recommendation for diagnosis and treatment is evaluated on an 1 to 5 scale of levels of evidence and an A, B, C scale of the grades of recommendations. The clinical guidelines also contain comments and explanations for the theses-recommendations, diagnostic algorithms, treatment strategies, reference materials on the use of recommended drugs. Conclusion. Current information  on epidemiology, clinical manifestations,  diagnosis and management  strategies for patients with sarcoidosis are covered in the presented clinical guidelines. Approved by the decision of the Scientific and Practical Council of the Ministry of Health of the Russian Federation (2022).Саркоидоз является полиорганным гранулематозом неизвестной природы. При помощи  современных методов диагностики это заболевание возможно выявить на ранних стадиях. Для установления диагноза на основании сопоставления лучевых, клинических, морфологических и функциональных данных при отсутствии специфических маркеров требуется комплексный поход. Варианты течения саркоидоза без поражения органов дыхания представляют  значительные трудности. Во избежание  раннего  назначения гормонов  и цитостатических препаратов и, наоборот, в целях своевременного реагирования на прогрессирование и угрожающие состояния крайне актуально понимание времени  и средств  начала  лечения  саркоидоза.  Методы. Клинические рекомендации созданы  на основании анализа данных последних исследований саркоидоза, опубликованных на русском и английском языках. Каждый  тезис-рекомендация по проведению диагностических и лечебных мероприятий оценивается по шкалам оценки  уровня достоверности доказательств (1–5)  и убедительности  рекомендаций (по категориям  А, В, С). Клинические рекомендации содержат также комментарии и разъяснения к указанным тезисам-рекомендациям, алгоритмы  по диагностике, тактике терапии,  справочные материалы по использованию рекомендуемых препаратов. Целевой  аудиторией  данных  клинических рекомендаций являются  терапевты,  врачи  общей  практики,  пульмонологи, фтизиатры,  ревматологи,  дерматологи,   специалисты  лучевой  диагностики,  иммунологи, клинические  фармакологи.  Заключение. По данным представленных клинических рекомендаций освещаются современные сведения об эпидемиологии, клинических проявлениях,  диагностике и тактике  ведения  пациентов с саркоидозом. Одобрены  решением Научно-практического совета  Министерства здравоохранения Российской Федерации (2022)

Difficulties in the differential diagnosis of disseminated processes in the lungs

Disseminated lung diseases are still a difficult diagnostic problem. Modern endoscopic techniques for minimally invasive diagnostics allow to verify the diagnosis in more than 3/4 of patients. However, the incidence of diagnostic errors at initial evaluation without biopsy remains high. The reasons for errors are the actual complexity of differential diagnosis of disseminated processes, failure to properly examine the patients, late or insufficient use of high-resolution computed tomography and bronchoscopy with releveant biopsies

VALVULAR BRONCHIAL BLOCKING IN TREATMENT OF RELAPSING SPONTANEOUS PHEUMOTHORAX IN THE PATIENT WITH PARASEPTAL PULMONARY EMPHYSEMA

Spontaneous pneumothorax was treated by placing two endobronchial valves during rigid bronchoscopy under anesthesia in a patient with severe end-stage chronic obstructive pulmonary disease (an emphysematous phenotype) and overall paraseptal emphysema in the presence of grade III respiratory failure, with complicated spontaneous pneumothorax recurring manifold even after surgical treatment for spontaneous pneumothorax. This gave rise to the expansion of the lung that had collapsed within 18 months, to healing of bronchopleural fistula, and to return to normal life.The duration of a follow-up was 1 year with the valves being present in the bronchi and another year after their removal; there was no recurrence of spontaneous pneumothorax. Examination of respiratory function established significant positive changes (forced expiratory volume, vital capacity, and forced vital capacity after one year, then after removal of endobronchial valves). There were more significant positive changes in blood gas composition: normalization of blood oxygen saturation was achieved

The Possibilities of Bronchoscopy and Endosonography in the Differential Diagnosis of Mediastinal Tuberculous and Metastatic Lesions

This clinical case shows the experience in concurrently using video-assisted bronchoscopy and endosonography in a patient with mediastinal adenopathy for differential diagnosis between tuberculous and metastatic changes. It demonstrates the possibilities and limitations of using both classical bronchoscopy and transesophageal endosonography with a EUS-b-FNA using echobronchoscope

MACROPHAGE AND CYTOKINE PROFILES OF BRONCHOALVEOLAR LAVAGE IN NEW AND RELAPSING RESPIRATORY SARCOIDOSIS

Goal of the study: to investigate structural and functional specific features of macrophages and cytokine profiles of bronchoalveolar lavage in new and relapsing respiratory sarcoidosis (RS).Materials and methods. The study included comparative light optical and electronic microscopic examinations of macrophages, macrophage formula and cytokine profile of bronchoalveolar lavage in 120 patients suffering from different forms of RS. It was found that in case of new RS mostly macrophages of M1 phenotype were detected in the lavage. They had high level of IL-8, IL-2, IL-1β production and ultrastructural signs of hypersecretion which was especially typical of platycytes. High level of IL-4, IL-5 content is typical of relapsing sarcoidosis: additionally to macrophages of M1 phenotype, there are macrophages of M2 phenotype with ultrastructural signs of phagocytic function which can be used for diagnostics of this form of RS

THE FIRST EXPERIENCE IN USING ELASTOGRAPHY IN COMBINATION WITH ENDOBRONCHIAL ULTRASONOGRAPHY FOR MEDIASTINAL PATHOLOGY: PRELIMINARY ASSESSMENT OF FEASIBILITY AND COMPARISON OF CHARACTERISTICS VIA DIFFERENT APPROACHES

Objective: to preliminarily assess the feasibility of elastography in endobronchial ultrasonography and to compare characteristics via different accesses.Material and methods. The investigation enrolled 3 patients (malignant, benign mediastinal adenopathy, paratracheal tumor). Elastography was carried out using a Pentax EB-1970UK echobronchoscope with a Hitachi Noblus ultrasound scanner. All the patients underwent fine-needle aspiration biopsy of mediastinal and pulmonary lymph nodes/masses with 22G needles (Cook, Medi-Globe). Sequential study of the same group of lymph nodes through different approaches through the esophagus and trachea was conducted, and its characteristics were compared in one patient.Results. A total of 8 lymph nodes and one paratracheal mass (Group 4L-7-4R-2R) were assessed in three patients. Reproducible images acceptable for visual assessment were obtained in all cases. The wall of the trachea and bronchi had no significant negative impact on the quality of an obtained image. Both malignant lesion of lymph nodes and paratracheal mass was accompanied by decreased tissue elasticity in the area of interest. Assessment of the same benign lymph node through different approaches revealed a clear discordance between elastographic findings with a drastic decrease in elasticity values via an endobronchial approach.Conclusion. Endobronchial ultrasonography elastography is technically feasible. The wall of the trachea and bronchi has no significant impact on the quality of an obtained image; elastographic data are reproducible during re-measurements. Comparison of elastographic characteristics through different approaches suggests that there is a tendency to overestimate data on lymph node density during endobronchial examination versus a transesophageal approach. Thus, the direct transfer of the accumulated data and patterns of elastographic diagnosis to an endobronchial approach is impossible and likely to require a revision of criteria to estimate malignancy-associated changes during its application