Clinical features of overlap autoimmune hepatitis/primary biliary cholangitis and morphological-proved sarcoid lesions (lungs, lymph nodes, skin) were performed. Data of long-term clinical observation presented in comparison with the results of laboratory datas, instrumental and morphological studies of liver tissue, lungs, skin. The modern aspects of pathogenesis of association autoimmune and granulomatous diseases arediscussed on the example of clinical cases of combination of cholestatic variants of autoimmune hepatitis and generalized sarcoidosis.

Идиопатические воспалительные миопатии (ИВМ) -редкие аутоиммунные заболевания, характеризующиеся воспалительным поражением скелетной мускулатуры. К наиболее распространенным ИВМ относят: полимиозит (ПМ), дерматомиозит (ДM), которые характеризуются клинико-иммунологической неоднородностью и различным ответом на лечение. Наиболее часто встречающимся системным проявлением при ПМ/ДМ является поражение дыхательной системы. Развивающиеся при этом респираторные нарушения весьма разнообразны и могут опережать симптомы мышечной патологии. Ключевые слова: идиопатические воспалительные миопатии; полимиозит; дерматомиозит; антисинтетазный синдром; глюкокортикоиды. Контакты: Ольга Алексеевна Антелава; antelavao@gmail.com Для ссылки: Антелава ОА, Бондаренко ИБ, Чичасова НВ, Насонов ЕЛ. Респираторные нарушения при полимиозите/дерматомиозите. Современная ревматология. 2014;(1):31-38. Respiratory disorders in patients with polymyositis/dermatomyositisIdiopathic inflammatory myopathies (IIM) are rare disorders characterized by inflammatory lesions in skeletal muscles. These diseases include polymyositis (PM), dermatomyositis (DM), and inclusion body myositis, which exhibit clinico-immunological heterogeneity and give different response to therapy. The most frequent manifestation in PM/DM patients is respiratory system dysfunction. The developing respiratory disorders are varied and may outpace the presentation of muscle pathology.

We herein report a case of interstitial lung disease secondary to the use of methotrexate in a patient with rheumatoid arthritis. Differential diagnosis between pneumonitis caused by methotrexate in patients treated with basic methotrexate therapy and interstitial pulmonary disease associated with rheumatoid arthritis is based on the clinical examination and instrumental data. The main condition for favorable clinical outcome in all drug-induced lung disease is drug withdrawal, what was proven in our report.

Primary ciliary dyskinesia (PCD) is a rare genetic autosomal recessive disease associated with a defect in the ultrastructure of epithelial cilia. Currently, there is no standard method for diagnosing PCD, so the diagnosis is based on the clinical picture and the results of tests, such as DNA diagnostics, nasal nitric oxide measurements, ciliary beat frequency in a nasal biopsy, ciliary ultrastructure, etc. Diagnosis of PCD can be difficult due to secondary damage to the respiratory epithelium, which often results in undiagnosed or false positive cases. Differential diagnosis with diseases forming widespread bronchiectasis (BE) and upper respiratory tract lesions, especially with cystic fibrosis (CF), is necessary.The aim of this paper is to introduce the difficulties of diagnosis, the appropriate level of detail of the clinical, laboratory and instrumental characteristics over a long period of time, and the organization of care for a patient with PCD. This article describes a clinical case of PCD in a young woman, diagnosed at the age of 17, presents the difficulties and typical mistakes in the management of such patients, and the lack of succession of pediatrician-pulmonologist care.Conclusion. The presented clinical case demonstrates how difficult the diagnosis of PCD is. Such patients need a complex examination, a thorough differential diagnosis to exclude other diseases with a similar clinical picture. Long-term follow-up is carried out by a multidisciplinary team with mandatory microbiological monitoring. The organized care for patients should begin in early childhood and continue in adulthood with proper succession of care and follow-up by of pulmonologist, preferably in specialized centers.

The idiopathic inflammatory myopathies are a group of rare, heterogeneous connective tissue disorders characterized by skeletal muscle inflammation. The four main forms of idiopathic inflammatory myopathies are dermatomyositis, polymyositis, inclusion body myositis, and necrotizing immune-mediated myopathy. Each form of myositis, other than inclusion body myositis, can be associated with damage to many organs, including the lungs, heart, joints, and skin. The most often observed damage to the lung tissue with the development of interstitial lung disease, which occurs with or without myositis. The severity of the course varies from mild to severe, with rapid development of respiratory failure. Interstitial lung disease can be fatal in patients with myositis, therefore, it is necessary to assess the damage to the lung tissue in the early stages of the disease.