The article presents a review of the literature and a clinical observation of a patient with long-term anamnesis of primary Sjцgren's syndrome (SS) in combination with sporadic inclusion body myositis (sIBM). The diagnosis of SS was confirmed in accordance with the Russian diagnostic criteria for SS 2001, as well as with the ACR 2012 and ACR/EULAR 2016 criteria. The diagnosis of sIBM was established on the basis of a characteristic clinical picture: the development of the disease in a woman after 50 years of age with slowly progressive asymmetric muscle weakness and a typical distribution, a moderate increase in the level of creatine phosphokinase (<10 norms for the entire observation period), the presence of a generalized primary muscle process according to needle electromyography, a typical picture of muscle involvement according to magnetic resonance imaging, and the ineffectiveness of high doses of glucocorticoids. The absence of histological confirmation does not contradict the diagnosis, since morphological examination of muscles in patients with a typical course of the disease fails to detect characteristic signs of sIBM in 20% of cases.Currently, there is no effective pathogenetic therapy for sIBM. Understanding the mechanisms of sIBM development will allow to develop effective methods of its treatment.

Objective: to analyze the nosological spectrum, demographic, clinical and laboratory characteristics of diseases with a significant enlargementof major salivary (SG) / lacrimal glands, and/or accessory organs of the eye and paranasal sinuses lesions in rheumatological practice.Patients and methods. This work includes 73 patients who underwent a complex clinical and laboratory, imaging, pathomorphological and histomolecular examination, which was necessary to establish a nosological diagnosis. In all cases, the diagnosis was confirmed pathomorphologically.Results and discussion. Sjogren's syndrome (SjS) was diagnosed in 30 (41%) patients (14 of them developed lymphoproliferative disorder, LPD, as a complication), granulomatosis with polyangiitis (GPA) – in 12 (16.4%), IgG4-related disease (IgG4-RD) – in 10 (13.7%), sarcoidosis – in 6 (8.2%), non Langerhans cell histiocytosis – in 2 (2.7%), AL-amyloidosis – in 1 (1.4%), Warthin's tumor – in 1 (1.4%), chronic atrophic rhinitis – in 1 (1.4%), infectious lesions – in 3 (4.1%) (HIV-associated – in 2, dirofilariasis – in 1), idiopathic inflammatory pseudotumor – in 6 (8.2%). In 1 (1.4%) patient, the diagnosis could not be established.A massive increase of major SG was observed in 46 patients, more often (in 28 cases) with SjS with LPD or without it, with IgG4-RD (in 7) and sarcoidosis (in 6). Orbital lesions were observed in 18 patients: in 7 with IgG4-RD, in 5 with idiopathic inflammatory pseudotumor, in 2 with sarcoidosis, in 2 with GPA, and in 1 each with non Langerhans cell histiocytosis and dirofilariasis. Nasal lesions in the form of chronic rhinosinusitis with or without nasal septum perforation, were found in 18 patients, 12 of whom suffered GPA and 6 – IgG4-RD.Two algorithms, that can facilitate the choice of additional studies and the direction of diagnostic search have been proposed for practicing rheumatologists.Conclusion. Taking into account the possible similarity of clinical manifestations of the diseases with the formation of mass-like tissue, the differential diagnosis should be based on pathomorphological study.

The article provides a literature review on the current understanding of respiratory tract damage in primary Sjögren’s syndrome (pSS) with an emphasis on interstitial lung disease (ILD), as well as approaches to the differential diagnosis, treatment, and screening of pSS-ILD.