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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.

The materials of the Russian NIIR/ARP-COVID-19 database, which included adult (over 18 years old) patients with immuno-inflammatory rheumatic diseases (IIRD) who underwent COVID-19 (coronavirus disease 2019), and a literature review concerning the course and outcomes of COVID-19 in patients with these diseases, are presented. From July 17, 2020 to April 23, 2021, 156 messages were received and analyzed. The study group was represented mainly by women (75%). The average age of patients was 52.0±14.8 years, of which 53 (33.9%) patients were aged 60 years and older. The most frequent clinical manifestations of COVID-19 were fever (62.2%), cough (40.4%), anosmia (37.8%), shortness of breath (33.3%). During the course of COVID-19, computed tomography of the chest organs was performed in 143 patients. When assessed on an “empirical” visual scale, stage 0 of lung lesion was observed in 49 (34.3%) patients, stage 1 in 40 (28%), stage 2 in 35 (24.5%), stage 3 in 19 (13.2%), stage 4 in 0. In the study group, a favorable outcome was observed in 137 (97.9%) patients. Fatal outcome was registered in 3 patients. The effect of COVID-19 on the course of IIRD was assessed in 131 patients: in 52 (39.7%) patients, the course of IIRD worsened, in 1 (0.8%) it improved, in 78 (59.5%) there was no effect. Literature data on the risk of infection with the SARS-CoV-2 virus, the course and outcomes of COVID-19 in rheumatic and musculoskeletal diseases are contradictory, which dictates the need to continue collecting data on the rheumatological problems of COVID-19 in the Russian patient population, taking into account international experience.

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.

Data is presented on the radioactive decay of isotopes 256Rf and 249, 250No synthesized in complete fusion reactions 50Ti + 208Pb, and 48Ca + 204Pb, with subsequent evaporation of neutrons from the excited state of the composite nucleus. Experiments use high-intensity beams of accelerated heavy ions 48Ca and 50Ti from the U-400 cyclotron at the Flerov Nuclear Reactions Laboratory of the Joint Institute for Nuclear Research and the SHELS kinematic separator.

Objective: to determine the frequency, spectrum and severity of liver affection in anti-centromere antibodies (ACA) positive patients with primary Sjogren's syndrome (pSS).Patients and methods. 119 ACA-positive patients with pSS were included in the study, 37 (31%) of them had signs of liver damage, 3 of these patients were excluded from the study (2 had cholelithiasis, 1 had viral hepatitis B). Signs of autoimmune liver damage were found in 34 (28.6%) patients, most of them were seropositive for antimitochondrial antibodies (AMA). The diagnosis of primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH) was established according to the recommendations of the American Association for the Study of Liver Diseases, the Russian Gastroenterological Association and the Russian Society for the Study of the Liver. In 5 (14.7%) patients the cause of cholestasis remained unspecified.Results and discussion. AMA were found in 73.5% of patients, elevated serum IgM levels – in 57.6%. Clinically liver damage in most cases was characterized by an asymptomatic, slowly progressive course without a dramatic increase of symptoms over time. Liver cirrhosis was found in 14.7% of patients. According to clinical, laboratory and morphological manifestations, PBC was diagnosed in 21 patients, 4 of them also had a cross syndrome with AIH. AMA-negative PBC was found in 3 patients and isolated AIH – in 1. In most cases, histological stage I of PBC was detected. During follow-up, median of 7 years (range from 2 to 15 years), in 7 patients with stage I PBC and in 7 AMA-positive patients without functional liver disorders no clinical, laboratory or instrumental progression of liver damage was noted. In this regard, it was suggested that these patients have epitheliitis of the biliary ducts as manifestation of glandular affection in pSS, and not true PBC.Conclusion. Autoimmune liver lesions are detected in 28.6% of ACA-positive patients with pSS, most (41.2%) of them develop epitheliitis of the biliary ducts as pSS manifestation or a combination of pSS with PBC (with the same frequency), less often PBC / AIH cross syndrome is diagnosed. PBC / pSS-related epitheliitis of the biliary ducts in ACA-positive patients is characterized by a slowly progressive asymptomatic course in most cases and rarely leads to the development of liver cirrhosis.

Objective: to compare clinical and laboratory manifestations in 2 groups of patients with primary Sjogren’s syndrome (pSS): with and without anticentromere antibodies (ACA); compare the incidence, clinical and laboratory characteristics of lymphomas in these two groups.Materials and methods. We examined 119 patients with ACA-positive pSS (pSS-ACA+). pSS was diagnosed based on Russian 2001 criteria, systemic sclerosis (SSc) – criteria ACR/EULAR 2013. To diagnose liver diseases, the level of transaminases, alkaline phosphatase and antimitochondrial antibodies (AMA) was determined, as well as liver biopsy. The diagnosis of primary biliary cholangitis (PBC) was established according to the recommendations of the American Association for the Study of Liver Diseases, the Russian Gastroenterological Association and the Russian Society for the Study of the Liver. Lymphomas were diagnosed according to histological, immunohistochemical and molecular studies of affected organs biopsies, according to the classification of the World Health Organization. A combination of pSS and SSc was diagnosed in 37 patients, and they were excluded from further analysis. We compared clinical and laboratory features in patients with pSS-ACA+ (n=82) and ACA-negative pSS (pSS-ACA–, n=64) and characterized lymphomas in the pSS-ACA+ (n = 14) and pSS-ACA– (n=10) groups.Results and discussion. In patients with pSS-ACA+, a later age of disease onset was revealed, the duration of the disease before lymphoma development did not differ. In patients with pSS-ACA+, we found a lower frequency of rheumatoid factor (RF), antibodies to Ro (anti-Ro) and La (anti-La), decreased C3-complement, hypergammaglobulinemia, increased IgG concentration, CRP, increased ESR, leukopenia and anemia. 51.2% of patients with pSS-ACA+ were seronegative for anti-Ro, anti-La and RF. Patients with pSS-ACA+ had a higher frequency of AMA and elevated IgM. The incidence of cryoglobulinemia and paraproteinemia did not differ. The frequency of recurrent parotitis in pSS-ACA+ was significantly lower, there were no differences in the frequency and severity of other signs of salivary and lacrimal gland damage. PBC and epitheliitis of the biliary ducts in patients with pSS-ACA+ were detected significantly more often. Damage to the peripheral nervous system, lungs, hypergammaglobulinemic purpura, arthralgia and autoimmune thyroiditis were significantly more often detected in the group of patients with pSS-ACA–. In the pSS-ACA+ group, Raynaud’s phenomenon was significantly more frequent, mainly with scleroderma-type capillaroscopic abnormalities. There was no difference in the frequency of other signs characteristic of SSc. MALT lymphomas were diagnosed in the study groups with the same frequency. Patients with lymphomas in the pSS-ACA+ group were characterized by significantly higher laboratory activity. All patients with lymphomas in both groups showed persistent parotid salivary gland enlargement. Lymphomas in both groups developed in patients with late stage salivary and lacrimal gland damage, systemic manifestations of pSS in both groups were rare.Conclusion. pSS-ACA+ is an independent subtype of pSS, which has a number of significant clinical and laboratory differences from the “classic” variant of the disease. ACA in pSS are associated with a low frequency of anti-Ro, anti-La, and RF, as well as an increased risk of PBC and limited SSc. MALT lymphomas in the pSS-ACA+ and pSS-ACA– groups developed with the same frequency and were associated with the progression of glandular damage, regardless of the presence of systemic manifestations.

Objective: to study clinical and laboratory features in patients with anticentromere antibody (ACA)-positive SjЪgren's disease (SD), as well as the sensitivity of different methods for determination of ACA, and to elaborate an algorithm for differential diagnosis in ACA-positive patients.Patients and methods. The V.A. Nasonova Research Institute of Rheumatology followed up 136 patients who were highly positive for ACA. The investigators used the 2001 Russian criteria for the diagnosis for SD; the 2013 ACR/European League Against Rheumatism (EULAR) criteria for that of scleroderma systematica (SDS); the guidelines of the American Association for the Study of Liver Diseases, the Russian Gastroenterological Association, and the Russian Society for the Study of the Liver for that of primary biliary cholangitis (PBC)/biliary duct epitheliitis in the presence of SD. Lymphomas were diagnosed by biopsies of affected organs according to the WHO classification. SD was diagnosed in 119 patients; SDS in 49 cases (37 with SDS concurrent with SD and 12 with isolated SDS), PBC/biliary duct epitheliitis in 23 (all cases with PBC/biliary duct epitheliitis concurrent with SD and/or SDS); 5 patients were excluded from the investigation. Further analysis included 131 ACA-positive patients. The patients were divided into three groups: SD (n=82 or 62.6%); SD+SDS (n=37 or 28.24%); SDS (n=12 or 9.16%).Results and discussion. Autoantibodies to centromere peptide (CENP) A and CENP-B in the same titers were detected in all ACA-positive patients, regardless of diagnosis. Comparative analysis of three patient groups revealed no statistically significant differences in the frequency of laboratory deviations. The signs characteristic of classical SD (rheumatoid factor (RF)), anti-Ro and anti-La antibodies, leukopenia, higher ESR values, hypergammaglobulinemia, and elevated IgG/IgA levels) were found in a small proportion of patients. The frequency and severity of glandular manifestations did not differ in SD and SD + SDS. PBC/biliary duct epitheliitis was present in 17.5% of ACA-positive patients (in most antimitochondrial antibody-positive cases); no statistically significant differences in its frequency were found between the groups. Other extraglandular manifestations in SD and SD + SDS were identified in a smaller number of patients. All sclerodermic spectrum manifestations were more common in SD and SD + SDS than in BS. Pulmonary arterial hypertension was not diagnosed in any patient from the SD group. MALT lymphomas were detected in 19 ACA-positive patients. Those were present only in BS patients and absent in the SDS group. MALT lymphomas developed in the first 10 years after the onset of SD. The transformation of MALT lymphoma into diffuse large B-cell lymphoma was observed in 2 patients. The main signs of lymphomas in SD patients were persistent parotid salivary gland enlargement, decreased levels of complement C4 and peripheral blood CD19+ cells, as well as cryoglobulinemic vasculitis, serum monoclonal secretion, lymphoid infiltration in the minor salivary glands (a focus score of >4), and severe damage to the salivary and lacrimal glands.Conclusion. ACA-associated SD is an independent disease subtype characterized by an increased risk for SDS, PBC, and MALT lymphomas and by a low frequency of the systemic manifestations and laboratory signs characteristic of classical SD. Regardless of the detected type of antibodies and the presence or absence of extraglandular manifestations, damage to the salivary and lacrimal glands progresses in SD, which often leads to lymphomas; therefore, the therapy that may prevent this complication should be initiated as soon as possible after SD diagnosis. The lymphoproliferation signs identified in this investigation should be taken into account in all ACA-positive patients with SD for the early diagnosis of lymphoid tumors before therapy is prescribed. An algorithm for differential diagnosis in seropositivity for ACA is presented. Determination of autoantibodies to CENP-A and CENP-B does not allow the differential diagnosis in ACA-positive patients.

This paper considers the new 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease, discusses essential differences with IgG4-RD comprehensive diagnostic criteria (Umehara H., 2011) and comments their potential use in clinical practice.

International audience; A great many experimental studies devoted to exploring in detail the properties of radioactive decay of the isotopes of transfermium elements by means of alpha, beta, and gamma spectroscopy have been performed over the past fifteen years at the Flerov Laboratory of Nuclear Reactions (Joint Institute for Nuclear Research, Dubna). High-intensity accelerated heavy-ion beams of ${}^{22}$Ne, ${}^{48}$Ca, ${}^{50}$Ti, and ${}^{54}$Cr from the U-400 cyclotron were used in experiments with the SHELS kinematic separator. An upgrade of the separator and detector system is planned in the future with the aim of improving the transportation efficiency for the nuclei under study and the detection efficiency for gamma rays.

IgG4-related disease (IgG4-RD) is a rare immune-mediated fibroinflammatory disease that is characterized by the occurrence of nodules in one or more organs and proceeds in most patients with the elevated levels of IgG4 in serum and/or in the tissues of the affected organs. The majority of patients are elderly men, and the disease in most cases has a slowly progressing systemic course. The cases of isolated IgG4-related injury to the viscera, which are much less common than the systemic type of the disease, are a very difficult differential diagnosis, and biopsy of these organs is associated with technical difficulties and poses a threat to the patient's health. The paper describes just such a case. It is interesting from several points of view: firstly, a rare site (solitary pulmonary nodular lesion); secondly, clinical and laboratory features (childhood onset, no immunological abnormalities – both higher serum IgG4 levels and lower complement components).Clinicians should remember that IgG4-RD does not always have a systemic course and characteristic serological markers. In these cases, histological verification of the diagnosis is of particular importance. If the histological pattern of a fibroinflammatory pseudotumor is identified, especially in the presence of multiple nodules, IgG4-RD should always be included in the differential diagnosis.

The experiments of detailed study of No and Rf isotopes radioactive decay properties in complete fusion reactions 50 Ti+ 208 Pb and 48 Ca+ 208,206,204 Pb with subsequent neutron evaporation from the excited compound nucleus at the kinematic separator SHELS were performed in FLNR JINR. The data of the 256 Rf decay properties and preliminary data of 250 No decay properties are presented.

Isomeric states in $^{256}\mathrm{No}$ were investigated using internal conversion electron and $\ensuremath{\gamma}$-ray spectroscopy with the GABRIELA detection system at the focal plane of the SHELS recoil separator, at the Flerov Laboratory for Nuclear Research (FLNR, JINR, Dubna). The nuclei of interest were produced using the highly asymmetric fusion-evaporation reaction $^{238}\mathrm{U}(^{22}\mathrm{Ne},4n)^{256}\mathrm{No}$. The emission of internal conversion electrons and $\ensuremath{\gamma}$ rays occurring between a $^{256}\mathrm{No}$ implantation and a subsequent $\ensuremath{\alpha}$-decay event were studied, resulting in the observation of high-$K$ isomerism in this nobelium isotope. The nature of the isomeric states is discussed in terms of possible two- and four-quasiparticle structures.

Objective: to study the clinical and laboratory features of patients with anticentromere antibody (ACA) positive Sjö gren’s syndrome (SjS); to assess the spectrum of autoantibodies in patients of this group; to determine the frequency with which the SjS patients who are highly positive for ACA, meet the international classification criteria for SjS and systemic sclerosis (SS); to reveal the incidence of MALT lymphomas in this patient group; to estimate the incidence of primary biliary cirrhosis (PBC)/biliary lesions as part of autoimmune epithelitis in SjS in this patient group.Material and methods. A total of 83 patients with ACA positive SjS were comprehensively examined at the V.A. Nasonova Research Institute of Rheumatology during the period 2012 to 2018. The inclusion criteria were con formity to the 2001 Russian SjS criteria and a high ACA level. MALT lymphomas were diagnosed on the basis of histological and immunohistochemical studies and polymerase chain reaction-based determination of B-cell clonality in the biopsy samples of affected organs according to the World Health Organization classification of Hematopoietic Tumors. The diagnosis of PBC/biliary lesions was made on the basis of histological and immunohistochemical studies of liver biopsy specimens.Results and discussion. The investigation revealed low detection rates for anti-Ro antibodies (32.5%), anti-La antibodies (7.2%) and rheumatoid factor (RF) (21.7%), which were typical for the classical SjS immunophenotype), increased ESR (14%), leukopenia (7%), hypergammaglobulinemia (17.6%), elevated levels of IgG (9.5%) and IgA (18.7%), and hypocomplementemia (16.1%) in the ACA positive SjS patients. Despite the low detection rate of RF, 15 (18%) patients in this group developed MALT lymphomas: 14 patients had salivary gland MALT lymphoma and one patient had tonsil MALT lymphoma with peripheral lymph node involvement (generalized marginal zone lymphoma). Also, the patients of this group showed high detection rates for AMA antibodies (34.6%), increased IgM level (29.7%) and a higher risk for PBC/biliary lesions as a manifestation of autoimmune epithelitis in SjS (14.5%). AMA-antibodies were absent in only two patients who were diagnosed with liver disease according to biopsy specimens. Nervous system and renal lesions, antiphospholipid syndrome, rheumatoid arthritis, hypergammaglobulinemic purpura, and cryoglobulinemic vasculitis were much less common and sporadic. Also ACA-positive SjS patients often have Raynaud’s phenomenon (54.9%) with scleroderma-type capillaroscopic changes (68%) and a limited form of SS (24%) according to the 2013 ACR criteria.Conclusion. ACA-positive SjS is a subtype of the disease, which is significantly different from the classic one in a number of clinical and laboratory signs and characterized by an increased risk for SS, MALT lymphomas, and PBC/biliary lesions as a manifestation of autoimmune epithelitis in SjS which in some cases leads to the underdiagnosis of SjS. ACA should be considered as pathogenetically related to SjS autoantibodies; and all patients who are seropositive for ACA should be examined for SjS and PBC/biliary lesions as a manifestation of autoimmune epithelitis in SjS regardless of whether they have SS or not, as well as complaints of dry mouth and eyes. Patients with significantly enlarged salivary glands should undergo biopsy to rule out or confirm MALT lymphoma before initiating hormonal, antilymphoproliferative, and anti-B-cell therapy.

to propose diagnostic algorithm of IgG4-related disease (IgG4-RD).One center retrospective research. 52 pts with IgG4-RD were included. The diagnosis was proved histologically and immunohistochemically. 48 out of 52 pts received treatment. Treatment included one of the following schemes (along with low oral glucocorticoids): rituximab monotherapy, cyclophosphamide monotherapy or their combination.The mean age was 47.4±5.9 years, the mean age of the disease onset was 43.9±16.0 years. Median time before the diagnosis was 24 months. The most often sites of IgG4-RD were lacrimal (63.5%), salivary (46.2%) glands, lungs (48%), lymph nodes (34.6%) and retroperitoneum (17.3%). In clinical picture the leading complain was organ enlargement, but not its dysfunction. Pain was characteristic for retroperitoneum localization. In 56.8% of pts with IgG4-related syalo - and/or dacryoadenitis there was association with ear - nose - throat organs affection. In 4 pts (7.7%) IgG4-RD was combined with some malignant disease, including MALT-lymphoma of lacrimal glands. Irreversible organ damage as an IgG4-RD outcome had 15.4% of pts. The main laboratory markers of IgG4-RD were ESR elevation (38.5%), blood eosinophilia (9.6%), immunological disturbances (serum total IgG and IgG4 elevation, IgE elevation, antinuclear antibodies, rheumatoid factor detection, hypocomplementemia). Serum IgG4 level1.35 g/l was elevated in 88% of pts and correlated with the number of affected organs (Spearman correlation coefficient 0.39, Student's test, р=0.0056). Monoclonal serum secretion and B-cell clonality in the tissue was detected in 4 (23.5%) out of 17 pts, but not all of them had both signs.Based on the analysis of clinical and laboratory characteristics of IgG4-RD a diagnostic algorithm was proposed that enhances the detection and examination of the patients with suspected IgG4-RD.Цель: предложить алгоритм диагностики IgG4-связанного заболевания (IgG4-СЗ). Материалы и методы. Одноцентровое ретроспективное исследование, в которое включено 52 пациента с IgG4-CЗ. Диагноз верифицирован гистологически и иммуногистохимически. Из 52 пациентов 48 лечились медикаментозно. Лечение проводилось по одной из схем (на фоне низких доз пероральных глюкокортикоидов): монотерапия ритуксимабом (РТМ); монотерапия циклофосфамидом (ЦФ); комбинированная терапия РТМ и ЦФ. Результаты. Средний возраст пациентов составил 47,4±15,9 года, средний возраст дебюта заболевания 43,9±16,0 лет. Медиана времени с момента дебюта до постановки диагноза - 24 мес. Наиболее часто поражались слезные (63,5%), слюнные (46,2%) железы, легкие (48%), лимфатические узлы (34,6%) и забрюшинное пространство (17,3%). В клинической картине преобладали жалобы на увеличение органов, а не на снижение их функции. Наличие болевого синдрома характерно для пациентов с ретроперитонеальным фиброзом. У 56,8% пациентов с IgG4-связанным сиало - и/или дакриоаденитом имелась ассоциация с поражением ЛОР-органов. У 4 (7,7%) пациентов IgG4-СЗ сочеталось с онкологическим заболеванием, в том числе с MALT-лимфомой слезных желез. Необратимые поражения органов как исход IgG4-СЗ были у 15,4% пациентов. Основными лабораторными отклонениями у пациентов с IgG4-СЗ были повышение скорости оседания эритроцитов (38,5 %), эозинофилия в крови (9,6%), иммунологические нарушения (повышение уровня общего IgG и IgG4 сыворотки, повышение IgE, выявление антинуклеарных антител, ревматоидного фактора и снижение уровня компонентов комплемента). Уровень IgG41,35 г/л повышен у 88% пациентов и положительно коррелировал с числом пораженных органов (коэффициент корреляции Спирмена 0,39, критерий Стьюдента, р=0,0056). Наличие моноклональной секреции в сыворотке крови и наличие В-клеточной клональности в ткани выявлено у 4 (23,5 %) из 17 исследованных, однако они не всегда сочеталась у одного пациента. Заключение. На основании анализа клинико - лабораторных проявлений IgG4-СЗ предложен алгоритм диагностики данного заболевания, который улучшит выявление и качество обследования пациентов с подозрением на IgG4-СЗ.

Objective : to identify the main types of lymphoma with the onset of involvement of the major salivary glands (MSGs), which are encountered in rheumatologic practice, and to evaluate the efficiency of minimally invasive incisional biopsies of the MSGs/lacrimal glands in the diagnosis of different diseases affecting the parotid, submandibular, sublingual, and lacrimal glands. Patients and methods . A total of339patients (291 women and 48 men) aged 18 to 78 years (median age, 55 years) with MSG involvement were examined at the V.A. Nasonova Research Institute of Rheumatology in 2004 to 2017. Minimally invasive incisional biopsies of the parotid, submandibular salivary (SMSG), and lacrimal glands were carried out to verify the diagnosis. Results and discussion . Different variants of hematological malignancies with MSG involvement were diagnosed in 187 (55%) patients; nonneoplastic diseases were in 152 (45%) patients. Primary tumors with the onset of MSG involvement were detected in 52 (15.3%) patients. Hematologic malignancies were diagnosed in 32 (61.5%) cases and 20 (38.5%) patients had various benign neoplastic or infectious diseases with MSG involvement. The prevailing hematologic malignances with MSG involvement were non-Hodgkin lymphomas (NHL), indolent MALT-lymphomas in 12 (23%) patients, follicular lymphomas in 6 (11.5%), and AL-amyloidosis affecting the SMSG in 9 (17.3%). The diagnosis was morphologically verified using minimally invasive incisional biopsy of the salivary/lacrimal glands; in all cases, the biopsy was informative for full morphopathological examination and diagnosis verification. Conclusion . Minimally invasive incisional biopsy of the MSG/lacrimal glands should be actively used in dentistry, rheumatology and blood oncology hospitals for verification of nosological diagnoses when the MSG and lacrimal glands are involved. A sufficient number of tissues obtained using these techniques allows one to abandon severe surgical interventions (partial and complete parotidectomy, SMSG removal, and orbitotomy) currently used for diagnostic purposes in various types of hospitals.

IgG4-related disease (IgG4-RD) is a systemic immune mediated condition that is characterized by the formation of tumor-like fibroinflamma-tory foci in different organs and by the elevation of serum and tissue IgG4 levels in the majority of patients. The pathogenesis of the disease, including the role of IgG4, has not been established exactly. Serum and tissue IgG4 hypersecretion is a nonspecific sign and occurs in many rheumatic, infectious, and malignant diseases. Objective : to determine the range of nosological entities associated with the increase in serum IgG4 levels, as well as the frequency and nature of this increase in patients with IgG4-RD. Patients and methods . The results of all serum IgG4 measurements carried out in the Laboratory of Immunology and Molecular Biology of Rheumatic Diseases, V.A. Nasonova Research Institute of Rheumatology, in 2017—2018 were analyzed. Serum IgG4 parameters were separately estimated in 52 patients with verified IgG4-RD according to the universal diagnostic criteria proposed by H. Umehara et al (2011). Results and discussion . In 2017—2018, a total of247patients were tested for serum IgG4 levels. The latter were elevated in 76 (30.8%) patients, but only 28 (36.8%) were diagnosed as having IgG4-RD. Along with IgG4-RD, antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis, rheumatoid arthritis (RA), and systemic lupus erythematosus (SLE) were characterized by increased serum IgG4 levels. The highest median IgG4 level was found in patients with a verified diagnosis of IgG4-RD: 6.31 vs 3.2, 3.22, and 2.69 g/L in ANCA-associated vasculitis, RA, and SLE, respectively. In 52 patients with IgG4-RD, the IgG4 level >1.35 g/L was found in 88% of cases. The median serum IgG4 level was 3.45 g/L (2.1; 11.4). The highest level was observed in patients with generalized lymphadenopathy and in those with IgG4-related sialoadenitis and dacryoadenitis (Mikulicz disease). The serum IgG4 level was positively correlated with the number of affected organs (Spearman's correlation coefficient, 0.39; p=0.0056, Student's t-test). All the patients showed a tendency towards decreasing serum IgG4 levels during treatment regardless of its clinical response; however, the levels returned to normal only in 73% after 12 months of treatment. Conclusion . The increased serum and tissue IgG4 concentration is not specific, but at the moment it is the only disease marker available in clinical practice. To correctly interpret the diagnosis, it is necessary to assess the entire set of clinical manifestations, imaging data, and morpholopathological findings.

The decay properties of the ground state and excited states of $^{257}$Rf have been investigated with the detector array GABRIELA at the FLNR, Dubna. The electromagnetic decay of a new excited state in $^{253}$No has been observed. The state lies 750 keV above the ground state and is favourably populated in the alpha decay of the low-lying spin isomer of $^{257}$Rf. It decays to the 9/2$^-$ ground state by an M1 transition and is assigned the 11/2$^-$[725] Nilsson configuration. The presence of this state suggests a possible reinterpretation of the decay of the high-K isomer in $^{253}$No., Comment: 7 pages, 4 figures

The neutron rich $$^{123}$$ Ag nucleus was populated via induced photofission of UC $$_x$$ at the ALTO ISOL facility. Its $$\beta $$ -decay properties were studied by means of detecting $$\beta $$ -delayed $$\gamma $$ and neutron activities. The measured half-life T $$_{1/2}$$ = 0.350(20) s agreed with the earlier data; the $$\beta $$ -delayed neutron emission branching P $$_{1n}$$ = 1.01(24)% was reestablished using $$\gamma $$ and neutron counting.

International audience; In the reaction of complete fusion of $^{48}$Ca-ions with a $^{204}$Pb target, a new α line, related to the decay of a new, previously unknown isotope ($^{249}$No), has been detected in an experiment carried out at the SHELS separator. The α decay energy (E$_{α}$ = 9129 keV) and the half-life (T$_{1/2}$ = 38.1 ± 2.5 ms) are measured using the GABRIELA detector array. The SF branching ratio was estimated (b$_{SF}$ ≤ 0.2%).

International audience; Spontaneous fissions of the $^{252}$No and $^{254}$No nobelium isotopes have been studied in a series of experiments with the SHELS separator. These isotopes are produced by colliding a beam of $^{48}$Ca ions from the U-400 cyclotron with the $^{206}$Pb and $^{208}$Pb targets. The measured characteristics of the $^{252}$No and $^{254}$No spontaneous fissions include their half-lives, total kinetic energies of fission fragments, and prompt-neutron multiplicities. The average number of neutrons per $^{254}$No spontaneous-fission act is measured for the first time as 4.88 ± 0.53.

IgG4-related disease is immunomediated fibroinflammatory condition, characterized by tumefective lesions in different organs with distinctive pathomorphological features and IgG4 hypersecretion in serum and tissues in the majority of patients. IgG4-RD has been established as a separate clinical in the early 2000s. In the review we focus on the evolution of views on ethiopathogenesis of the disease, therapeutic and diagnostic options and classification of the disease.IgG4-связанное заболевание (IgG4-CЗ) системное иммуноопосредованное фибровоспалительное заболевание с опухолеподобным характером поражений органов, характеризующееся характерной патоморфологической картиной и гиперсекрецией IgG4 в сыворотке и тканях большинства пациентов. Как отдельная нозологическая единица IgG4-CЗ сформировалось лишь в начале 2000 г. В обзоре рассматривается эволюция взглядов на этиологию и патогенез заболевания, подходы к его диагностике, классификации и лечению.

International audience; The neutron yield from the spontaneous fission of the short-lived neutron-deficient$^{254}$Rf nucleus produced in the complete fusion reaction with the beam of multiply charged ions from the U400 accelerator at the Flerov Laboratory of Nuclear Reactions (FLNR), Joint Institute for Nuclear Research (JINR), is measured using the combined detection system of the SHELS velocity filter. The half-life and the branching ratio of$^{254}$Rf are also measured. The data on the multiplicity of prompt neutrons from spontaneous fission of$^{256}$Rf ($\bar {\nu }$ = 3.87 ± 0.34) are presented for the first time.

The analysis of fission events following the implantation of evaporation residues produced in the fusion reaction of 50Ti and 209Bi at different bombarding energies has revealed 5 millisecond decays, which are attributed to the spontaneous fission of proton-evaporation channels. The average cross sections for proton evaporation are found to be ∼100 and 10 times smaller than the largest neutron-evaporation channel cross section at the same excitation energy. These results suggest that the proton evaporation channel, albeit weak, may represent a realistic alternative to synthesize new, more neutron rich super heavy nuclei. Keywords: Fusion-evaporation reaction, Proton evaporation, Super heavy nuclei, Cross section

International audience; The TETRA long neutron counter is operated at ALTO ISOL facility behind the PARRNe mass separator. TETRA has been proven to be a unique instrument for measurements of β-decay properties of short-lived neutron-rich nuclei having applications for the nuclear structure and/or astrophysical r-process calculations. A proper calibration of TETRA can allow validation of the experimental procedure used for determination of β-delayed one-neutron emission probabilities (P1n). It requires the use of a well-known β-neutron decaying radioactive source which can be only produced and measured on-line due to its short half-life. Thus, the present paper reports on measurements of P1n and T1/2 of 96,97Rb nuclei using TETRA . The results obtained are in a good agreement with the values available in the literature. This proves that the developed techniques can be applied to unknown P1n and T1/2 of neutron-rich species.

International audience; Experiments aimed at synthesizing and investigating the features of radioactive decay of isotopes of superheavy elements (Z ≥ 110) have been conducted in the Flerov Laboratory of Nuclear Reactions at the Joint Institute for Nuclear Research. Complete fusion reactions of an accelerated beam of$^{48}$Ca ions with transactinide targets with the subsequent evaporation of several neutrons from excited compound nucleus are typically used in these experiments. “Exotic” reaction channels with the evaporation of protons and several neutrons may be used to produce more neutron-rich isotopes of superheavy elements. The production cross sections were measured in experiments at the SHELS separator for Db and Rf isotopes produced in xn and pxn channels of the$^{50}$Ti +$^{209}$Bi →$^{259}$Db* reaction. The ratio of cross sections σ(xn)/σ(pxn) was on the order of 100.

IgG4-related disease (IgG4-RD) is a fibroinflammatory condition characterized by the occurrence of tumor-like foci in different organs with a unique histological pattern (moirо-like fibrosis, obvious lymphoplasmacytic infiltration with large numbers of IgG4+ plasma cells, and obliterating phlebitis) and elevated serum IgG4 levels in the majority of patients. Its first-line therapy is glucocorticoids at a starting dose of 0.6 mg/kg/day (equivalent to prednisolone); however, this treatment entails a great number of adverse events and high recurrence rates. The paper provides a review of today's literature on the treatment of IgG4-RD; particular emphasis is laid on the description of therapy with glucocorticoids and rituximab.

The paper describes Russia's first diagnosed case of Erdheim--Chester disease (systemic histiocytosis) in a 65-year-old man who has been long treated for Ormond's disease (idiopathic retroperitoneal fibrosis). It also gives the data available in the literature on the pathogenetic components of these diseases and on the similarity of many clinical, laboratory, and morphological characteristics of these two immunoinflammatory diseases and covers the issues of their differential diagnosis. Invasive procedures with a careful morphological/immunomorphological examination of biopsy specimens obtained from affected tissues are shown to be necessary for accurate diagnosis.Описан первый случай диагностики в России системного гистиоцитоза - болезни Эрдгейма-Честера у 65-летнего мужчины, длительно получавшего терапию по поводу болезни Ормонда (идиопатического ретроперитонеального фиброза). Приведены данные литературы по патогенетическим звеньям, сходству многих клинических, лабораторных и морфологических характеристик этих двух иммуновоспалительных заболеваний, освещены вопросы дифференциальной диагностики. Показано, что проведение инвазивных процедур с тщательным морфологическим/иммуноморфологическим исследованием биоптатов пораженных тканей является необходимым условием для правильной постановки диагноза.

International audience; In the last years we carried out several experiments aimed to investigate properties of short-lived SF isotopes. The neutron-deficient isotopes of nobelium were produced in fusion-evaporation reactions using 206,208Pb targets and an intense 48Ca-beam. Fusionevaporation residues were separated by the SHELS separator and implanted into a largearea double-sided 48×48 strip silicon detector surrounded by 3He-based neutron counters. Half-lives and decay branching ratios for 252,254No isotopes were measured. The average number of neutrons per spontaneous fission of 254No determined for the first time.

The GABRIELA [1] set-up is used at the FLNR to perform detailed nuclear structure studies of transfermium nuclei. Following the modernization of the VASSILISSA separator (SHELS) [2] the GABRIELA detection system has also been upgraded. The characteristics of the upgraded detection system will be presented along with results from some recent electronics tests., Comment: 6 pages, 3 figure, 1 table uncorrected pre-print of EXON 2018 conference proceedings

International audience; TETRA long neutron counter has proven to be an unique tool to identify and to measure delayed neutron emission from photo-fission fragments produced at ALTO ISOL facility. TETRA uses robust technology based on 3He filled-counters operating in the proportional mode. The cross-section of the reaction (n, 3He) is very larger for the thermal neutron energy range. Thus, β-delayed neutrons are moderated and can be detected with a high efficiency. However, due to the moderation process, information on the initial neutron energy is typically lost. Energy of emitted neutrons is needed for detailed description of the process of delayed (multi) neutron emission from neutron rich nuclei. In the present paper we discuss one of a method which can be used to recover information on the initial neutron energy.