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Objective: to investigate the immunogenicity and safety of 23-valent polysaccharide pneumococcal vaccine in patients with rheumatic diseases (RD). Subjects and methods. The prospective open-label comparative study enrolled 133 people (102 (76.7%) women and 31 (23.3%) men) aged 23 to 76 years, including 79 patients with rheumatoid arthritis (RA), 16 with systemic sclerosis, and 7 with dermatomyositis/polymyositis, as well as 31 subjects without systemic inflammatory RD (a control group), who had a recent history of at least two cases of lower respiratory tract infections (bronchitis, pneumonia). At their inclusion, all the patients with RD were receiving ant-inflammatory therapy, including 52 taking methotrexate (MT), 14 – leflunomide (LEF), and 13 – MT + tumor necrosis factor-α (TNF-α) inhibitors. The 23-valent polysaccharide pneumococcal vaccine Pneumo-23 (Sanofi Pasteur, France) was administered in a single dose of 0.5 ml subcutaneously during continuous MT or LEF therapy for the underlying disease or 3–4 weeks before the use of TNF-α inhibitors. Clinical examinations of the patients and conventional laboratory studies were performed during control visits (1, 3, and 12 months after vaccination). The serum levels of anti-pneumococcal capsular polysaccharide antibodies were measured in 102 patients by enzyme immunoassay using commercial VaccZymeTM Anti-PCP IgG Enzyme Immunoassay kits (The Binding Site Group Ltd, United Kingdom). Results and discussion. No clinical and radiological symptoms of pneumonia were recorded in any case during the follow-up period of 12 months. The patients with RD and the control group showed a significant, more than double increase in anti-pneumococcal antibodies 12 months following vaccination. Vaccination was well tolerated: 90 (68%) patients displayed no adverse events; 37 (28%) had pain, cutaneous swelling and hyperemia up to 2 cm in diameter at the site of injection for vaccination;6 (4%) had low-grade fever. There were no episodes of a RD exacerbation or any new autoimmune disorders during the follow-up period. Conclusion. The findings were suggestive of the sufficient immunogenicity and good tolerability of 23-valent pneumococcal vaccine in patients with RD.

Dermatomyositis (DM) and polymyositis (PM) belong to idiopathic inflammatory myopathies (IIM) and are characterized by inflammatory injury to the skeletal muscles. In DM, unlike PM, there is skin injury that serves as a pathognomonic sign of this condition. Panniculitis (PN) is one of the rare cutaneous manifestations in this disease. Objective: to investigate the clinical and laboratory characteristics of IIM accompanied by PN and to elaborate guidelines for managing these patients. Subjects and methods. Examinations were made in 318 patients (75 men and 243 women) aged 18 to 80 years who were diagnosed with IIM (mean disease duration of 18.97±7.4 months) and followed up at the V.A. Nasonova Research Institute of Rheumatology in 1996 to 2015. Results and discussion. In 12 (3.8%) of the 318 patients, lobular PN that was associated with the diagnosis of DM verified in all cases. The clinical picture in this patient group was also characterized by skin injury as erythema on the face and trunk and Gottron’s papules (100%), periungual capillaritis (91.7%), ulceronecrotic vasculitis (57.3%), periorbital edema (75%), fever (41.7%), alopecia (50%), and joint involvement (25%). Conclusion. The development of PN is associated with the acute period of DM and the emergence of new foci is related to an exacerbation of this disease, which requires active therapy.

Objective: to assess the efficacy and tolerability of Rituximab treatment in patients with serious immune inflammatory rheumatic diseases (IRD) like systemic lupus erythematosus (SLE), systemic sclerosis (SS), systemic vasculitis (SV), Sjogren syndrome (SjS), dermatomyositis/polymyositis (DM/PM). Subjects and methods. The clinical efficacy has been analyzed in 229 patients with IRD: SLE (n=97), SV (n=50), SS (n=40), SjS (n=23) and DM/PM (n=19). Rituximab treatment was accompanied by administration of glucocorticoids and/or immunosuppressive drugs. Most patients demonstrated resistance to or low tolerability of standard therapy. Efficacy of treatment was analyzed in each group with the criteria relevant for each disease. To compare clinical response to the treatment between the groups we used gradations accepted by international registries: complete (good) response, partial response, no response. Average duration of monitoring comprised 72 (1–288) weeks after the first introduction of Rituximab. Average Rituximab dose administered to patients over the period of monitoring was low and varied from 1.6±0.84 in DM/PM to 3.1±1.75 in SV. About 80% of patients received one or two courses of Rituximab except for patients with SS (half of them received three and more courses). Results. «Complete response» was observed in 50.6%, «partial response» – in 35% of patients. Rituximab courses provided positive dynamics in clinical scores and allowed to reduce supportive dose of glucocorticoids and to lower the dose or withdraw of immune-stimulating drugs. Multiple Rituximab courses provided stable and longlasting effect. Recurrences were observed less frequently, whereas efficacy of the therapy increased during a year and longer. Occurrence of adverse events and mortality rate were comparable to data of other national Rituximab registries. Conclusion. The results of the study may prove administration of Rituximab in patients with resistance to standard therapy

Полимиозит (ПМ) и дерматомиозит (ДМ) – аутоиммунные заболевания скелетной мускулатуры неизвестной этиологии, которые относятся к системным заболеваниям соединительной ткани и объединяются общим термином – идиопатические воспалительные миопатии (ИВМ) [1-3].Ведущим клиническим признаком ПМ/ДМ является поражение скелетной мускулатуры, проявляющееся слабостью мышц плечевого и тазового пояса, проксимальных отделов верхних и нижних конечностей, мышц шеи и спины и др. Может развиваться отек мышц. Мышечная слабость нарастает от минимальных проявлений (утомляемость) до выраженного двигательного дефицита в течение нескольких недель или месяцев. Пациенты с трудом встают (или не могут встать) с низкого стула, не могут подняться в транспорт, поднять руки для умывания или причесывания. Наблюдаются также неуклюжая походка и эпизоды неожиданных падений, связанных со слабостью мышц тазового пояса и бедер. Пациенты не могут самостоятельно встать с кровати и оторвать голову от подушки. Мышечные атрофии развиваются у больных, длительно страдающих ПМ/ДМ и не получающих адекватную терапию глюкокортикоидами (ГК). Характерно поражение мышц глотки, гортани и верхней трети пищевода, ведущее к дисфонии, дисфагии. Основными признаками поражения кожи при ДМ являются: эритематозная сыпь на лице, на волосистой части головы, на груди («декольте») и на плечах («шаль»), параорбитальный гелиотропный отек, шелушащаяся эритема на коже тыльной поверхности пястнофаланговых, проксимальных межфаланговых, локтевых и коленных суставов (эритема Готтрона), фотосенсибилизация. Наряду с этим у многих больных наблюдаются такие системные проявления, как феномен Рейно, ревматоидноподобный полиартрит, плотный отек кистей, склеродактилия, рука«механика» и гипотония пищевода, что сближают ПМ/ДМ с другими системными заболеваниями соединительной ткани. У значительного числа больных ИВМ (от 20 до 55%) появляются симтомы дыхательной недостаточности, которая способствует ограничению двигательной активности пациента и обусловлена как сопутствующим интерстициальным поражением легких, так и слабостью дыхательной мускулатуры [1-4].

Idiopathic inflammatory myopathies are rare autoimmune diseases with inflammatory lesions in skeletal muscles. They include polymyositis, dermatomyositis, juvenile myositis and inclusion body myositis. These are clinically and immunologically heterogeneous conditions differently responding to therapy. The authors consider the main manifestations of polymyositis/dermatomyositis and principal differences between them. Therapy is based on the prescription of glucocorticoids in combination with immunesuppressors. Better understanding immunological, genetic, and molecular mechanisms opens up new prospects for the management of idiopathic inflammatory myopathies.

The study was undertaken to examine the impact of glucocorticoid therapy (GCT) on mineral bone density (MBD) in patients with idiopathic inflammatory myopathies. Double-energy X-ray absorptiometry was used to determine MBD of the vertebral column and proximal femur in 42 females (30 with the preserved menstrual cycle (PMC), 12 in postmenopause (PMP) with polymyositis/dermatomyositis (PM/DM). All the patients received GCT. A random population sample including 106 females aged 20 to 69 years formed a control group. MBD of the neck of the femur was significantly lower in patients from all age groups, in the lumbar vertebral column in young (20-29 years) and old (over 50 years) females than in the controls. The development of osteoporosis (OP) was more frequently observed in PMP females: OP in the lumbar spine was in 16.7 and 66.7% of the PMC and PMP females, respectively; that in the neck of the femur was in 11 and 33%. There was no relationship between MBD and the specific features of GCT, namely the duration, cumulative dose, and the maximum dose. There was a reduction in MBD in patients with PM/DM on GCT in all age groups, more frequently in PMP patients.

The paper deals with the problems of optimizing the diagnostic criteria for idiopathic inflammatory myopathies (IIM), a group of heterogeneous rare autoimmune diseases characterized by inflammatory lesion in the skeletal muscles. The representatives of this group are traditionally considered to be polymyositis (PM), dermatomyositis (DM), and inclusion-body myositis. The authors detail the history of classification criteria for IIM from those proposed by T.A. Medsger et al. (1970) relying on its clinical picture, laboratory data and instrumental findings, as well as the criteria (including the first introduced exclusion ones) elaborated by A. Bohan and J.B. Peter in 1975, which remain fundamental in both clinical practice and researches. The basis for the clinical and serological criteria proposed by Y. Troyanov et al. (2005) for IIM is the identification of myositis-overlap syndromes. The classificational (subtype identification) and therapeutic value of the criteria based on clinical and serological characteristics was supported by the Hungarian investigators A. Vancsa et al. (2010) who investigated the relationship between the clinical and therapeutic characteristics of IIM and positivity for myositis-specific and myositis-associated antibodies. The criteria developed by M.C. Dalakas (1991, 2003) are based on the specific immunopathological features of a histological pattern, which allow the differentiation of DM, PM, and inclusion-body myositis from other myopathic syndromes. The 2004 European Neuromuscular Center (ENMC) criteria first identify necrotizing autoimmune myopathy and nonspecific myositis as individual subtypes. The serological classification of IIM, which is based on the assessment of autoantibodies that play an important role in the pathogenesis of the disease, is of indubitable interest. There is an obvious need for the correct and timely diagnosis of both IIM as a whole and its subtypes in particular, which is complicated by the heterogeneity of the latter. The proposed approaches to diagnosing and classifying IIM have their advantages and disadvantages so that work in this direction is yet to be under way.