Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with various organs and systems involved in the pathological process. There is strong evidence that age at disease onset may influence the clinical presentations and prognosis of SLE. The paper analyzes the clinical and laboratory features of onset of SLE after the age of 50 years and possible reasons for differences in the manifestations of the disease in relation to racial and genetic factors. It considers the problems of differential diagnosis. There are data available in the literature and the results of the author's own investigations.

Temporomandibular joint (TMJ) involvement occurs in patients with different rheumatic diseases (RDs). Pain, limitation of mouth opening can lead to significant problems in both oral hygiene and when eating. Conservative treatments for TMJ lesions are not always effective. Objective: to evaluate the efficiency of minimally invasive surgical interventions (TMJ arthrocentesis and arthroscopy) in patients with RDs. Patients and methods. The investigation enrolled 64 patients with different RDs (43 with rheumatoid arthritis, 11 with psoriatic arthritis, 8 with systemic lupus erythematosus, and 2 with ankylosing spondylitis) who were divided into three groups in relation to the severity of TMJ involvement in accordance with the Wilkes classification. All the patients underwent TMJ magnetic resonance imaging at baseline and 6 months after treatment. Also at baseline, 14 days, and 1, 6, and 12 months after surgery, the investigators assessed TMJ pain intensity by visual analogue scale and the parameters of mandibular movements. Patients with Wilkes stages IV and V TMJ involvement underwent arthroscopic intervention into the TMJ and those with III stage received TMJ arthrocentesis with arthrolavage. Results and discussion. After surgical treatment, all the groups were noted to have a significant decrease in TMJ pain intensity compared with the baseline level; moreover, the severity of TMJ pain most significantly decreased on day 7 after surgery. Later on, positive changes remained within subsequent follow-up months. There were data similar in the higher degree of mouth opening. The results of surgical treatment in patients with Wilkes stage V TMJ involvement were worse than in those with stages III and IV. Conclusion. Minimally invasive TMJ surgery in patients with RDs is effective and associated with the low frequency of postoperative complications and exacerbations of RDs. The efficiency of minimally invasive TMJ surgery is higher in patients with the early and middle stages of TMJ involvement according to the Wilkes classification. Further investigations are needed to assess the long-term results of minimally invasive TMJ surgery in patients with RDs.

Idiopathic lobular panniculitis (ILP) is regarded as a clinical and pathological variant of lobular panniculitis with typical clinical symptoms. To assess the nature of the disease and to solve therapy problems, there is a need to clarify and grade disease activity, which is the basis for this investigation. Objective: to create a rating scale for the activity of the inflammatory process in ILP on the basis of clinical and laboratory parameters. Patients and methods. 67 patients (9 men and 58 women) with a verified diagno sis of ILP, who have been followed up at the V.A. Nasonova Research Institute of Rheumatology in 2007 to 2017, were examined. The patients' age was 20 to 76 years; the mean disease duration was 78.91 [48; 540] months. Physical examination, chest computed tomography, immunological and TB tests, and pathomorphological examination of the skin biopsy of the node were carried out; the serum concentrations of α1-antitrypsin, amylase, lipase, ferritin, creatine phosphokinase, and body mass index were determined. Results. Analysis of clinical manifestations could identify four ILP forms: nodular (n=30), plague (n=10), infiltrative (n=15), and mesenteric (n=12) ones that had clinical and laboratory features. Along with lesions of the skin and subcutaneous adipose tissue, 51 (76.11%) patients complained of weakness (p<0.001); 48 (71.64%) had rising body temperatures to febrile values (p=0.0001). 31 (46.26%) patients were noted to have articular syndrome that was characterized by polyarthralgia in 90.32% of cases (p<0.0001) and by knee and/or ankle arthritis in only 3 (9.67%) patients. The examinees showed a significant increase in erythrocyte sedimentation rate (p=0.01) and C-reactive protein levels (p<0.0001). Based on these findings, the authors developed an ILP activity index (AI) that included the characteristics of the status of 7 organ systems. The maximum score for individual systems is 1 to 3 in relation to the number of estimated parameters. Conclusion. The proposed ILP AI is of practical value. There is a need for further investigations and maybe a search for new parameters of ILP activity.

Rheumatoid arthritis (RA) and subclinical hypothyroidism (SHT) have close pathogenetic relationships, the synergism of which worsens the clinical course and increases the risk of visceral complications. Objective: to study the ultrasound parameters of endothelial dysfunction (ED) in patients with RA concurrent with SHT. Patients and methods: 139 patients with RA were divided into two groups: 1) 48 patients with RA; 2) 91 patients with RA concurrent with SHT. The ultrasound parameters of endothelial regulation of vascular tone were determined by intravascular ultrasonography using an ESAOTE MyLAB40 apparatus in accordance with the international guideline. Results and discussion. The study of endothelial regulation of vascular tone established that all the examinees with RA had ED that increased with the higher degree of activity and longer duration of RA. Patients with RA in the presence of SHT had more significant abnormalities in the ultrasound parameters of endothelial regulation of vascular tone and in the degree of ED, which increased with the longer duration and higher activity of the disease. The early detection and correction of comorbidity in patients with RA will reduce cardiovascular risk and improve quality of life.

Objective: to study the efficiency and safety of tocilizumab (TCZ) therapy in patients with systemic-onset juvenile idiopathic arthritis (sJIA). Patients and methods. The retrospective study included 48 children with active sJIA in whom TCZ therapy was initiated because of the inefficiency of using different anti-rheumatic drugs. The investigators studied the time course of changes in the main indicators of sJOA activity, as well as the possibility of achieving remission in accordance with the Wallace criteria (2004). Results and discussion. The main demographic characteristics of the study group (median [25th and 75th percentiles]) included the age of the patients (9.9 [5.0; 12.7] years) and the duration of TCZ therapy (27.0 [5.9; 89.7] months). 40 (83.3%) patients achieved remission; the median of its achievement was 138.5 [56.0; 255.0] days. The patients who had achieved remission had milder sJOA: hepatosplenomegaly, pulmonary and cardiac involvements, and macrophage activation syndrome were less frequently observed. At baseline, they also had higher hemoglobin values and a lower erythrocyte sedimentation rate, and decreased levels of leukocytes, granulocytes, C-reactive protein (CRP), lactate dehydrogenase (LDH) and ferritin. The main predictors for achieving remission, which were estimated using the analysis of sensitivity and specificity, as well as Cox regression models, were CRP ≤82.0 mg/l (OR – 7.9; HR – 1.17); ESR ≤32 mm/h (OR – 17.0; HR – 0.85), ferritin ≤273 ng/ml (OR – 56.5; HR – 2.6), hemoglobin >113 g/l (OR – 17.0; HR – 1.33), LDH ≤676 U/l (OR – 113.6; HR – 3.2), platelets >335⋅109 /l (OR – 5.0; HR – 2.5), and a decline in white blood cells two weeks after the first infusion by more than 11% (OR – 13.0; HR – 6.0) and in granulocytes by more than 12% (OR – 14.0; HR – 4.7). Conclusion. Children with a less severe course of sJIA more promptly reached remission during TCZ therapy. Male gender, high inflammatory activity, previous glucocorticoid therapy, long time to achieve remission, and TCZ dosing regimen deviations were the main factors for an exacerbation of sJOA. Male sex, signs of the high disease activity, preceding CS treatment, long time to achievement of inactive disease and treatment protocol deviations increased risk of sJIA flare.

The paper gives the data available in the literature on the relatively rare disease hypertrophic osteoarthropathy (HOA). This syndrome includes symptoms, such as clubbed fingers (hypertrophy of the distal phalanges), periostosis (subperiosteal new bone formation along the distal diaphysis of the long bones with further progression in the proximal direction) and arthritis, as well as skin changes (pachydermia) that are not an obligate sign. There is information concerning the pathogenesis, clinical, laboratory, and instrumental manifestations of this disease. The paper describes a clinical case of primary HOA in a teenager who has been misdiagnosed with juvenile idiopathic arthritis (JIA). It shows the necessity of including HOA in the spectrum of differential diagnosis in children with articular involvement since HOA may resemble JIA in its clinical manifestations. Patients with primary HOA who have been misdiagnosed with JIA have a long ineffective experience with immunosuppressive and sometimes even glucocorticoid therapies. The specific features that can distinguish the full form of primary HOA from chronic arthritis is the absence of inflammatory changes in the synovial fluid, the presence of clubbed fingers, pachydermia and the lack of morning stiffness.

The review deals with the problem of antibody production in scleroderma systematica (SDS), with a focus on antiribonucleoprotein (RNP) antibodies: frequency, structure, and clinical associations. SDS is a progressive polysyndromic disease with characteristic lesion of the skin, locomotor apparatus, viscera (heart, lungs, digestive tract, and kidneys), and common vasospastic disorders as Raynaud's syndrome, the basis for which are the processes of connective tissue disorganization with a predominance of fibrosclerotic changes and vascular pathology as peculiar endarteritis obliterans. The presence of antibodies to different autoantigens is a distinguishing feature of SDS. Among the patients who meet the classification criteria for SDS, there is a subgroup of patients who are not found to have SDS-specific antinuclear antibodies, but have antibodies to soluble nuclear autoantigens, namely, antibodies to RNP. This type of autoantibodies is described in various systemic connective tissue diseases: systemic lupus erythematosus, dermatomyositis, polymyositis, and SDS. The detection rate of anti-RNP antibodies in SDS varies from 5 to 30% in different ethnic groups. In addition, positivity for anti-RNP antibodies is a characteristic feature of mixed connective tissue disease. A more detailed study of anti-U1-RNP antibody-positive patients with SDS, by identifying a new subtype of SDS, comparing, and searching for dissimilarities of anti-U1-RNP antibody carriers from other well-described phenotypes of SDS, is of interest today. This problem is relevant, by taking into account the personalized approach to following up patients, which is actively being elaborated in rheumatology.

Cardiovascular events (CVEs), such as myocardial infarction, stroke, and sudden cardiac death, which are due to the early development and rapid progression of atherosclerosis, are one of the main causes of mortality in rheumatoid arthritis (RA). Traditional risk factors (TRFs) and immunoinflammatory mechanisms underlying the pathogenesis of these diseases are shown to be responsible for atherosclerosis and its clinical manifestations in RA. Since the key role in the development of atherosclerosis and related CVEs in RA is played by chronic inflammation and autoimmune disorders, effective anti-inflammatory therapy occupies an important place in the prevention of the latter. The review gives an update on the effect of abatacept on overall mortality and CVEs, TRFs for cardiovascular disease, and the clinical and subclinical manifestations of atherosclerosis in RA.

The paper presents an overview of studies assessing health-related quality of life (HRQoL) in patients with systemic lupus erythematosus (SLE). It describes the HRQoL concept and prerequisites for its creation. The classification of questionnaires used in patients with SLE is considered in relation to the study objectives. The main key triad (fatigue, pain, and depression), which causes a reduction in HRQoL in SLE patients, is derived on the basis of many studies. The paper provides brief comparative characteristics of eight specific HRQoL questionnaires in SLE patients: Symptom checklist (SSC), SLEQol, LupusQol, L-QoL, LupusPRO, and Lupus Impact Tracker (LIT), LUP-QoL, and SMILEY. Special attention is paid to HRQoL assessment in patients receiving different treatment options: glucocorticoids, cytostatic and anti-B-cell therapy.

The paper presents an analytical literature review of the use of rituximab (RTM) in patients with systemic lupus erythematosus (SLE). It considers current views on the mechanisms of action of RTM, its efficacy in different organ damages in patients with SLE, particularly in those with lupus nephritis and central nervous system involvement. There is evidence suggesting that it is expedient to use RTM in patients with high activity of SLE, especially when the latter is refractory to standard therapy with glucocorticoids and cytostatics. Attention is paid to the steroidsparing effect of RTM and to the reduction of the risk of irreversible organ damages associated with high-dose prednisone. The paper discusses the possibility of using a RTM biosimilar (Acellbia) and prospects of RTM therapy for early SLE.

Ankylosing spondylitis (AS) is a socially significant rheumatic disease that generally starts in young adults and, if untreated, often leads to temporary incapacitation, and by 40 years, and to disability. Despite the relatively young age of patients, the course of AS is accompanied by a number of comorbidities that most commonly include hypertension, dyslipidemia, and involvement of the gastrointestinal tract and kidney. The main group of drugs recommended for the treatment of the disease is nonsteroidal anti-inflammatory drugs (NSAIDs) that, when used long, exert analgesic, anti-inflammatory, and even disease-modifying effects on the one hand and can cause adverse reactions on the other. When choosing NSAIDs, one should consider not only the activity of the pathological process, but also the presence of comorbidities in the patient. The paper gives data on the efficacy and safety of nimesulide in AS. The drug has proven its efficacy and safety in various rheumatic diseases over nearly a quarter century of its use in Russia.

Gout is a chronic disease that requires permanent urate-lowering therapy. Allopurinol is the gold standard of this therapy. The novel drug febuxostat, a selective xanthine oxidase inhibitor, has been synthesized and introduced into clinical practice in the last 10 years. The paper reviews the literature on the main clinical trials of febuxostat, which show its efficacy that is comparable to or more higher than that of allopurinol, as well as the possibility of using this drug for reduced kidney function, allergic reaction to allopurinol or resistance to therapy with allopurinol, which considerably improves prognosis in these patients. The long-term use of febuxostat is noted to result in almost complete resorption of tophi and in termination of gouty arthritis attacks. These findings allow febuxostat to be considered as a promising and essential medication for the treatment of gout. Great hopes are pinned on the extension of its application; there are ongoing investigations regarding the possibility of using this drug for asymptomatic hyperuricemia and other conditions accompanied by higher uric acid levels.

Aceclofenac is a popular nonsteroidal anti-inflammatory drug that has been used to control pain in rheumatic diseases for more than 25 years. The drug is used in 19 European countries and there are as many as its 298 generics. The good safety profile of aceclofenac makes it the drug of choice for treating osteoarthritis, the most common joint disease. Since 2005 in Russia, there have been 14 investigations of the efficacy and safety of aceclofenac (Airtal®) in 4096 patients. These studies have confirmed the high efficacy of aceclofenac: its therapy reduces pain compared to the baseline level by 52.9±15.9%. The rate of adverse reactions (ARs) averaged about 3%. Controlled studies showed ARs to aceclofenac in an average of 8.8% of cases and those to control drugs (diclofenac, nimesulide, meloxicam, and paracetamol) in 20.2%. It is noted that the experience of the Russian clinical trials demonstrates the high efficacy and favorable safety profile of Airtal®.