Inflammatory bowel diseases (IBD) Crohn's disease (CD) and ulcerative colitis (UC) are a group of idiopathic abnormal bowel conditions with unclear etiology and similar clinical courses. In addition to intestinal syndrome, these diseases are often associated with extraintestinal manifestations, the most common of which is the involvement of the peripheral joints and axial skeleton. The increase in the incidence of IBD, especially CD, in childhood warrants further study of this condition's features, including the most common extraintestinal manifestation: joint involvement (JI) in pediatric patients with IBD. Aim. To analyze the features of JI in CD and UC and the timing of its occurrence associated with IBD onset. Materials and methods. We reviewed 146 case histories of patients aged 4 to 18 diagnosed with CD and UC treated in the Gastroenterological Department and Rheumatological Department No.1 of the Mother and Child Center of Sechenov University from 2017 to 2024. The diagnosis of CD and UC was established following the clinical guidelines of the Union of Pediatricians of Russia for diagnosing and treating CD (2021) and UC (2024) in children. A rheumatologist examined the joints and assessed the JI. The classification criteria of the Assessment of SpondyloArthritis International Society (ASAS, 2011) for predominantly peripheral spondyloarthritis and the Garmisch-Partenkirchen JAS Diagnostic Criteria (1987) were used to verify the cause of the joint involvement. Mean values and standard deviation (M±σ) were used to summarize and estimate demographic continuous variables. Absolute values and percentages were calculated for patients in each study group. Results. Of the 146 patients, 60 (41%) had a combination of JI and IBD. Sixty children aged 4 to 18 with IBD and JI were divided into three groups: 36 (60%) patients with CD, 19 (32%) with UC, and 5 (8%) children with undifferentiated colitis (UndC). The mean age of patients with CD was 14.06±0.5, with UC–13.6±0.3, and UndC–15.2±0.2 years. Among all 60 patients, JI manifested with peripheral arthritis (PA) in 25 (42%) subjects, spondyloarthritis (SpA) in 16 (27%) subjects, or only arthralgias without evident inflammatory changes in the joints in 19 (31%). The analysis of JI variants by groups in children with CD (n=36) showed PA in 15 (42%), SpA in 12 (33%), and isolated arthralgia in 9 (25%) patients. The prevalence of PA and arthralgia among boys and girls with CD was comparable (60 and 40% of PA, 56 and 44% of arthralgias, respectively); the prevalence of SpA in boys with CD was significantly higher: found in 11 (92%) boys. In the group of children with UC (n=19), a high prevalence of PA and isolated arthralgia was reported: 8 (42%) and 9 (47%) patients, respectively. SpA was diagnosed in 2 (11%) children with UC. Of the 60 children with JI and IBD, 30 (50%) developed the disease with joint involvement. CD onset was associated with JI significantly more often than UC onset: of 30 children with JI, 21 (70%) were first diagnosed with rheumatic joint disease, and then CD was detected during follow-up. The disease first manifested with joint involvement in 6 (20%) patients with UC and 3 (10%) with UndC. The time interval from the onset of JI to the onset of the intestinal syndrome (and/or IBD diagnosis) varied widely from 1 month to several years; in 1 patient, it was more than 8 years. In 8 (27%) patients, this time interval was 1 to 3 months; in 6 (20%) patients, the symptoms of the underlying disease appeared within 12 to 24 months from the joint involvement manifestation. The atypical onset and course of the disease make diagnosis challenging and require increased doctor attention to patient management. As an illustration, the article presents a clinical case of an atypical onset and course of CD associated with juvenile SpA.

Introduction. A range of diseases that require differential diagnostic search in persistent joint syndrome in children include juvenile idiopathic arthritis (JIA) and joint lesions in inflammatory bowel diseases (IBD). Aim . To analyze and compare the nature of joint syndrome in children with JIA and Crohn’s disease (CD) with joint lesions. Materials and methods . The joint syndrome was analyzed in a group that included 23 children with JIA and 24 children with Crohn’s disease with joint lesions. Statistical data were processed using parametric tests and exact formulas for percentage-based relationships. Results . JIA typically affected the knee, ankle, hip, and wrist joints (21 (91.3%), 14 (60.9%), 15 (65.2%), and 5 (21.7%) children, respectively). Enthesopathies were detected in 4 (17.4%) children. CD caused spondyloarthritis in 5 children (10.8%) and peripheral joint lesions in 15 patients (58.3%). Knees, hips, and small joints of the hands and feet were affected most frequently (14 (58.3%), 13 (54.2%), 9 (37.5%), and 4 (16.7%) children, respectively). Enthesopathies were detected in 10 children (41.7%). An analysis of the comparative frequency of joint lesions in JIA and CD showed that the knee, ankle, and wrist joints were significantly more often involved in JIA, while enthesitis were significantly more often detected in CD (p < 0.05). 5 patients (10.8%) had CD-associated spondyloarthritis. Antinuclear antibodies were identified significantly more often in patients with JIA (p < 0.05). The article is also illustrated with two clinical case reports. Conclusions . The data presented demonstrate differences in the nature of joint syndrome in children with JIA and CD. A detailed history taking, physical examination, and assessment of the nature of the joint syndrome make it possible to establish a correct diagnosis and select a rational approach to the management of patients.

Background. Inflammatory bowel diseases, which include Crohn's disease and ulcerative colitis, are immune-mediated inflammatory diseases with a complex pathogenesis. In addition to the involvement of the intestinal wall in the pathological process, systemic extraintestinal manifestations are observed in Crohn's disease and ulcerative colitis, and there is also often an association with psoriasis and psoriatic arthritis. Objective. Purpose of the study was to analyze the nature of the articular syndrome in children with inflammatory bowel diseases associated with juvenile psoriatic arthritis. Materials and methods. A retrospective analysis of the nature of the articular syndrome was carried out in a group of children with inflammatory bowel diseases associated with juvenile psoriatic arthritis (20 patients, mean age 12.7 years (95% CI [11.01; 14.29]). The diagnosis of juvenile psoriatic arthritis was established according to the Vancouver diagnostic criteria (1989). The diagnosis of Crohn's disease was established in accordance with Clinical recommendations for Crohn's disease for pediatric patients, 2025. The diagnosis of ulcerative colitis was established in accordance with Clinical guidelines for ulcerative colitis for pediatric patients, 2025. Statistical processing was performed using parametric methods and exact formulas for percentages. Results. 17/20 (85%) of our patients were diagnosed with Crohn's disease, inflammatory form, 3/20 patients (15%) – ulcerative colitis. The diagnosis of definite juvenile psoriatic arthritis, according to the Vancouver diagnostic criteria, was made in 11/20 patients (55%), probable – in 9/20 (45%). Boys predominated in the group – 14/20 (70%). The mean disease duration was 5.0 years (95% CI [3.21, 6.79] years). 13/20 (65%) patients had relatives 1 and 2 degrees of relatives suffered from psoriasis. All children with definite psoriatic arthritis (11/20 children) had plaque (vulgar) psoriasis. In 15/20 (75%) patients, the onset of articular syndrome was asymmetrical oligoarthritis. At the height of the disease, the oligoarticular variant was observed in 10/20 (50%) children, rheumatoid-like (polyarticular) in 3/20 (15%), spondyloarthritis with damage to peripheral joints in 7/20 (35%). The most commonly affected joints were the knee (15/20 (75%)), ankle (5/20 (25%)), and hip (8/20 (40%)) joints. Conclusions. The articular syndrome in inflammatory bowel diseases associated with juvenile psoriatic arthritis is characterized by a frequent onset of the disease with joint damage (in 40%), asymmetric oligoarthritis at the onset and at the height of the disease, while isolated juvenile psoriatic arthritis is characterized by a pattern of development of the articular syndrome from oligo- to polyarthritis. The association of inflammatory bowel diseases and juvenile psoriatic arthritis is characterized by a high percentage of spondyloarthritis with damage to peripheral joints (35%). The most common joints involved are the knee and hip joints (75% and 40%), with a high incidence of enthesitis (55%), while in isolated juvenile psoriatic arthritis the knee and ankle joints are most often affected. The course of inflammatory bowel diseases against the background of psoriatic arthritis requires an individual approach to patient management and selection of therapy aimed at the common links in the pathogenesis of these conditions.

A retrospective study contains clinical and immunological characteristics of juvenile systemic scleroderma manifestations in 78 children according to data from a single rheumatology center. The average age of onset was 8.3 years, girls outnumbered boys, the form with diffuse skin involvement was more common, and among visceral involvement, the gastrointestinal tract and lungs were the most commonly affected; antinuclear factor was detected in 81% of children, anticentromere antibodies in 31%, and antitopoisomerase antibodies in 8.5%. The 5-year survival rate of patients in the main group was 100%, and the probability of survival at 74 months was 70%. Our data largely coincide with the studies of other authors. No obvious natural pathomorphosis of juvenile systemic scleroderma has been established in the 2000s compared to 1960–1980; therapeutic pathomorphosis of the disease is likely to occur. Key words: pathomorphosis, age of onset, visceral involvement, autoantibodies, survival

The prognosis of Takayasu arteritis (TA) depends on timely and adequate therapy, but in about half of patients, the disease is refractory to standard therapy or recurs against the background of a decrease in the dose of glucocorticoids (GCs). The use of biological disease-modifying antirheumatic drugs (bDMARDs) in the treatment of TA looks promising, however, the experience of their use in TA in children is presented by isolated observations. Objective of the study: to evaluate the efficacy and safety of tocilizumab (TCZ) in the treatment of refractory and recurrent forms of TA in children. Materials and methods of research: the study retrospectively included 9 children who were prescribed TCZ when standard therapy was ineffective. Before starting TCZ therapy, all patients were diagnosed with an active form of the disease. The median duration of TCZ therapy was 24 months. Results: against the background of TCZ therapy, the median ESR decreased from 22 to 5 mm/h (p value <0,01), the level of C-reactive protein from 6 to 0 mg/l (p value <0,025). All patients achieved remission. No relapses were observed. The median GCs dose decreased from 0,377 to 0,15 mg/kg/day for prednisolone, 2 patients with GCs were completely canceled. The ITAS.A activity index decreased from 3–12 (median 7) to 0 in 7 and to 1 in 2 more patients. The drug was well tolerated. Adverse reactions included one case of pityriasis versicolor and one case of postoperative phlegmon of the neck and subclavian region on the right. Conclusion: TCZ has shown efficacy and safety for the induction and maintenance of remission in children with TA. The presented results of the study indicate the prospects for further study of the use of TCZ for TA in pediatric practice.

BACKGROUND: In coexistence of diabetes mellitus type 1 (DM1) with severe autoimmune and inflammatory diseases some patients need simultaneous administration of insulin and glucocorticoids (GC). GC therapy in patients with DM1 can worsen glycemic control. AIM: To determine characteristics of insulin therapy of DM1 in children and adolescents receiving GC. DESCRIPTION OF CLINICAL CASES: We observed 5 patients with DM1 receiving GC for juvenile idiopathic arthritis (JIA), juvenile systemic sclerosis (JSS), juvenile dermatomyositis (JDM), ulcerative colitis (UC), and reactive arthritis (RA). Intra-articular administration of GC did not significantly influence glycemic control. In case of GC pulse therapy hyperglycemia and increased insulin requirements were recognized in 36 hours after GC receipt, persisted from few hours up to 3 days after each administration. While therapy with oral GC in high doses the worst glycemic control was registered in daylight hours. To overcome insulin resistance change of time of injection and 10%-increase of long-acting insulin analogue, additional injections of ultrashort-acting insulin analogues, temporal prescription of short-acting human insulin were used. While GC therapy insulin daily dose was individual and could reach 2.0 U/kg. After transition to maintaining doses of GC or discontinuation of GC therapy patients returned to standard or relatively low insulin requirements. Levels of glycosylated hemoglobin differed significantly among patients at different stages of treatment, were maximal while long-term therapy with high doses of oral GC, but mostly depended on patients compliance. CONCLUSION: Bettering of glycemic control while receiving GC can be reached by timely dose correction of insulin therapy, selection of individual schemes, taking into account time of receipt and pharmacokinetic characteristics of GC. Adherence of the patient and his family to treatment of DM1 plays an important role in glycemic control.