Background: Takayasu’s arteritis (TA) is a systemic vasculitis that primarily affects the aorta and major arteries. Despite aggressive treatment with glucocorticoids (GCs) and non-biological disease-modifying antirheumatic drugs (nbDMARDs), about 30% of patients experience resistance to therapy or relapse. This study aimed to identify risk factors associated with refractory and relapse TA in pediatric patients. Methods: A retrospective, open-label, case–control study was conducted with 56 pediatric patients with TA diagnosed between February 2011 and October 2022. Fourteen patients were excluded due to insufficient data in their medical records, leaving 42 for further analysis. The patients were divided into two groups: Group 1 (18 patients) with no evidence of relapse and Group 2 (24 patients) with relapse despite first-line treatment at the end of the follow-up period. Clinical, laboratory, and instrumental data were collected and analyzed using R v4.2 and Python v3.10. Results: The median time to relapse was 18 [IQR: 13; -] months according to the Kaplan–Meier curve. Patients with ITAS.A with a diagnosis of TA ≥ 12 had a higher probability of relapse, according to the log-rank criterion (p = 0.006). Symptoms of critical ischemia, such as limb claudication, were more common in Group 2 at diagnosis (p = 0.047), and a trend toward a longer diagnostic delay was observed (p = 0.067). Conclusions: Pediatric patients with an initial ITAS.A score above 12 have a higher risk of relapse when treated with a combination of GCs and nbDMARDs as first-line treatment. Further research is needed to identify high-risk patients more accurately and optimize therapeutic strategies.

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

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.

The purposes of the study were as follows: to perform a comparative analysis of demographic and clinical data in children with Takayasu's arteritis (TA) who underwent surgical treatment of the underlying disease, to determine approaches to surgical interventions and to evaluate its outcomes. Materials and methods of the research: 51 children (boys:girls ratio 1:4.7) aged from 8 months till 17 years old (11 [9; 14] years as median) with a reliable TA diagnosis were observed from February, 2001 till January, 2022. The median age of the TA debut was 11 [9; 14] years. The single-center (I.M. Sechenov University Children's Clinical Hospital with the N.F. Filatov Clinical Institute of Children's Health of the I.M. Sechenov First Moscow State Medical University located in Moscow, Russia) case-control study included 40 patients: Group 1 (n=10) consisted of patients who underwent the surgical intervention, and Group 2 (n=30) consisted of children who have not been indicated for surgical treatment at the end of the follow-up. Patients did not differ statistically significantly in terms of gender (p=1.0), age (p=0.729), disease duration (p=0.542) and duration of follow-up at the hospital (p=0.708). The data that the authors have evaluated included gender, age, duration of the disease, including before the diagnosis was verified, the clinical picture at the onset and the type of TA, data from laboratory and instrumental studies before diagnosis and during follow-up, treatment options, assessment of the TA activity according to the ITAS-A score. Results: the median duration of TA before diagnosis was statistically significantly higher in the Group 1 (p=0.013) and amounted to 23.5 [13; 36] months (9.5 [2; 20] months in the Group 2). The median systolic blood pressure (SBP) and diastolic blood pressure (DBP) at the onset on the TA diagnosis in patients from the Group 1 (165.0 and 110.0 mm Hg, respectively) were statistically significantly higher (p<0.001) than in the Group 2 (116.5 and 65.0 mm Hg, respectively). The arterial hypertension (p<0.001) and headaches (p=0.006) were statically significantly more often in patients from the Group 1, while in patients from the Group 2 the general inflammatory reactions were more typical, such as episodes of fever (80%, p=0.003) and weight loss (33.3%). The median amount of vessels involved in the pathological process at the onset of TA in the Group 1 patients (6.5; [4; 8]) was also statistically significantly higher (p=0.043) than in the Group 2 patients (4; [3 ; 6]). In patients of the 1st Group, the abdominal aorta (90%, p=0.006), descending thoracic aorta (70%, p=0.007) and renal arteries (70%, p<0.001) were affected statistically significantly more often. The Group 1 patients had 14 surgical interventions in total. The median SBP decreased after the 1st surgical intervention from 170 [160; 172] to 130 [105; 145] mm Hg, and the median DBP dropped from 110 [100; 114] to 70 [65; 85] mm Hg. The early postoperative period proceeded favorably after 13 out of total of 14 surgical interventions: in a single patient the neck phlegmon was developed on the same side a week after subclavian-carotid prosthesis on the right side. During the observation period in the hospital there were no deaths recorded in the Group 2. In a single female patient from Group 1 the aneurysm of the anastomosis of the infrarenal aorta ruptured on the 42nd month after the surgical intervention, which has led to her death. Conclusion: the duration of TA before the start of therapy, the extensive damage to the arterial bed, including involvement of the abdominal aorta and renal arteries, the absence or mild severity of the general inflammatory syndrome and arterial hypertension at the onset of the disease increase the likelihood of surgical treatment. The surgical intervention for TA should be carried out against the background of immunosuppressive therapy, according to the strict indications and considering the risk/benefit ratio for each patient.

Objective. The aim of the study: analysis of the structure of neurological disorders in children with linear scleroderma of the head. Methods. Clinical and neurological observation, MRI, EEG, examination of genetic thrombophilia markers. Results. A total of 60 patients with linear scleroderma of the face and head (LSF) were examined: 23 boys (38.33%) and 37 girls (61.67%) aged between 3 and 16 years. The average age of disease onset was 6.64 ± 0.53 years. Neurological disorders were found in half of the patients (n = 32; 51.67%). Among them, disease onset in 26 children (81.3%) was characterized by typical skin changes, while in 8 (25%) it was characterized by neurological signs. Neurological disorders included headaches (in 43.8% of children), epileptic seizures (in 25%), cranial nerve involvement, acute cerebrovascular disorders (9.4%), and tics (9.4%). MRI of the brain revealed changes in 27 out of 32 children with neurological disorders (84%): white matter lesions (63%) and less commonly gliotic changes in the periventricular area (31%). EEG monitoring detected abnormalities in 21 children (65%), but a typical pattern of epileptiform activity was only found in 14 children (24%), including 6 children (10% of cases) with epileptiform activity without clinical seizures. Among the three children with acute cerebrovascular disorders, all were diagnosed with genetic thrombophilia, two had cerebral vascular malformations, and one had antiphospholipid syndrome. Conclusion. The pathogenesis of strokes in children with LSF involves sclerodermic vasculopathy, cerebral vascular anomalies, and mutations in blood coagulation genes leading to hypercoagulability.