Our study aimed to evaluate the clinical and laboratory features of juvenile idiopathic arthritis (JIA) children with temporomandibular joint (TMJ) arthritis. In the retrospective cohort study, we analyzed data of 753 patients with JIA aged 2–17 years, depending on TMJ arthritis or not. TMJ arthritis can to be diagnosed in the presence of at least two of the following clinical signs of inflammation: pain in TMJ, jaw opening limitation, jaw opening deviation, and micrognathia. We compared clinical, laboratory, and treatment features in JIA patients depending on the involvement of TMJ. TMJ arthritis was detected in 43 (5.7%) of our patients and associated with a longer course of the disease, polyarticular JIA category, treatment with systemic corticosteroids, and longer achievement of the remission and involvement of cervical spine, hip, and shoulder. Active joints >8 (OR = 14.9, p = 0.0000001), delayed remission >7 years (OR = 3.1; p = 0.0004), delayed hip involvement (OR = 4.6; p = 0.041), hip osteoarthritis (OR = 4.0; p = 0.014), cervical spine arthritis (OR = 10.3, p = 0.000001), and corticosteroid treatment (OR = 2.3, p = 0.0007) were associated with TMJ involvement. Patients with TMJ arthritis require more biologics (OR = 3.2, p = 0.0006, HR = 2.4, p = 0.005) and have decreased probability of remission achievement (p = 0.014). Consequently, TMJ arthritis was associated with a severe disease course. Early biologic treatment and corticosteroid avoidance might decrease TMJ involvement.

Background: Pediatric lupus nephritis (LN) is one of the most serious manifestations of systemic lupus erythematosus (SLE) in children, determining the outcomes of the disease. There are no standardized treatment protocols for pediatric LN, and the role of biologics has not yet been conclusively defined. Objectives: analyze the safety and efficacy of rituximab biosimilar BCD020 in pediatric patients with lupus nephritis. Methods: in a retrospective cohort study, the data from the case histories of 25 patients with LN (10 boys and 15 girls) with an onset age of 13 (9–16) years, who failed conventional non-biologic treatment or developed corticosteroid dependence/toxicity, were included. The diagnosis was made using Systemic Lupus International Collaborating Clinics (SLICC) classification criteria. Rituximab biosimilar BCD020 was prescribed in a dosage of 375 mg/m² every week (2–4 infusions) with repeated courses every 6–12 months (2–4 infusions) according to disease activity, B-cell depletion, and IgG levels. The dynamics of clinical and laboratory data, the activity of the disease by SLEDAI, and corticosteroid doses were assessed at the onset and during the rituximab trial. Results: The main patient’s characteristics were: Pre-rituximab non-biologic conventional treatment included: cyclophosphamide 15 (60%), MMF 8 (32%), azathioprine 3 (12%), hydroxychloroquine 12 (48%), and pulse therapy of methylprednisolone followed by oral methylprednisolone 25 (100%). The time before rituximab was 7.0 (3.0–24.0) months, and the whole observation period was 7.0 (0; 24) months. The initial pre-rituximab treatment slightly reduced SLEDAI levels and the proportion of patients with LN. A significant reduction of SLEDAI, the anti-dsDNA level, proteinuria, hematuria, C4 complement, ESR, and the median corticosteroid dose by 80% from the initial value, as well as the proportion of patients without corticosteroids, was observed after rituximab administration. Two deaths were observed due to catastrophic SLE with macrophage activation syndrome, accompanied by a severe infection (invasive aspergillosis, n = 2). Three patients developed serious adverse events: pneumonia (n = 2), transient agranulocytosis (n = 1) after the third rituximab infusion, and meningitis, caused by Listeria monocytosis, after the first rituximab infusion. Eight patients received antibacterial treatment for different respiratory infections without hospital admissions. Conclusions: Rituximab biosimilar BCD020 showed effectiveness in LN, whereas previous non-biologic treatment was insufficiently effective. Randomized controlled trials are required to evaluate the efficacy and safety of rituximab and evaluate the benefits when compared with conventional SLE treatment.

Background: Pulmonary involvement in systemic juvenile idiopathic arthritis (SJIA) is a rare but dangerous complication. The main risk factors are already known, such as macrophage activation syndrome, a refractory course of systemic juvenile arthritis, infusion reaction to interleukin 1 and/or interleukin 6 blockers, trisomy 21, and eosinophilia. However, information about respiratory system involvement (RSI) at the onset of SJIA is scarce. Our study aimed to evaluate the specific features of children with SJIA with RSI and their outcomes. Methods: In a single-center retrospective cohort study, we compared the information from the medical records of 200 children with SJIA according to ILAR criteria or SJIA-like disease (probable/possible SJIA) with and without signs of RSI (dyspnea, shortness of breath, pleurisy, acute respiratory distress syndrome, and interstitial lung disease (ILD)) at the disease onset and evaluated their outcomes (remission, development of chronic ILD, clubbing, and pulmonary arterial hypertension). Results: A quarter (25%) of the SJIA patients had signs of the RSI at onset and they more often had rash; hepato- and splenomegaly; heart (pericarditis, myocarditis), central nervous system, and kidney involvement; hemorrhagic syndrome; macrophage activation syndrome (MAS, 44.4% vs. 9.0%, p = 0.0000001); and, rarely, arthritis with fewer active joints, compared to patients without RSI. Five patients (10% from the group having RSI at the onset of SJIA and 2.5% from the whole SJIA cohort) developed fibrosing ILD. All of them had a severe relapsed/chronic course of MAS; 80% of them had a tocilizumab infusion reaction and further switched to canakinumab. Unfortunately, one patient with Down’s syndrome had gone. Conclusion: Patients with any signs of RSI at the onset of the SJIA are required to be closely monitored due to the high risk of the following fibrosing ILD development. They required prompt control of MAS, monitoring eosinophilia, and routine checks of night oxygen saturation for the prevention/early detection of chronic ILD.

Background/Objectives: Cervical spine arthritis (CSA) in children with juvenile idiopathic arthritis (JIA) can lead to clinically significant and irreversible functional impairment. Our study aimed to evaluate the features of the JIA disease course in children with CSA. Methods: In the retrospective cohort study, the data from medical charts of children with JIA (n = 753) who corresponded to the ILAR criteria and were treated from 2007 to 2016 were included. CSA was diagnosed by clinical manifestations (pain and limited range of motion) with radiological confirmation in the available cases. Results: CSA had 101 JIA patients (13.4%), predominantly with polyarticular (48%, OR = 1.8 (1.2; 2.7), p < 0.001) and systemic (18.9%, OR = 3.6 [2.0; 6.6], p < 0.001) JIA categories. CSA was associated with longer disease duration, higher inflammatory activity, a higher number of active joints, a lower probability of achieving remission (HR = 1.33 (95% CI: 1.01; 1.76, p = 0.04)), and a higher probability of being treated with biologics (HR = 1.78 (95% CI: 1.22; 2.59, p = 0.002)). Patients with temporomandibular arthritis (OR = 10.4 [5.4; 19.8], p < 0.001) and shoulder arthritis (OR = 14.1 [7.5; 26.3], p < 0.001) had the highest risk of having CSA. Conclusions: CSA was an independent predictor of treatment with biologics and failure to achieve remission. Identified predictors can help to find the group of patients with higher suspicion for whom the functional tests and MRI are required to not miss the CSA. A radiology assessment of CSA should be performed as far as possible in children, unless there are risks of general anesthesia for younger patients.

Kawasaki disease is an acute febrile illness of childhood, one of its characteristic features is lymphadenopathy. Most often it manifests unilateral painful more than 1.5 sm in diameter enlargement of single or several cervical lymph nodes. In some cases increase in size can be observed in other lymph nodes groups: axillary, inguinal, paratracheal, para-aortic, retroperitoneal, mesenteric. In that case the dimension is smaller than 1.5 sm in diameter. Usually it occurs at the same time as fever and fades away after inflammation is terminated. Most typical morphological features are non-purulent necrotic changes with subcapsular necrotic lesions and non-specific changes: presence of enlargement of paracortical zone and expansion of sinus. The article presents a clinical case of Kawasaki disease diagnosis accompanied by severe lymphadenopathy, persistence of fever and progression of lymphadenopathy, appearance of supraclavicular lymph node after the introduction of intravenous immunoglobulin. This reflects the non-smooth course of the disease and required differential diagnosis, primarily with lymphoproliferative disease. A review of “unusual” cases of lymphadenopathy in patients with Kawasaki disease described in the literature is presented. The importance of carrying out a histological examination of the lymph node in a nonsmooth course of the disease is underlined. Histological variants of lymph node involvement in patients with Kawasaki disease are described.