Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unclear etiology that belongs to interstitial lung diseases and is characterized by a triad of symptoms: hemoptysis, iron deficiency anemia, and diffuse interstitial changes on computed tomography (CT) of the chest cavity. IPH can occur at any age, but the disease develops in childhood in 80% of cases, more often before 10 years of age. A significant proportion of pulmonary hemosiderosis cases in this age group remain undiagnosed. The purpose of the study is to demonstrate a clinical case illustrating the features of differential diagnosis and therapy of IPH during the COVID-19 pandemic. Conclusion. An 8-year-old girl was diagnosed with IPH based on fever, shortness of breath, weakness, and severe iron-deficient anemia. The chest CT scan revealed diffuse interstitial ground-glass opacity in lungs. The lung biopsy revealed hemosiderin deposits and hemosiderophages in the alveolar lumens. Treatment with systemic glucocorticoids (SGC) and azathioprine was effective. The patient was followed for 3 years.

Purpuse. Evaluation of the proteomic tears profile in children with uveitis associated with juvenile idiopathic arthritis in relation to the search for pathognomonic markers of this pathology. Material and methods. The study included 29 children aged 2 to 17 years. 17 patients had active uveitis associated with juvenile idiopathic arthritis, 2 – juvenile idiopathic arthritis without uveitis, 3 – systemic vasculitis, 4 – idiopathic uveitis, 3 – healthy children. The evaluation of the qualitative and quantitative composition of proteins in tears in children from the presented groups was carried out. Results. The study showed the possibility of early diagnosis of uveitis, associated with juvenile idiopathic arthritis, using the proteomomic profile of tears. Lactotransferin is one of the key proteins that allows it’s identification. It can be considered the most likely biomarker thanks to which it is possible to recognize the etiology of uveitis even before the appearance of clinical symptoms of joint damage. This will allow us to start adequate therapy, which can save young patients from deep disability in the future. Key words: early diagnosis of uveitis, biomarkers of tears, proteomic profile, juvenile idiopathic arthritis

Systemic lupus erythematosus (SLE) is an immunopathological disease characterized by various systemic malfunction and a prognosis for unfavorable outcomes. In the current standards of treatment for SLE the biological therapy is not included in the first line of therapy. The data from previous studies do not allow drawing a clear conclusion about the benefits of using biological therapy. The purpose of this research was to study the outcomes of SLE therapy using the rituximab biosimilar BCD020 compared with the standard non-biological therapy. Materials and methods used: a single-center cohort retrospective study of 79 medical records of children aged 0 to 18 y/o suffering from SLE were analyzed: 19 received rituximab as part of multicomponent therapy in the first 6 months from the onset of the disease, the remaining 60 received standard non-biological therapy. Damage to organs and systems, laboratory data, doses of corticosteroid therapy and disease activity were assessed using the SELENA-SLEDAI tool at the time of initiation of therapy and one year after. Results: higher degree of disease activity involving the central nervous system and kidneys was noted in the group of children receiving rituximab therapy. No statistically significant difference between the data of the two groups, including disease activity, was noted in 12 months after the start of the therapy. There was a statistically significantly more pronounced decrease in the SLEDAI activity index after a year in the group of children who received rituximab (p=0.001) compared to children who did not receive it. During rituximab therapy, infectious complications were noted in 3 cases, including the development of meningitis; and a single patient had developed with bicytopenia. Conclusion: rituximab was used in children with higher disease activity, with involvement of organs and systems whose damage leads to an unfavorable prognosis (CNS and renal damage). Rituximab can be considered as an option in the treatment of severe variants of SLE with a poor prognosis due to its ability to more intensively arrest disease activity compared to standard non-biological therapy.

Systemic lupus erythematosus is an immunopathological disease which is characterized by a poor prognosis. Biologics applied in the disease treatment allow reducing the corticosteroid toxicity and controlling the disease.Purpose. To evaluate the efficacy and safety of rituximab therapy in children with systemic lupus erythematosus.Material and methods. The retrospective study included data of 48 patients with systemic lupus erythematosus treated with rituximab. Systemic lupus erythematosus was diagnosed based on the SLICC classification criteria. Patients were assessed at baseline disease status, at the time of rituximab initiation and follow-up. The indications for the rituximab were: lupus nephritis, CNS involvement, and hematological involvement resistant to the standard therapy, and in cases of severe corticosteroid toxicity.Results. During rituximab therapy the significant decrease of the SELENA–SLEDAI activity index was observed. There was a significant decrease of the level of antibodies against dsDNA, normalization of the levels of hemoglobin, ESR, complement C4. The proportion of patients with cytopenia decreased up to their complete absence in patients receiving therapy for three years. The number of patients with active lupus nephritis decreased from 16 at the time of rituximab initiation to 1 after 3 years of therapy. Significant dynamics of proteinuria and hematuria was noted, except for 1 patient. The daily dose of corticosteroids was reduced by 90% from baseline in patients treated for 3 years. Serious adverse events included three deaths in patients with high systemic lupus erythematosus activity with uncontrolled macrophage activation syndrome associated with infections. Various infectious complications, hypogammaglobulinemia, which required replacement therapy with intravenous immunoglobulin, were also recorded.Conclusion. Rituximab can be considered as an option for the treatment of severe forms of systemic lupus erythematosus which are resistant to standard therapy. Further studies are required to evaluate efficacy and safety.

Systemic corticosteroids are continued to be administered in juvenile idiopathic arthritis (JIA) patients, especially in systemic JIA (sJIA), despite the ability of biologic therapy. One of the complications of long-term CS treatment is delayed hip arthritis development with risk of secondary hip osteoarthritis formation and total hip arthroplasty (THA).We compared different types of hip joint lesions in JIA, especially, secondary hip osteoarthritis development and THA rates in systemic and non-systemic JIA, and evaluate systemic corticosteroids contribution to those complications.The study included 753 JIA patients. They were divided into 2 groups: patients with sJIA and non-systemic JIA (nsJIA). Clinical and demographic characteristics, CS treatment regimens were compared.Results. Hip arthritis was found equally often in both groups, but both secondary hip osteoarthritis (19% vs 5,3%) and THA (8.6% vs 1.6%) prevailed in the sJIA. Patients with sJIA had delayed hip involvement (57.9% vs 30.6%; p=0.019), earlier secondary hip osteoarthritis development (4.5 vs 5.1 years after the JIA onset) with younger age of secondary hip osteoarthritis achievement (13.7 vs 15.2 years; р=0.045), they also had higher inflammatory activity, greater systemic corticosteroids administration (94.8% vs 56.1%; р=0.0000001) and higher cumulative systemic corticosteroids dose (3085 mg vs 2000 mg; p=0,005). More than half patients (56.1%) with nsJIA had systemic corticosteroids treatment and impaired calcium-phosphorus metabolism.Conclusion. Systemic corticosteroid treatment and delayed hip involvement are independent predictors of secondary hip osteoarthritis in all JIA categories. Calcium and phosphate metabolism disturbances are additional predictor for secondary hip osteoarthritis in non-systemic JIA categories

Osteoarthritis (OsA) of the hip joint (HJ) is one of the most serious structural complications of juvenile idiopathic arthritis (JIA). Objective. Assess risk factors for OsA of the HJ in patients with JIA. Patients and methods. This retrospective study included 753 JIA patients aged 2 to 17 years. The diagnosis of JIA was based on the ILAR criteria. The diagnosis of HJ OsA (n = 48) was based on the Dale radiological classification; the indications for joint replacement were formed according to the WOMAC scale and Oxford hip score. Results. The development of HJ OsA was registered within 5 years (range 2,4; 9,4 years) after JIA onset in 6.4% of patients with JIA (n = 48) or 31.4% of patients who had hip joint lesions. The frequency of HJ replacement (n = 16) was 2.1%, 10.5%, 33.3% in all children with JIA, among patients with HJ arthritis, and patients with HJ osteoarthritis, respectively. The main predictors of HJ OsA included systemic arthritis, administration of systemic corticosteroids, >4 active joints, total alkaline phosphatase <165 U/L, long time without remission, involvement of joints of the upper half of the body, and no timely initiation of biologicals. The predictors of HJ replacement were systemic arthritis, pulse therapy with methylprednisolone, and late administration of biologicals. Conclusion. Timely control of JIA activity, achievement of remission, avoidance of glucocorticosteroid therapy or its early replacement with biologicals reduced the risks of developing structural lesions of the HJ in patients with JIA. Key words: osteoarthritis, hip joint, ischemic osteonecrosis, juvenile idiopathic arthritis, risk factors, corticosteroids

Introduction. In children with rheumatic diseases, severe fungal infections (invasive mycoses – IM) are not well understood.Objectives. To analyze risk factors, disease course of IM in children with systemic rheumatic diseases.Materials and methods. For diagnosis of IM were used criteria EORTC/MSGERC, 2019. We reviewed the literature over the past 15 years on IM in children with rheumatic diseases from the international databases Pubmed and Web of Science.Results. In retrospective multicenter study were included 8 children with IM and systemic rheumatic diseases: ANCA-associated vasculitis (n=4), systemic lupus erythematosus (n=3), juvenile rheumatoid arthritis (n=1). Median age was 13,5 (8-17) y., boys – 67%. Invasive aspergillosis was diagnosed in 5 patients and invasive candidiasis – 3. The risk factors of invasive mycoses were high rheumatic disease activity (100%), corticosteroids (prednisolone ≥ 0,3 mg/kg/d) use for ≥21 d (87,5%), immunosuppressive therapy (87,5%), recent (≤ 2 weeks) pulse steroid therapy (75%), hemophagocytic lymphohistiocytosis (62,5%), prolonged (≥ 10 days) severe neutropenia (≤ 0,5х109/l) (62,5%), and prolonged (≥10 days lymphopenia (≤ 1,0х109/l) (37,5%). In patients with invasive aspergillosis the involved organ was the lung, in patients with invasive candidiasis a candidemia was diagnoses. All patients received antifungal therapy. The overall 30 days survival rate was 37,5%.Сonclusions. Children with high rheumatic diseases activity and intensive treatment with immunosuppressive agents should be considered as patients with a high risk of invasive mycoses with a high mortality.

Mevalonate kinase deficiency (MKD) is a rare autoinflammatory disease with an autosomal recessive inheritance. The severity of the disease is correlated with the residual enzyme activity. There are two types of mevalonate kinase deficiency: a less severe type called hyperimmunoglobulinemia D syndrome (HIDS) and a more severe type called mevalonic aciduria. A little more than 300 cases with MDK have been described worldwide. The rarity of this disease, and polymorphism of clinical manifestations lead to late diagnosis and late onset of specific treatment. We described the 7-month-old infant with pathogenic variants c.118C> T (p.R40W) and c.206_207del (p.S69fs) in the MVK gene in a compound heterozygous state with the successful use of an interleukin-1 blocker.

TNF-α inhibitors are used in the treatment of non-systemic variants of juvenile idiopathic arthritis (JIA) in case of ineffectiveness or intolerance to methotrexate. Despite 20 years of experience of using of etanercept in pediatric rheumatology, a long-term study of the efficacy and safety of the drug in real clinical practice remains necessary. Objective of the study: to study the efficacy and safety of etanercept for treatment of various non-systemic JIA subtypes in real clinical practice. Materials and methods of research: data from the case histories of 375 patients (242 girls and 133 boys) with articular forms of JIA for 2010–2020 were included in a retrospective, open, uncontrolled, nonrandomized, continuous multicenter cohort study. The age of debut was 6.3 (2.9; 10.7) years. Demographic characteristics, subtypes of arthritis, indicators of laboratory activity of inflammation, and outcomes were estimated: achievement of remission, exacerbation, and switching from etanercept to another genetically engineered biological drug (GEBD) were assessed. Results: remission was recorded in 78.9% on average after 6 months. The factors that determine the likelihood of achieving remission were the absence of previous therapy with GEBD (p=0.001) and compliance with therapy – OR=2.5 (95% CI: 1.3; 4.7), p=0.006. Exacerbations were recorded in 29.5% and were associated with the presence of the HLA B27 antigen – OR=2.6 (95% CI: 1.1; 6.0), p=0.028, antinuclear factor seropositivity (p=0.060). Change of etanercept to another GEBD was made in 17.4% of children and was associated with a failure to achieve remission – OR=7.7 (95% CI: 4.0–14.3), p=0.000001, with previous exacerbations – OR=14,8 (5.3; 41.2), p=0.0000001 and the development of de novo uveitis – OR=2.4 (95% CI: 1.1–5.3), p=0.038. The arthritis subtype and the presence of concomitant methotrexate therapy did not significantly affect treatment outcomes. Conclusion: achievement of remission, compliance with therapy, history of previous therapy with GEBD, exacerbation of JIA and development of de novo uveitis determined the main outcomes of etanercept therapy. The JIA subtype, as well as concomitant therapy with methotrexate, did not significantly affect the outcomes of the disease, which makes it possible to consider etanercept therapy a very effective and safe method of treating JIA as a genetically engineered first-line therapy of any variants of articular forms of JIA, even in monotherapy with ineffectiveness or intolerance to methotrexate.

Background. Etanercept is a biological drug most commonly used in patients with juvenile idiopathic arthritis (JIA). The results of its use are showed in local studies. Objective. Our aim was to evaluate the efficacy and safety of the use of etanercept in children with non-systemic JIA, to determine the predictors of remission and the risk factors for the development of exacerbations. Methods. In a retrospective cohort study, the results of etanercept treatment (remission, exacerbations, adverse events) in children with non-systemic JIA were analyzed. The minimum follow-up period was 6 months. Results. The period of remission within 6–36 months occurred in 77/131 (58.8%), exacerbations developed in 18/129 (14.0%) patients. Predictors of achieving remission were the age of JIA onset < 8 years [relative risk (RR) 2.05; 95% confidence interval (CI) 1.27–3.23], the age of prescribing etanercept ≤ 10 years (RR 1.7, 95% CI 1.22–2.38), the time of the disease prior to etanercept prescription < 2.5 years (RR 2.4, 95% CI 1.4–4.4), the presence of HLA-B27 antigen (RR 2.15, 95% CI 0.98–4.75; p = 0.06). The risk of exacerbations was higher in children with polyarticular JIA (RR 2.7, 95% CI 0.9–8.2; p = 0.08), whereas methotrexate therapy reduced the risk of exacerbations (RR 0.32, 95% CI 0.1–1.15; p = 0.05). Etanercept was discontinued due to primary (improvement by the ACRpedi criteria after 3 months of therapy <30%) or secondary (loss of previously achieved ≥ 30% improvement) failure in 14/152 (9.2%) patients; de novo uveitis developed in 8/152 (5.3%) patients; reactions at the injection site — in 6/152 (4.0%) patients. Conclusion. Therapy involving etanercept is more likely to induce remission in younger patients with JIA onset at the age of 8 years and a history of less than 2.5 years. A high risk of exacerbations was noted in patients with polyarticular JIA, and low one — in those receiving methotrexate as a part of combined therapy.

OBJECTIVES:The aim of our study was to evaluate disease courses and outcomes of sJIA children undergoing tocilizumab (TCZ) treatment, and to establish the predictors which distinguish inactive disease and disease flares.METHODS:Our retrospective study included 48 active sJIA children who were refractory to different anti-rheumatic drugs and who were then started on TCZ. The effectiveness of TCZ was assessed by the changes of sJIA attributed signs and symptoms and the remission was judged according to the Wallace (2004) criteria.RESULTS:The main demographic parameters (Me; IQR) were shown; mean age: 9.9 (5-12.7) years and mean duration of TCZ administration: 27.0 (5.9-89.7) months. During the TCZ treatment 40 cases (83.3%) achieved remission in 138.5 (56.0; 255.0) days. Patients who achieved remission had milder disease course, and presented less frequent epatosplenomegaly, lung, heart involvement and MAS. They had higher Hb and lower WBC, granulocytes, ESR, CRP, LDH, ferritin. The main predictors of achievement of inactive disease, calculated with 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), Hb>113 g/l (OR=17.0, HR=1.33), LDH≤676 U/l (OR=113.6, HR=3.2), PLT>335*109/l (OR=5.0, HR=2.5), and intensive depression of WBC in 2 weeks after the 1st TCZ infusion>11% (OR=13.0, HR=6.0) and granulocytes>12% (OR=14.0, HR=4.7).CONCLUSIONS:sJIA children with milder disease course have more posssibilty of achieving disease remission during TCZ treatment. Male sex, signs of high disease activity, previous CS treatment, the long time needed to achieve inactive disease and treatment protocol deviations increased the risk of sJIA flare.

In the article the case of parvovirus B19 infection in a child, which occurred in the form of carditis and myositis, was presented for the first time. There was the rapid progression of the symptoms of acute rhabdomyolysis with a sharp increase in blood levels of creatine phosphokinase, lactate dehydrogenase, myoglobin, ferritin and troponin 1. Ultrasound examination revealed diffuse inflammatory changes in the muscles of the upper and lower extremities with multiple foci of disorganization of the muscular tissue. A high level of PVB19 DNA was determined by the quantitative PCR method, which allowed verifying the parvoviral B19 infection. Rapid clinical and laboratory response in the observed patient was the result of adequate complex therapy, including due to the early use of drugs with antiviral and immunomodulating action.

Background. Etanercept is a biological drug most commonly used in patients with juvenile idiopathic arthritis (JIA). The results of its use are showed in local studies.Objective. Our aim was to evaluate the efficacy and safety of the use of etanercept in children with non-systemic JIA, to determine the predictors of remission and the risk factors for the development of exacerbations.Methods. In a retrospective cohort study, the results of etanercept treatment (remission, exacerbations, adverse events) in children with non-systemic JIA were analyzed. The minimum follow-up period was 6 months.Results. The period of remission within 6–36 months occurred in 77/131 (58.8%), exacerbations developed in 18/129 (14.0%) patients. Predictors of achieving remission were the age of JIA onset < 8 years [relative risk (RR) 2.05; 95% confidence interval (CI) 1.27–3.23], the age of prescribing etanercept ≤ 10 years (RR 1.7, 95% CI 1.22–2.38), the time of the disease prior to etanercept prescription < 2.5 years (RR 2.4, 95% CI 1.4–4.4), the presence of HLA-B27 antigen (RR 2.15, 95% CI 0.98–4.75; p = 0.06). The risk of exacerbations was higher in children with polyarticular JIA (RR 2.7, 95% CI 0.9–8.2; p = 0.08), whereas methotrexate therapy reduced the risk of exacerbations (RR 0.32, 95% CI 0.1–1.15; p = 0.05). Etanercept was discontinued due to primary (improvement by the ACRpedi criteria after 3 months of therapy <30%) or secondary (loss of previously achieved ≥ 30% improvement) failure in 14/152 (9.2%) patients; de novo uveitis developed in 8/152 (5.3%) patients; reactions at the injection site — in 6/152 (4.0%) patients.Conclusion. Therapy involving etanercept is more likely to induce remission in younger patients with JIA onset at the age of 8 years and a history of less than 2.5 years. A high risk of exacerbations was noted in patients with polyarticular JIA, and low one — in those receiving methotrexate as a part of combined therapy.

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.

Kawasaki disease (KD) is acute systemic vasculitis of unknown etiology. Approximately 20–25% of untreated patients develop coronary artery changes with a range of severity from asymptomatic coronary artery dilatation to giant coronary artery aneurysms with thrombosis, myocardial infarction, and sudden death. To date there is no official data on the incidence of KD in Russia. In Russia, the disease is not enough known now to a wide circle of physicians and often masks other more common diseases. Since 2010, the detection rate of KD has dramatically increased in Saint-Petersburg. Objective : to analyze the experience in diagnosing and treating KD in two largest hospitals of Saint Petersburg. Patients and methods . The retrospective study included data on 30 children (18 boys, 12 girls) who were hospitalized with a diagnosis of KD in the Saint-Petersburg State Pediatric Medical University Clinic and Children’s Hospital One (Saint Petersburg) between January 2011 and September 2016. Data are represented by median and extreme values. The age of the children was 2.8 (0.2; 4.6) years; of them 5 (16.7%) patients were under the age of 1 year. The children were hospitalized on 5 (1; 14) days of disease onset; KD was diagnosed on 9 (3; 52) day of the disease. Results. Immediately after diagnosis, 27 (90%) children received aspirin. In early stages (before 10 days of the disease), intravenous immunoglobulin (IVIG) therapy was performed in 15 (50%) children, one of them received IVIG before disease day 5 (on day 3), but without effect. On disease days 11-20 (immediately after diagnosis), 10 (33.3%) children were prescribed with IVIG; thereafter fever was abolished in all the patients. Their body temperature became normal on day 11 (6; 23). Ultrasonography revealed coronary artery lesions in 13 (43.3%) patients. Out of the 30 children followed up, one baby who fell ill at the age of 3 months and received IVIG died on day 30 of the disease. Conclusion. Currently, there continues to be a delayed diagnosis of KD. IVIG therapy was effective, especially in cases of timely diagnosis. It is necessary to increase awareness of KD among clinicians and ultrasound diagnosticians.

Kawasaki disease (KD) is acute systemic vasculitis of unknown etiology. Approximately 20–25% of untreated patients develop coronary artery changes with a range of severity from asymptomatic coronary artery dilatation to giant coronary artery aneurysms with thrombosis, myocardial infarction, and sudden death. To date there is no official data on the incidence of KD in Russia. In Russia, the disease is not enough known now to a wide circle of physicians and often masks other more common diseases. Since 2010, the detection rate of KD has dramatically increased in Saint-Petersburg. Objective : to analyze the experience in diagnosing and treating KD in two largest hospitals of Saint Petersburg. Patients and methods . The retrospective study included data on 30 children (18 boys, 12 girls) who were hospitalized with a diagnosis of KD in the Saint-Petersburg State Pediatric Medical University Clinic and Children’s Hospital One (Saint Petersburg) between January 2011 and September 2016. Data are represented by median and extreme values. The age of the children was 2.8 (0.2; 4.6) years; of them 5 (16.7%) patients were under the age of 1 year. The children were hospitalized on 5 (1; 14) days of disease onset; KD was diagnosed on 9 (3; 52) day of the disease. Results. Immediately after diagnosis, 27 (90%) children received aspirin. In early stages (before 10 days of the disease), intravenous immunoglobulin (IVIG) therapy was performed in 15 (50%) children, one of them received IVIG before disease day 5 (on day 3), but without effect. On disease days 11-20 (immediately after diagnosis), 10 (33.3%) children were prescribed with IVIG; thereafter fever was abolished in all the patients. Their body temperature became normal on day 11 (6; 23). Ultrasonography revealed coronary artery lesions in 13 (43.3%) patients. Out of the 30 children followed up, one baby who fell ill at the age of 3 months and received IVIG died on day 30 of the disease. Conclusion. Currently, there continues to be a delayed diagnosis of KD. IVIG therapy was effective, especially in cases of timely diagnosis. It is necessary to increase awareness of KD among clinicians and ultrasound diagnosticians.

Autoinflammatory diseases (AIDs) are a group of rare disorders characterized by persistent or recurrent inflammation caused by the hyperactivation of mediators and innate immune cells (neutrophils, monocytes/macrophages). The paper describes the authors' own case of NLRP12-associated AIDs and the efficiency of canakinumab therapy and reviews a series of cases of the similar disease, which are given in the literature.

Background: Juvenile idiopathic arthritis (JIA) associated uveitis may be the cause of not only visual acuity decrement, but also blindness. At the same time, in some patients therapy with methotrexate can not prevent the development of these complications.Objective: Our aim was to investigate the efficiency and safety of using a tumor necrosis factor inhibitor (adalimumab) in patients with JIA-associated uveitis.Methods: We conducted a retrospective single-arm study of a series of cases. The results of using adalimumab were evaluated in patients with JIA-associated chronic anterior uveitis, who have been under observation for no less than 1 year before and after starting using adalimumab. The latter was prescribed due to progressing and/or recidivous methotrexate-resistant uveitis.Results: We have analyzed clinical case records of 36 children with JIA-associated uveitis. At the start of therapy with adalimumab, actual uveitis was diagnosed in 30 (83%) patients. Remission was achieved in 29 of 30 cases in 2 (2; 12) weeks in patients with actual uveitis. 11 (31%) patients had a uveitis exacerbation 28 (13; 69) weeks after adalimumab therapy started. Adalimumab reduced the exacerbation frequency from 4 (1; 9) to 0 (0; 1) exacerbations per year for one patient (p < 0,001), and reduced the proportion of patients who were treated with topical glucocorticosteroids (from 83 to 8%). There were no differences (in achieving remission and reducing exacerbation frequency) with regard to patients’ sex, involvement of one or both eyes in the disease onset, antinuclear factor seropositiveness, uveitis type and character of joints affection.Conclusion: Adalimumab promotes fast and long-lasting remission of JIA-associated methotrexate-resistant uveitis.