Background. Primary tumoral calcinosis is an orphan disease. There are few data in the literature on the incidence of this disease, as well as clinical recommendations for treatment. Clinical case. This report presents the case of an 11.5-year-old boy with primary tumoral calcinosis and equinus deformity of the foot. The patient had multiple foci of the subcutaneal calcification, cannot walk, experienced fatigue, and had high fever and equinus deformity of the left foot. Immunological and genetic studies were performed, but any specific mutations were not found. After the diagnosis was verified and interleukin-1 inhibitor therapy was prescribed, there was a significant positive trend observed in the patient: a significant improvement in the patients general condition, a decrease in the number of calcinates, and a reduction in inflammation. Calcification of the Achilles tendon and gastrocnemius muscle was the cause of the deformity of the left foot. Discussion. Significant improvement was achieved during treatment: the boy started walking, fatigue was decreased, no new calcificates were formed, and inflammation was under the control. Using an inhibitor of interleukin-1 as a permanent therapy of primary tumoral calcification allowed performsurgical treatment without complications from an operation site, as well as a relapse of deformity. Conclusion. The clinical case presented here demonstrated the application of an interdisciplinary approach to the treatment of an extremely rare disease.

В статье приведены данные литературы о сравнительно редком заболевании -гипертрофической остеоартропатии (ГОА).

BackgroundPatients with juvenile idiopathic arthritis (JIA) may have lower protective levels of anti-vaccine antibodies (AVA) due to high inflammatory activity, interrupted or incomplete vaccination schedule, using of immune-modulating drugs, e.g. systemic corticosteroids (CS), methotrexate (MTX) and biologics [1].ObjectivesThe aim of our study was to evaluate levels of AVA in the patients with JIA.MethodsWe included data about 90 JIA (26 M and 64 F) aged from 2 to 17 years, who received scheduled vaccination before the age of 2 years and before JIA onset. In all patients the Ig G anti-measles (AM), anti-parotitis (AP), anti-hepatitis B (AHB), anti-diphtheria (AD) and anti-rubella (AR) AVA levels were detected with ELISA. In each patient we evaluate the type of the JIA (oligoarthritis – OA (n=38), polyarthritis – PA (n=36), systemic-SA (n=7) and enthesitis-related arthritis – ERA (n=10), routine disease activity and treatment. In healthy controls were measured anti-measles (n=40) and anti-parotitis (n=30) antibodies (AB) for comparison with JIA.ResultsThe main demographic characteristics: age of inclusion in the study 11 (8-15) years, disease onset–6 (4-8) years, JIA duration–4 (2-7) years. The AM AB in JIA patients were 0.2 (0.0-0.5) IU/ml and in HC 0.3 (0.2-1.1) IU/ml (p=0.00002), despite the higher age of JIA patients than HC (p=0.0000001); AP AB were 2.6 (1.0-5.1) IU/ml vs 1.1 (0.0-4.9) IU/ml in JIA and HC, respectively (p=0.08). Protective levels of AM AB was detected in 50% of all JIA population, vs. HC – 87.5% (p=0.00005), AP–67.7% vs. 60% in HC (p=0.076), AHB – 54.4%, AD–50%, AR–97.8%. The main data related to vaccination status in the table. We have found correlation between JIA duration and levels AM AB (r=-0.27, p=0.015), AP AB (r=-0.22, p=0.039), AD AB (r=-0.27, p=0.015); MTX treatment with AM AB (r=-0.37, p=0.001), and AD AB (r=-0.29, p=0.007); treatment with biologics and AM AB (r=-0.25, p=0.018), and using more than one biologics with AR AB (r=-0.27, p=0.047). In the regression model only MTX had a negative impact on AM (r=-0.001) and AD AB level (p=0.01). ParameterOAPASAERAPOnset age, y5 (4-7)6 (4-8)8 (4-11)9 (7-12)0.01JIA duration, y3 (2-5)5.4 (2-9)4 (1-6)6 (5-10)0.006# AM vaccinations2 (1-2)2 (1-2)1 (1-2)2 (2-2)0.005# of patients with AM revaccination,%5160431000.06AM IgG, IU/ml0.2 (0-0.6)0 (0-0.4)0.2 (0; 1.6)0.2 (0.1-0.3)0.2Protective AM IgG level,%583957500.42# of patients with AP revaccination,%5061431000.05# AP vaccinations1.5 (1-2)2 (1-2)1 (1-2)2 (2-2)0.004AP IgG, IU/ml3 (1.5-5)2.3 (0-5)1.3 (0; 5)2.6 (1.1-3.3)0.67Protective AP IgG level,%827271750.8# AD vaccinations4 (4-5)5 (4-5)4 (4-5)5 (4-6)0.09AD IgG, IU/ml0.1 (0.03-0.3)0.1 (0.03-0.2)0.04 (0.0; 0.2)0.16 (0.05-0.4)0.64Protective AD IgG level,%535029500.71# AHB vaccinations3 (3-3)3 (3-3)3 (3-3)3 (3-3)0.87AHB IgG, IU/ml5 (0-20)17 (2-45)1 (0; 10)19 (4-228)0.09Protective AHB IgG level,%456729)630.13# AR vaccinations1 (1-2)1 (1-2)1 (1-1)2 (2-2)0.57AR IgG, IU/ml72 (35-145)54 (27-96)200 (18; 200)67 (42-2000)0.52Protective AR IgG level,%1009710010...

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