The use of radiosynovectomy in patients with juvenile idiopathic arthritis. Assessment of treatment efficacy and safety.

OBJECTIVES: The aim of the study was to evaluate the usefulness of knee joint radiosynovectomy (RS) in patients suffering from juvenile idiopathic arthritis (JIA). MATERIAL AND METHODS: One hundred RS procedures performed in 58 patients with JIA in average age 10.4 years were evaluated. RESULTS: After 6 weeks, a decrease in the number of cases with joint pain from 90.3% to 29%, with joint oedema from 100% to 74.5%, with joint exudate from 100% to 60.6%, with gait disorders from 19.4% to 3.2%, with joint mobility disorders from 51.1% to 26.6% in the RS cases was observed. A reduction of the score in the Colorado scale from 10.9 to 4.66, in the pain visual analogue scale (VAS) from 50 to 10, in the illness VAS assessed by the patient/parent from 69.9 to 32.4, in the illness VAS assessed by the physician from 68.8 to 36.9 was observed. Six months after the RS procedure, a reduction in the number of cases with joint pain from 89.5% prior to the procedure to 29.5%, with oedema from 100% to 58.3%, with exudate from 100% to 46.9%, with gait disorders from 20% to 2.1%, with joint motility disorders from 51.1% to 26.1% was achieved. The score in the Colorado scale was reduced from 10.9 to 4.04, in the pain VAS from 40 to 0, in the illness VAS assessed by the patient/parent from 69.7 to 27.9, in the illness VAS assessed by the physician from 68.8 to 32.4. In ultrasound examinations, the greatest improvement compared to the initial condition was recorded in the 6th month after the RS. Radiosynovectomy was positively evaluated by parents and patients in 34 anonymous surveys. Early and late observations (average 1473 days) did not show lesions at the isotope injection site, and no neoplastic lesions were observed. CONCLUSIONS: Radiosynovectomy is a valuable therapeutic option for local treatment in patients with JIA.

Assessment of anti-HBs antibody concentration in children with juvenile idiopathic arthritis treated with biological drugs, vaccinated against viral type B hepatitis in infancy.

OBJECTIVES: The introduction of vaccinations against viral hepatitis B in the years 1994-1996 in Poland significantly improved the epidemiological situation of hepatitis B virus (HBV) infections in our country. According to the report of the National Institute of Public Health - National Institute of Hygiene in 2018, 40 cases of acute hepatitis B were noted while still in the 1980s between 10 and 20 thousand new cases were reported annually. The aim of the study was to determine whether in children treated with biological drugs (adalimumab, etanercept, infliximab) due to juvenile idiopathic arthritis (JIA), vaccinated against hepatitis B in infancy, a protective concentration of anti-HBs antibodies persists. In patients, the value ≥ 10 mIU/ml is regarded as a protective concentration of antibodies, determined at least four weeks after administration of the last vaccine dose. Among healthy individuals, presence of anti-HBs antibodies in any concentration means seroprotection. No booster vaccinations are recommended in basically vaccinated healthy individuals. MATERIAL AND METHODS: The concentrations of anti-HBs antibodies were determined in 56 children with JIA (38 girls - 67.9% and 18 boys - 32.1%) aged from 2 years and 4 months to 17.5 years, treated for at least three months with biological drugs. The diagnosis of JIA was made based on the International League of Associations for Rheumatology (ILAR) criteria. All studied patients were at the stable stage of the disease and received a full course of hepatitis B vaccination during infancy (in accordance with 0,1,6 months injection scheme). RESULTS: In the studied children a protective anti-HBs antibody concentration was found in 34 cases (60.7%), and 22 children (39.3%) had anti-HBs antibody concentration < 10 mIU/ml (in these children no seroprotection was found). CONCLUSIONS: The post-vaccination antibody concentration should be determined in children with JIA, treated with biological drugs and, in case of absence of a protective concentration, revaccination should be started.

Diagnostic and therapeutic difficulties in a patient with chronic recurrent multifocal osteomyelitis coexisting with ulcerative colitis.

Chronic non-bacterial osteomyelitis (CNO) is a rare autoinflammatory bone disease, affecting mainly children. CNO includes a broad clinical spectrum of symptoms and signs, from mild, limited in time, unifocal osteitis to severe, chronic, active or recurrent, multifocal osteomyelitis. In 2014 diagnostic criteria for CNO were proposed, the Bristol Criteria for the Diagnosis of Chronic Non-bacterial Osteitis, taking into account the clinical picture - location and number of inflammatory foci, characteristic changes on radiological examination (X-ray) and magnetic resonance imaging (MRI), C-reactive protein (CRP) concentration, and changes in bone biopsy. The paper presents the case of a four-year-old boy in whom the diagnosis of multifocal osteomyelitis coexisting with ulcerative colitis was established. Attention was paid to the long diagnostic process of the disease, requiring in the first place differentiation with proliferative diseases. The choice of drugs was also a significant problem in the patient described in view of both intolerance of individual preparations and their ineffectiveness.

Rheumatic fever - new diagnostic criteria.

Rheumatic fever (RF) is an autoimmune disease associated with group A ß-hemolytic streptococcal infection, in the course of which the patient develops carditis, arthritis, chorea, subcutaneous nodules and erythema marginatum. Rheumatic fever diagnosis is based on the Jones criteria, developed in 1944, then revised twice by the American Heart Association (AHA), in 1992 and recently in 2015. The last revision of the Jones criteria consists mainly in the supplementation of the major criteria with echocardiographic examination, the introduction of a concept of subclinical carditis and the isolation of low, medium and high risk populations among the patients. AHA recommends that all the patients with suspected RF undergo Doppler echocardiographic examination after the Jones criteria have been verified, even if no clinical signs of carditis are present.

Self-healing juvenile cutaneous mucinosis.

Girl, aged 4 years old, began the disease with pain of the lower extremities, fever up to 38°C and signs of upper airway infection. Then the patient developed oedema and redness of the whole face, thickened skin, subcutaneous nodular foldings of the frontal, occipital, cervical and axillary regions, extensor areas of the joints; fine, hard whitish nodules in the frontal region and over interphalangeal joints of the hands, pruritus; oedemas of the ankles, knees and joints of the hands, cervical lymphadenopathy and hepatomegaly. Blood tests at the moment of the diagnosis revealed elevation of markers of inflammation as ESR and CRP, leukocytosis, thrombocytosis, hypoalbuminemia, and hyper-alfa-2-globulinemia. Histopathological examination of the skin biopsy specimen and subcutaneous tissue revealed myxoid subcutaneous tissue located under the dermis and a section consisting of myxoid mesenchymal tissue with inflammatory infiltration by histiocytic cells. The presence of acid mucopolysaccharides in fields of the myxoid tissue was also observed. The self-healing juvenile cutaneous mucinosis (SJCM) was diagnosed.

Assessment of the level of vaccine-induced anti-HBs antibodies in children with inflammatory systemic connective tissue diseases treated with immunosuppression.

OBJECTIVES: Protective vaccinations are the most effective method of prevention of type B virus hepatitis. The aim of the study was to determine whether in children receiving immunosuppressive therapy due to inflammatory systemic connective tissue diseases the protective concentration of the anti-HBs antibodies produced after vaccination against type B virus hepatitis in infancy is maintained. MATERIAL AND METHODS: The concentration of anti-HBs antibodies was assessed in the sera of 50 children with inflammatory connective tissue diseases - 37 girls (74%) and 13 boys (26%), aged 1.5-17.5 years - during the immunosuppressive treatment, which lasted at least 6 months. The control group consisted of 50 healthy children - 28 girls (56%) and 22 boys (44%) aged 2-17 years. All children were vaccinated in infancy with Engerix B vaccine according to the 0-1-6 months schedule. The antibody concentration of ≥ 10 mIU/ml in patients is regarded as protective. RESULTS: No protective antibody concentrations were found in 25 cases (50%) in the group of diseased children and only in 2 children in the control group (4%). CONCLUSIONS: The concentration of vaccine-induced antibodies should be assessed in children with inflammatory systemic connective tissue diseases and, in case of the absence of a protective concentration, revaccination should be started. The use of glucocorticosteroids, synthetic and biological disease-modifying antirheumatic drugs is no contraindication to vaccination against hepatitis B.

Etanercept treatment in juvenile idiopathic arthritis: the Polish registry.

BACKGROUND: To evaluate the long-term safety and efficacy of etanercept treatment in Polish patients with juvenile idiopathic arthritis (JIA). MATERIAL/METHODS: The study involved patients, fulfilling the JIA criteria of the International League of Associations of Rheumatology (ILAR), who were started on etanercept therapy after methotrexate and other synthetic disease-modifying antirheumatic drugs (DMARDs) had proven ineffective. Patient data were collected in an electronic registry. Disease improvement was assessed based on Giannini's criteria. RESULTS: The statistical analysis involved 188 patients. Significant improvement was observed in all clinical and laboratory parameters after the first month of therapy and was maintained in the following months. ACR Pediatric 30, 50, 70, 90, and 100 improvement was observed in 81.4%, 65.9%, 27.5%, 16.2%, and 15%, respectively, of patients after 3 months and in 94.7%, 88.4%, 62.1%, 34.7%, and 26.3%, respectively, after 24 months of treatment. Throughout the 72-month safety observation period, 1162 adverse events were reported; the exposure-adjusted AE rate was 2.96 per patient per year. CONCLUSIONS: In patients with various subtypes of JIA resistant to conventional DMARD treatment, etanercept resulted in significant and long-lasting improvements in disease activity. Combination treatment with etanercept and a DMARD was well tolerated.

Hepatitis B virus genotypes in children with chronic hepatitis B in Poland.

OBJECTIVES: To analyse the distribution of HBV genotypes in Polish children with chronic hepatitis B, and to assess the relation between the viral genotype and the severity of liver damage. METHODS: Serum samples from children with chronic hepatitis B were used for biochemical and serological testing, and for determination of HBV genotypes by a nested-multiplex-polymerase chain reaction. Liver biopsies were obtained for histological assessment, which was performed according to the Batts and Ludwig scoring system of chronic hepatitis. RESULTS: Of 78 children with chronic hepatitis B, 74 had an identifiable HBV genotype: 86.5% were infected with genotype A, and 13.5% were carriers of genotype D. The frequency of HBeAg clearance and the levels of alanine aminotransferase and serum aspartate transaminase were comparable in both genotype groups. There was no correlation between the HBV genotype and either activity of liver inflammation or liver fibrosis. CONCLUSIONS: This study shows that the distribution of HBV genotypes in Polish children with chronic HBV infection reflects the general prevalence of HBV genotypes in Europe. The course of chronic hepatitis B in children is not significantly influenced by viral genotypes A or D.