Revising diagnosis of juvenile idiopathic arthritis in adults: a single-center retrospective study.

The study aimed to assess how many adult patients with juvenile idiopathic arthritis (JIA) treated with biologics fulfill classification criteria for adult rheumatic diseases and to evaluate the course of JIA in adulthood. 138 patients with JIA over 18 years old treated with biologics were included in a cross-sectional observative study. Among 138 adult patients with JIA treated with biologics, 81 patients remained with JIA diagnosis. 57 patients were rediagnosed. 31 patients met the criteria for spondyloarthropathy, among them 18 patients for ankylosing spondylitis, 10 patients for psoriatic arthritis, and 3 patients for non-radiographic axial spondyloarthritis. Rheumatoid arthritis was diagnosed in 24 patients and adults' Still disease in 2 patients. 84 patients of all adults with JIA received one biologic agent, 40 received two biologic agents, and 14 received three or more biologic therapies. 10 patients received biologic agents out of recommendations for JIA. Of the adult JIA patients treated with biologics, 41% met the classification criteria for adult inflammatory diseases. Spondyloarthropathy and rheumatoid arthritis were most commonly diagnosed. Nearly 40% of adult JIA patients required at least one modification of biological treatment. Therefore, it is worth considering a revision of JIA to adult-onset inflammatory disease entities, as it broadens the spectrum of disease-modifying drugs.

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.

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.

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.

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.