Joanna Lipińska

Anticitrullinated Protein Antibodies and Radiological Progression in Juvenile Idiopathic Arthritis

Objective. The aim of the study was to investigate whether determination of anticitrullinated protein antibodies (ACPA) provides predictive information on severity of disease course and joint destruction in children with juvenile idiopathic arthritis (JIA). Methods. Sera from 74 children with JIA were examined for ACPA using the ELISA test. To assess joint destruction, plain radiographs of both hands were scored twice according to the Steinbrocker scale: at the beginning of observation and after 8.9 to 15.2 months (median 11.5 months) of the followup. Correlations between ACPA serum levels and the disease characteristics (type of JIA onset, disease activity, disease duration, radiological status) were investigated. Results. Twenty-six out of 74 examined children with JIA (35.0%) were ACPA-positive [> 5 relative units (RU)/ml]. ACPA were present in all types of JIA onset, including 36.6% of children with early stage JIA (disease duration < 6 months). All of the IgM-rheumatoid factor (RF)-positive children with polyarticular type of JIA onset were simultaneously positive for ACPA. ACPA levels correlated positively with disease activity at the beginning of the study (rho = 0.7196; p < 0.0001) and after followup (rho = 0.2485; p = 0.0486). Disease duration did not significantly affect ACPA serum levels. ACPA levels correlated positively with radiological joint destruction in children with JIA, both at the beginning of the study (rho = 0.4599; p = 0.0004) and after the followup period (rho = 0.5523; p < 0.0001). Conclusion. ACPA were superior to IgM-RF in diagnosing JIA and provided predictive information on severity of disease course and radiological outcome.

Recurrent arterial and venous thrombosis in a 16-year-old boy in the course of primary antiphospholipid syndrome despite treatment with low-molecular-weight heparin: a case report

IntroductionAntiphospholipid syndrome is a multisystem autoimmune disease characterized by arterial and/or venous thrombosis and persistent presence of antiphospholipid antibodies. It can be a primary disease or secondary when associated with other autoimmune diseases.Case presentationWe present a case of a 16-year-old Caucasian boy with a massive arterial and venous thrombosis in his lower limbs as well as in his central nervous system with clinical symptoms such as headaches and chorea. He did not present any clinical or laboratory signs of a systemic inflammatory connective tissue disease, including systemic lupus erythematosus. Based on the clinical picture and results of the diagnostic tests (positive antibodies against β2-glycoprotein and a high titre of anticardiolipin antibodies) we finally diagnosed primary antiphospholipid syndrome. During a 9-month follow up after the acute phase of the disease, he was treated with low-molecular-weight heparin. Neurological symptoms were relieved. Features of recanalization in the vessels of his lower limbs were observed. After a subsequent 6 months, because of the failure of preventive treatment – an incident of thrombosis of the vessels of his testis – treatment was modified and heparin was replaced with warfarin.ConclusionAlthough the preventive treatment with warfarin in our patient has continued for 1 year of follow up without new symptoms, further observation is needed.

Comparison of uveitis in the course of juvenile idiopathic arthritis with isolated uveitis in children – own experiences

Objectives Uveitis and juvenile idiopathic arthritis (JIA) relatively often coexist. Inflammatory changes in the anterior segment of the eye are the most common extra-articular symptom in children with JIA, and JIA is, in turn, the main systemic cause of anterior uveitis in children. The aim of our study was to compare the course of anterior uveitis accompanying JIA and isolated uveitis. Material and methods We analyzed 25 children with JIA and uveitis (group I) and 28 children with isolated uveitis (group II). The study population was retrospectively selected from the patients treated in our center in the years 1998–2016 through a search of the hospital database. All data were presented as descriptive statistics. Results In group I there was a higher percentage of girls than in group II (64% vs. 50%) and uveitis occurred at a significantly younger age (8.7 years vs. 11.6). Patients from group I more often presented with immunological abnormalities (positive antinuclear antibodies or HLA-B27 antigen). The majority of children from group I developed uveitis prior to (44%) or simultaneously with (20%) arthritis. In patients who first presented with uveitis, arthritis appeared on average after 28 months (median 12 months). In children in whom arthritis developed first, uveitis appeared on average after 51 months (median 36 months). In some patients the time interval between the involvement of these two organs was as long as 9–10 years. Four children from group I and three from group II were qualified for biological treatment. Conclusions The results of our analysis indicate the need for constant cooperation between the pediatric rheumatologist and the ophthalmologist. Although the risk of uveitis in JIA decreases with the disease duration, in some cases this complication can develop after many years. Children with present antinuclear antibodies, at younger age and of female gender should be subject to particularly close observation.

Choroba Kawasakiego u 11 dzieci – charakterystyka przebiegu klinicznego i reakcji na leczenie oraz wyniki długofalowej obserwacji pacjentów

Streszczenie Wstep Choroba Kawasakiego nalezy do ukladowych zapalen naczyn, wystepujących najcześciej u niemowląt i malych dzieci, czego powaznym powiklaniem mogą byc zmiany w naczyniach wiencowych. Dzieki postepowi diagnostyki coraz cześciej bywa rozpoznawana rowniez u dzieci polskich, u ktorych obserwuje sie przedluzającą sie gorączke i typowe objawy kliniczne. Cel Analiza przebiegu klinicznego, wynikow leczenia oraz dlugofalowej obserwacji pacjentow z rozpoznaniem choroby Kawasakiego. Pacjenci i metody Badaniem objeto 11 dzieci hospitalizowanych w Klinice Kardiologii i Reumatologii Dzieciecej UM w Łodzi w latach 2005–2010. W przeprowadzonej analizie uwzgledniono dane demograficzne, wstepne rozpoznanie, charakterystyke objawow klinicznych i badan laboratoryjnych, reakcje na leczenie oraz dlugofalową obserwacje pacjentow. Wyniki Wszystkie dzieci spelnialy kryteria konieczne do ustalenia rozpoznania. Jedynie u 1 dziecka nie obserwowano zajecia naczyn wiencowych. 8 dzieci dobrze zareagowalo na standardowe leczenie immunoglobulinami, u 3 konieczna byla modyfikacja w postaci dodatkowego wlewu z immunoglobulin lub podania pulsu z metylprednizolonu. W dlugofalowej obserwacji stwierdzano systematyczną regresje zmian w naczyniach. Wnioski Autorzy podkreślają znaczenie wczesnego ustalenia rozpoznania i wdrozenia prawidlowego leczenia oraz koniecznośc dlugoterminowej obserwacji dzieci po przebytej chorobie Kawasakiego.