Dorota Turowska-Heydel

Etanercept treatment in juvenile idiopathic arthritis: The Polish registry

Summary 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.

Methotrexate efficacy and tolerability after switching from oral to subcutaneous route of administration in juvenile idiopathic arthritis

Objectives Methotrexate (MTX) is one of the most frequently used, highly effective disease-modifying drugs in juvenile idiopathic arthritis (JIA) therapy. The drug can be administered orally or subcutaneously, but the efficacy and tolerance of these two routes of administration raise doubts in JIA patients. The aim of the study was to evaluate MTX efficacy and tolerability after switching from the oral to the subcutaneous route of administration in children with JIA. Material and methods A single-centre, questionnaire-based assessment of MTX efficacy and tolerance in 126 unselected JIA patients with longer than 6 months of follow-up was performed. In all patients, MTX was initially administered orally. The response to MTX treatment was analysed according to American College of Rheumatology (ACR) paediatric criteria. Results Six-month MTX therapy was effective (ACR score ≥ 30) in 83 children (65.9%). The oral route of MTX administration was changed to subcutaneous in 32 patients after a mean period of 14 months due to intolerance (n = 20) or reluctance to take the oral formulation (n = 12). This group of children was significantly younger (p = 0.02) but did not differ from the group of children that continued oral treatment in other aspects, including MTX dose. Six months after switching from oral to subcutaneous MTX the ACR score remained unchanged. Three children (9.4%) still reported symptoms of drug intolerance. Conclusions The switch from oral to subcutaneous MTX may increase the response rate in JIA patients with intolerance of its oral formulation. The reluctance to take oral MTX can be anticipated in early childhood, and should be considered in the individualization of therapy, having also in mind the lower risk of severe gastrointestinal adverse drug reactions.

Original paper Prevalence of HLA-B27 antigen in patients with juvenile idiopathic arthritis

Introduction Human leukocyte antigen B27 (HLA-B27) is considered as a risk factor for development of juvenile idiopathic arthritis (JIA). The aim of this study was to analyse the prevalence of HLA-B27 antigen in JIA categories and its influence on disease onset and response to conventional therapy. Material and methods The retrospective analysis included 461 unselected children with JIA hospitalized in a single reference rheumatology centre between July 2007 and June 2012. The diagnosis was based on criteria by the International League of Association for Rheumatology. HLA-B27 was determined in 387 of all patients (84%) by hybridization of the amplified, labelled product to immobilize it on the microarray probe. Results HLA-B27 antigen was found in 104 of 383 affected children (27.2%), 48 of 206 girls (23.3%), and 56 of 177 boys (31.6%) – most frequently in patients with enthesitis-related arthritis (71%), psoriatic arthritis (50%) and unclassified cases (86.7%). The age of JIA onset was slightly (by 1 year) but significantly different in patients with and without HLA-B27 antigen [11 (8.5–14) vs. 10 (5–13.5) years.; p < 0.001]. The use of disease-modifying antirheumatic drugs (DMARDs) and corticosteroids was more frequently clinically ineffective in HLA-B27 positive than negative patients (23.1% vs. 15.2%; p = 0.09). Patients with polyarthritis, systemic, and psoriatic arthritis more frequently received biological therapy. HLA-B27 positive patients with enthesitis-related arthritis received biological therapy more frequently than HLA-B27 negative ones (20.4% vs. 0, respectively; p = 0.09). Conclusions HLA-B27 antigen is a strong risk factor for the development of enthesitis-related arthritis, and to a lesser extent for psoriatic arthritis and extended course of oligoarthritis. The presence of this antigen does not affect the disease onset but seems to predict resistance to therapy with disease-modifying drugs and corticosteroids.

Pachydermodactyly – a report of two cases

Pachydermodactyly (PDD) is a rare and benign form of digital soft tissues fibromatosis, which affects the skin of the fingers. The disorder is characterized by asymptomatic, symmetric, progressive soft tissue swelling of the proximal interphalangeal (PIP) joints of the fingers. The etiology of disease remains unknown. It is usually acquired, even though there are some publications that document family cases. It affects mainly adolescent men. We report two boys with the bilateral swelling of the of the PIP joints of the fingers and skin and subcutaneous tissue thickening. Based on clinical manifestations, radiological study and histopathological examination, pachydermodactyly was diagnosed. PDD is a rare and benign disorder, although it is important to consider other diseases, especially rheumatic conditions, in the differential diagnosis in order to avoid unnecessary additional tests and treatments.

Analysis of Clinical Symptoms and Laboratory Profiles in Children with Juvenile Idiopathic Arthritis in Malopolska Region (Poland) in the Years 2007-2010

1St. Louis Regional Specialistic Childrens Hospital, Department of Older Children with subunits of Neurology, Rheumatology and Rehabilitation, Krakow, Poland; 2Childrens University Hospital, Department of Paediatrics, Rheumatology and Environmental Diseases, Krakow, Poland; 3 Department of Pathophysiology, Medical University of Silesia, Katowice, Poland; 4 Department of Pediatric Rheumatology, Institute of Rheumatology, Warsaw, Poland

A nine-year-old patient affected by chromosomal aberration with the suspicion of juvenile idiopathic arthritis

We present a case study of a 9.5-year-old girl affected by chromosome 12 aberration with the suspicion of juvenile idiopathic arthritis (JIA). All the tests performed at the hospital and the presence of a genetic disorder ledus to search for a diagnosisother than JIA. We initially diagnosed spondyloepiphyseal dysplasia tarda (SEDT), which is related to chromosome 12. Certain signs and symptoms of this disease were presented by our patient at the time of admission. After analysing all the tests and the general conditionof the patient, we were unable to confirm this diagnosis. However, it is possible that the symptoms may occur during subsequentyears and may allow confirmation of SEDT in the future.

Vaccination and biological agents therapy for rheumatic diseases

W artykule omówiono ogólne zasady stosowania i rodzaje szczepień profilaktycznych u osób dorosłych i u dzieci. Dokonano przeglądu literatury na temat ryzyka infekcji u pacjentów z zapalnymi chorobami reumatycznymi oraz efektywności i bezpieczeństwa stosowanych u nich szczepień. Wykazano celowość stosowania szczepień u tych chorych, z uwagi na zwiększone ryzyko infekcji, zwłaszcza gdy są oni leczeni lekami biologicznymi. Przedstawiono opracowane w 2011 r. przez EULAR rekomendacje stosowania szczepień u osób dorosłych i u dzieci z chorobami reumatycznymi. S u m m a r y