Jacek Postępski

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.

Complex Regional Pain Syndrome type I with atypical scintigraphic pattern — diagnosis and evaluation of the entity with three phase bone scintigraphy. A case report

Complex Regional Pain Syndrome (CRPS) is a neurological disorder of unknown etiology which may lead to severe disability. Its diagnosis is very difficult and based on diagnostic criteria which have been changing over last years. Still, there is no golden standard in diagnosis of this entity. Three-phase bone scan is a widely used diagnostic modality which has been proved useful in CRPS evaluation. The syndrome may present various scintigraphic patterns. Different diagnostic modalities can also be helpful when CRPS is suspected including plain film radiography, magnetic resonance imaging and ultrasonography. Multidisciplinary approach is necessary for proper and quick diagnosis. We present a case of CRPS in 12-year-old girl in whom the diagnosis was based on the bone scan.

Exceptional manifestation of polyautoimmunity in a very young girl – a case report

Polyautoimmunity is defined as the presence of more than one autoimmune disease in a single patient. The exact pathogenic mechanisms responsible for the coexistence of distinct autoimmune diseases within an individual have not been clearly explained. We report a case of a very young girl with the extremely rare co-existence of four distinct autoimmune diseases i.e. juvenile idiopathic arthritis, type 1 diabetes mellitus, coeliac disease and autoimmune hepatitis, recognized based on validated international classification criteria. The best to our knowledge there has been no case reporting coexistence of these particular four disorders in an individual. Moreover, all these diseases occurred during first three years of life, which also cause that case unique. Molecular studies of human leukocyte antigen (HLA) class II in our patient showed the presence of the HLA DRB1*01, HLA DRB1*03, HLA DQB1*02, HLA DQB1*05 molecules, which may suggest immunogenetic links between those autoimmune diseases. The presented case highlights the importance of active screening for other autoimmune diseases, if a patient with one autoimmune disease manifests with new or nonspecific symptoms.

TNF inhibitors in the treatment of uveitis in the course of juvenile idiopathic arthritis

Uveitis is the most common extraarticular complication of juvenile idiopathic arthritis (JIA). TNF inhibitors are a promising new therapeutic option in the treatment of chronic uveitis in JIA.

Effectiveness of tnf inhibitors therapy in children with juvenile idiopathic arthritis aged 2 to 5 years.

Juvenile idiopathic arthritis (JIA) therapy in the youngest patients (pts) is particularly difficult. Etanercept (ETA) and adalimumab (ADA) are approved for use in moderate and severe JIA in pts older than 2 years, but available data about treatment in the youngest children are limited.