CGD is an immunodeficiency caused by deletions or mutations in genes that encode subunits of the leukocyte NADPH oxidase complex. Normally, assembly of the NADPH oxidase complex in phagosomes of certain phagocytic cells leads to a “respiratory burst”, essential for the clearance of phagocytosed micro-organisms. CGD patients lack this mechanism, which leads to life-threatening infections and granuloma formation. However, a clear picture of the clinical course of CGD is hampered by its low prevalence (∼1∶250,000). Therefore, extensive clinical data from 429 European patients were collected and analyzed. Of these patients 351 were males and 78 were females. X-linked (XL) CGD (gp91phox deficient) accounted for 67% of the cases, autosomal recessive (AR) inheritance for 33%. AR-CGD was diagnosed later in life, and the mean survival time was significantly better in AR patients (49.6 years) than in XL CGD (37.8 years), suggesting a milder disease course in AR patients. The disease manifested itself most frequently in the lungs (66% of patients), skin (53%), lymph nodes (50%), gastrointestinal tract (48%) and liver (32%). The most frequently cultured micro-organisms per episode were Staphylococcus aureus (30%), Aspergillus spp. (26%), and Salmonella spp. (16%). Surprisingly, Pseudomonas spp. (2%) and Burkholderia cepacia (<1%) were found only sporadically. Lesions induced by inoculation with BCG occurred in 8% of the patients. Only 71% of the patients received antibiotic maintenance therapy, and 53% antifungal prophylaxis. 33% were treated with γ-interferon. 24 patients (6%) had received a stem cell transplantation. The most prominent reason of death was pneumonia and pulmonary abscess (18/84 cases), septicemia (16/84) and brain abscess (4/84). These data provide further insight in the clinical course of CGD in Europe and hopefully can help to increase awareness and optimize the treatment of these patients.

Adalimumab therapy seems to be an efficacious option for the treatment of children with juvenile rheumatoid arthritis. (ClinicalTrials.gov number, NCT00048542.)

RHS may be a more common complication of systemic disease in childhood than previously thought. This life-threatening complication should be diagnosed promptly, as it calls for the immediate withdrawal of potentially triggering medications, anti-infective therapy when relevant, and urgent immunosuppressive treatment, measures that are very often effective. Cyclosporin A may be the drug of choice.

Objective. To assess the efficacy of etanercept in patients with juvenile idiopathic arthritis (JIA), and to assess the tolerance of these patients to etanercept.Methods. All JIA patients with active chronic polyarthritis, who were first treated with etanercept between November 1999 and June 2001 in 18 French centers because of poor response or intolerance to methotrexate, were included in this open-label, prospective, multicenter study. A standardized questionnaire was sent to the treating physicians. We assessed the validated international core-set score for JIA activity every 3 months and performed an intent-totreat analysis. We also compared the risk of treatment failure in patients defined as having systemic-onset, oligoarticular-onset, or polyarticular-onset JIA.Results. Sixty-one patients were enrolled and were followed up for a median of 13 months. Treatment had to be stopped in 1 patient who became pregnant and in 12 patients due to severe side effects, including neurologic or psychiatric disorders, retrobulbar optic neuropathy, major weight gain, severe infection, cutaneous vasculitis with systemic symptoms, hemorrhagic diarrhea, uveitis flare, and pancytopenia. All of these side effects disappeared after discontinuation of etanercept. Crohn's disease was subsequently diagnosed in 1 child. Scores improved by >30% in 73% of patients after 3 months, but this proportion decreased to 39% after 12 months. The response rate was significantly lower in patients with systemic-onset JIA than in those with oligoarticular-or polyarticular-onset JIA.Conclusion. Treatment of JIA with etanercept may be associated with a wide spectrum of severe side effects. Although most patients initially respond to etanercept, this initial response is not always followed by sustained improvement over longer periods of time. In addition, the higher rate of treatment failure in the group with systemic-onset JIA indicates that these patients in particular may require alternative treatments.Juvenile idiopathic arthritis (JIA) represents several conditions that are characterized by early-onset arthritis (before age 16 years) that persists in one or more joints for at least 6 weeks, and in which infectious arthritis and other well-defined illnesses have been actively excluded (1). Most of these conditions were formerly known as juvenile rheumatoid arthritis (JRA) or juvenile chronic arthritis. JIA may be associated with severe disability and life-threatening complications, particularly in patients in whom polyarthritis develops and who do not respond satisfactorily to treatment (2). Patients in whom polyarthritis develops are usually treated with methotrexate, 0.2-1.0 mg/kg of body weight weekly (3,4). Other disease-modifying or immunosuppressive drugs often are less effective or are not toler-

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