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    ABSTRACT: We describe a patient with systemic onset juvenile idiopathic arthritis refractory to disease-modifying antirheumatic drugs, intravenous gamma globulin, and TNF inhibitors (etanercept and infliximab), in whom treatment with rituximab resulted in remission of systemic symptoms (rash and fever), a fall in erythrocyte sedimentation rate, C-reactive protein, and ferritin serum levels, with recovery in disability index and improvement of arthritis. A total of 4 cycles of rituximab were given over 18 months because of relapses. Since her last course, she remains stable and asymptomatic. To our knowledge, this is the first case reported on a long-lasting beneficial effect of rituximab in a patient with soJIA.
    Journal of clinical rheumatology: practical reports on rheumatic & musculoskeletal diseases 10/2009; 15(7):363-5. DOI:10.1097/RHU.0b013e3181ba3c6f · 1.25 Impact Factor
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    ABSTRACT: Interleukin-1 antagonist anakinra is increasingly used as third-line therapy in adult-onset Still disease (AoSD) after corticosteroids (CS) and immunosuppressive drugs have failed or have induced serious adverse effects. We recently had to use anakinra earlier in the course of AoSD in two patients. In both cases, the disease had a major social impact. One patient was a plane pilot, and he was forbidden to continue his job as long as he was on CS. He also had developed CS-induced central serous chorioretinopathy (CSC), and methotrexate did not allow a prompt reduction in the prednisone dosage. Anakinra had a dramatic effect and allowed the complete withdrawal of CS and methotrexate and the full remission of CSC, and the patient could pilot again. The doses of anakinra have been since then successfully reduced by two thirds. In the second case, AoSD occurred a few weeks before the patient's A-level exams. The disease was resistant to prednisone 1 mg/kg for 15 days. Anakinra controlled all symptoms in 3 days and was stopped 3 months later. She has not relapsed since then. No adverse drug reaction has occurred. These cases suggest that a treatment by anakinra of short duration could be used early in AoSD to induce a prompt remission, to avoid the adverse effects of high dose CS and/or immunosuppressive drugs and to reduce the social impact of the disease.
    Clinical Rheumatology 10/2010; 29(10):1199-200. DOI:10.1007/s10067-010-1459-6 · 1.77 Impact Factor
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    ABSTRACT: Pierre QuartierUnité d'Immunologie-Hématologie et Rhumatologie pédiatriques, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, FranceAbstract: Cryopyrin-associated periodic syndrome (CAPS) include a group of rare autoinflammatory disorders, the spectrum of which ranges from the mildest form, ie, familial cold autoinflammatory syndrome to more severe phenotypes, ie, Muckle-Wells syndrome, and chronic infantile neurological cutaneous and articular syndrome, also known as neonatal-onset multisystem inflammatory disease. Three interleukin (IL)-1 antagonists have been tested in adults and children with CAPS, ie, anakinra, a recombinant homolog of the human IL-1 receptor antagonist; rilonacept, a fusion protein comprising the extracellular domains of IL-1 receptor I and the IL-1 adaptor protein, IL-1RAcP, attached to a human immunoglobulin G molecule; and canakinumab, the anti-IL-1β monoclonal antibody. Following rapid clinical development, rilonacept and canakinumab were approved by both the US Food and Drug Administration and the European Medicines Agency for use in adults and children. This review describes how the study of CAPS has helped us to understand better the way the innate immune system works, the pathogenesis of autoinflammatory syndromes, and the key role of IL-1. It also reviews the effects of IL-1 blockade in CAPS and other disorders, in particular systemic juvenile idiopathic arthritis, adult-onset Still's disease, and gout. Finally, this review covers some issues addressed by very recent and ongoing work regarding treatment indications, from orphan diseases to common disorders, continuous versus intermittent treatment, the pharmacokinetics, pharmacodynamics, and optimal dosages of the different drugs, as well as the need for Phase IV trials, exhaustive registries, and long-term follow-up of several patient cohorts.Keywords: inflammation, interleukin-1, cytokines, treatment
    Open Access Rheumatology: Research and Reviews 01/2011; DOI:10.2147/OARRR.S6696