Intravenous gamma globulin therapy in systemic juvenile rheumatoid arthritis

Abstract

Intravenous (IV) gamma globulin has been successfully used as replacement therapy for antibody‐deficient patients and, more recently, in the treatment of autoimmune diseases such as idiopathic thrombocytopenic purpura, myasthenia gravis, and Kawasaki disease. In view of the successful treatment of these diseases, we initiated a pilot study of the effect of IV gamma globulin in systemic juvenile rheumatoid arthritis (JRA). Eight patients with active systemic JRA that was unresponsive to first‐line agents, second‐line agents, and/or corticosteroids received this therapy monthly for 6 months. Outcome measures included changes in articular and extraarticular features, steroid dosage, and laboratory parameters. Following IV gamma globulin therapy, there was significant improvement in arthritis and/or morning stiffness in 5 of 8 patients, while extraarticular features significantly improved in 7 of 8 patients. At study entry, 6 of 8 patients were receiving prednisone; at study end, prednisone was discontinued in 3 patients and decreased by more than 50% in the other 3. Overall, there was an 80% reduction in the prednisone dosage. Initially, all patients had anemia, low levels of serum albumin, and an elevated erythrocyte sedimentation rate, while a thrombocytosis was seen in 7 of 8 patients. Serum IgG was initially elevated in 6 patients. IV gamma globulin therapy resulted in a significant increase in hemoglobin and albumin levels and a significant decrease in the mean serum IgG level, platelet count, and erythrocyte sedimentation rate. In only 1 patient did IV gamma globulin fail to significantly improve the clinical or laboratory features of the disease. We suggest that this therapy may be beneficial in the treatment of systemic JRA.

Hyperferritinemia as indicator for intravenous immunoglobulin treatment in reactive macrophage activation syndromes

“…Recent studies found only limited benefit of IVIG during the chronic course of juvenile-onset Still's disease [17,18]. But this does not contradict our results, since we used IVIG as an emergency treatment during fulminant macrophage activation in adults.…”

Systemic juvenile rheumatoid arthritis complicated by two different renal lesions

“…Based on the work of Silverman et al [5], we decided to treat her with intravenous immunoglobulin (IVIG). She received infusions of 1.5 g/kg every 2 weeks.…”

Adult Still's Disease with Sjoegren's Syndrome Successfully Treated with Intravenous Pulse Methylprednisolone and Oral Cyclophosphamide.

“…It has been reported that some patients can be adequately treated with cytotoxic drugs including azathioprine, chlorambucil, cyclophosphamide (5) and lately, methotrexate (16-1 8). Recently, a few patients with juvenile-onset Still's disease have been treated with novel therapeutic modalities, including y-interferon ( 1 9), intravenous y-globulin (20) and cyclosporin (21). Such treatments have not yet been used for patients with adult Still's disease, but they certainly offer the possibility of future application especially to the refractory cases.…”