A Case of Remission of Systemic Juvenile Rheumatoid Arthritis(Still's Disease) Treated with High-dose Intravenous Gammaglobulin

ABSTRACT

High dose intravenous gammaglobuline (IVLG) therapy is effective in some of the autoimmune diseases. Although the exact mechanism of action of IVIG is uncertain, the action as a neutralizing antibody against unknown etiologic agents, the action of blocking of Fc receptors of effector cells, or the action as a antiidiotypic antibody are suggested. We report a case of 12 year old girl with systemic juvenile rheumatoid arthritis who was treated with high dose IVIG and got a remission. In August 1990 she was admitted to our hospital. because of intermittent fever, transient rash and multiple arthralgia. Under the diagnosis of systemic juvenile rheumatoid arthritis, aspirin (4.0g/day) had been given with symptom improvement. She was readmitted in October 1990 because of aspirin intoxication and acute fulminant hepatitis. She was discharged after recovery and any medicine was not prescribed. In November 1990 she was admitted because of epigastric pain, vomiting, intermittent fever, multiple arthritis, and mild hepatomegaly. Total parenteral alimentation had been given under the diagnosis of superior mesenteric artery syndrome and gold sodium thiomalate (Myochrysine, 5 and 10 mg, two weekly IM injection) was given in conjunction with prednisolone (30 mg/day) and naproxen (375 mg/day). She was admitted again in February 1991 due to the fever, coughing, rash, and hepatosplenomegaly. Pneumonia and leukopenia (2100/mm(3)) were found and gold sodium thiomalate injection was discontinued. Gammaglobulin 1 g/kg/day was given intravenously for 2 consecutive days with dramatic symptom improvement. Five more monthly IV gammaglobulin had been given and the side reaction of injection were nausia, fever, and headache which were controlled by the decrease of infusion rate. Four months after the last IVIG injection she had no symtom of arthritis and the hepatosplenomegaly was decreased. Hemoglobin level was increase to 12.2 mg/dL form 6.2mg/dL and ESR was decrease to 15mm/h. The oral prednisolne and ibuprofen were stopped one year after th last IVIG injection. All the laboratory parameters of arthritis and physical examinations had been normal for more than two year after the stop of all the medications until March of 1994. We suggest that high dose intravenous gammaglobulin can be one of treatments for severe systemic juvenile rheumatoid arthritis.