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Successful treatment of a patient with refractory adult-onset still disease with anakinra.

To the Editor: Adult-onset Still disease (AOSD), the adult variant of the systemic form of juvenile rheumatoid arthritis (RA), is an uncommon disorder of unknown origin. Traditionally, nonsteroidal anti-inflammatory drugs and steroids have been used for treatment of AOSD. Many patients require second-line agents to control manifestations of the disease or to reduce steroid dependence, such as methotrexate, cyclosporine, cyclophosphamide, and gold, with variable results. Newer biologic agents, such as etanercept and infliximab, have shown promising results in patients with AOSD. We report a patient with severe and refractory Still disease who responded to treatment with the interleukin-1 receptor antagonist (IL-1Ra), anakinra. P.P. is a 37-year-old man with a history of AOSD diagnosed in 1985 when he became acutely ill with high fevers, sore throat, polysynovitis, pleuropericarditis, an erythrocyte sedimentation rate (ESR) of 130 mm/ hour, and leukocytosis (white blood cell [WBC] count 43,000/mm). At that time, he was treated with high-dose prednisone and a tapering course of indomethacin, which he last took in 1987. He did well until May of 1990, when he had a relapse of AOSD with similar symptoms successfully controlled with a high dose of prednisone, which was gradually tapered and discontinued over 10 months. He remained in complete remission for 10 years. In November of 2001, his disease relapsed, with high fevers up to 102 °F; chills; drenching night sweats; polyarthralgias involving shoulders, elbows and hands; myalgias; fatigue; sore throat; cervical lymphadenopathy; leukocytosis (WBC count of 16,000/mm), elevated ESR, and ferritin at 792 ng/mL. He fulfilled Yamaguchi diagnostic criteria for AOSD, although typical rash was not observed during this relapse. He was prescribed prednisone 80 mg/day in 4 split doses and indomethacin 50 mg every 8 hours, with improvement on that regimen initially. However, his condition worsened when prednisone was tapered to 30 mg/day. In January of 2002, he was admitted to the hospital with dyspnea, chest tightness, fevers, drenching night sweats, profound fatigue, arthralgias, and generalized body aches. Laboratory data included significant leukocytosis (WBC count 47,000/mm), ESR 120 mm/hour, ferritin 5742 ng/mL, and mildly elevated transaminases (alaninaminotransferase 91, aspartateaminotransferase 61). Antinuclear antibodies and rheumatoid factor were negative. Chest x-ray revealed basilar atelectasis without pleural effusion, and echocardiogram was negative for pericardial effusion. While in the hospital, he was treated with intravenous Solu-Medrol 240 mg daily for 5 days, with partial symptomatic improvement, and was discharged on oral prednisone 120 mg/day (1 mg/kg/day) in 4 split doses with gradual tapering. Indomethacin was discontinued. Methotrexate was initiated at 15 mg weekly and gradually increased to 25 mg weekly. Despite this regimen, he remained symptomatic with disabling fatigue, arthralgias, myalgias, and night sweats. Also, laboratory abnormalities persisted, with leukocytosis (WBC count 20,000–30,000/mm), ESR 100 mm/hour, ferritin 1000 ng/mL, low albumin (2.8 g/dL), and elevated transaminases. The clinical course was complicated by the development of steroid side effects, particularly hypertension, weight gain, Cushingoid features, and bleeding gastric ulcer, felt to be a result of combined use of indomethacin and prednisone. At that point, it was concluded that the addition of another remittive agent to control disease activity and to reduce steroid requirement was indicated. Anakinra (Kineret; Amgen, Inc., Thousand Oaks, CA) 100 mg subcutaneously daily was initiated in March of 2002. Pretreatment serum level of IL-1was obtained. Within 1 month of anakinra treatment, we observed 330 Journal of CLINICAL RHEUMATOLOGY