－S 569 －
― S-437 ―
Refractory adult-onset Still's disease: Two case reports of rapid clinical response with anakinra
Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea
*Hyungjin Kim, Jaejoon Lee, Joong Kyong Ahn, Eun-Mi Koh, Hoon-Suk Cha
Background: Adult-onset still's disease (AOSD) is a rare systemic inflammatory disorder with variable clinical features. AOSD refractory to conventional treatment often poses challenges to clinicians. Anakinra, an IL-1 receptor antagonist, has been proposed as an alternative effective treatment modality in refractory AOSD. We report here, for the first time in Korea, two cases of refractory AOSD in which prompt clinical responses were achieved with Anakinra treatment. Cases: The first patient is a 29 year-old man with AOSD for 3 years. The patient presented initially with chronic migratory polyarthritis, evanescent salmon-colored skin rash with fever, and leukocytosis. Previous treatments with various immunosuppressive agents including methotrexate, leflunomide, cyclosporine, etanercept, and infliximab have failed in controlling his disease activity. A long-term corticosteroid therapy was required despite its suboptimal clinical efficacy. Just prior to the initiation of Anakinra treatment, WBC count was 12,850 x103/uL, erythrocyte sedimentation rate (ESR) was 52 mm/hr, and C-reactive protein (CRP) level was 10.56 mg/dL. Patient reported persistent arthritis with fever. The second patient is a 37 year-old man with AOSD for 2 years. The patient presented with spiking fever, skin rash, leukocytosis and elevated liver enzymes. Serum ferritin level was elevated at 16,500ng/mL. He had frequent clinical flares with fever, sore throat, myalgia and pleuritic chest pain despite treatment with methotrexate and etanercept. Repeated attempts to reduce the doses of corticosteroid were promptly followed by clinical flare-ups. Just prior to Anakinra treatment, WBC count was 13,990 x103/uL, ESR was 22 mm/hr, and CRP was 11.42 mg/dL. Treatment with Anakinra 100 mg/d subcutaneously was initiated in both cases.
A complete clinical remission, improvement in laboratory parameters including WBC count, ESR, and CRP were observed within a few days.
Steroid doses were tapered down without further clinical flare-ups. Conclusion: Anakinra appears to be an effective alternative treatment modality in patients with AOSD refractory to conventional DMARDs and corticosteroid therapy.
― S-438 ―
A case of adult onset Still’s disease with systemic inflammatory response syndrome complicated by fatal status epilepticus
Department of Internal Medicine, Yeungnam University College of Medicine, Daegu, Republic of Korea
*Yong Uk Jung, Myung Jin Oh, Dae Young Yun, Young Hoon Hong, Choong Ki Lee
Adult Onset Still’s Disease (AOSD) is characterized by quotidian or double quotidian spiking fevers with an evanescent rash, arthritis and multi-organ involvement. Cerebral involvement is only rarely described with AOSD; there is only one prior case of status epileticus with AOSD reported in the medical literature. Case: A 25-year-old woman had a spiking fever, sore throat, tender lymph nodes, a fine pink colored skin rash, arthralgia, myalgia. The laboratory tests showed a WBC 6,700 /μL with neutrophils 66%, lymphocytes 20% and atypical lymphocytes 3%; the hemoglobin was 10.8 g/dL, platelets 235,000 /μL, ESR 38 mm/H, CRP 2.19 mg/dL, AST 173 U/L, ALT 199 U/L, LDH 1,549 U/L, serum-ferritin 4,613.9 ng/mL. Urinalysis revealed no proteinuria, hematuria or pyuria. Serum protein electrophoresis showed a polyclonal gammopathy. The final blood culture report was negative. On the whole body integrated PET/CT, moderate 18F-fluorodeoxyglucose (18F-FDG) uptake was noted along the cervical lymphatic chain bilaterally at level IB and IIA. The patient had a lymph node biopsy, which showed reactive changes only. NSAID and prednisolone were prescribed for AOSD with SIRS. After four days of therapy, with mild confusion, the patient went into status epilepticus lasting several hours and died after cardiovascular collapse. Although it is unclear whether the CNS is directly correlated with underlying AOSD, clinicians should consider the possibility and follow patients closely for such complications to prevent devastating outcomes. Therefore we should think the patient with AOSD can have neurologic symptoms.
