RESUMEN
Adult-onset Still's disease (AOSD) causes fever, rash, pharyngalgia, and arthralgia through autoinflammation. Its complement titer has not previously received attention because this usually increases during the inflammatory process. Our female patient in her 60s was admitted to the hospital with fever, rash, arthralgia, and pharyngalgia. Her white blood cell count was 19,130/µL, hemoglobin was 11.0 g/dL, platelet count was 26.0 × 104/µL, and ferritin titer was 6,175 ng/mL. Anti-nuclear antibodies and anti-neutrophil cytoplasmic antibodies were negative. The presence of infectious diseases and malignancies was excluded. She was diagnosed with hypocomplementemia at the onset of AOSD because of her low complement component 4 (C4) titer (<5.0 mg/dL). Her complement component 3 (C3) titer was 104.5 mg/dL, which was within normal limits. There was no sign of thrombotic microangiopathy (TMA) or hemophagocytosis. She was treated with high-dose corticosteroids, including pulse methylprednisolone therapy, cyclosporine, methotrexate, and intravenous immunoglobulin, but was resistant to these, and her disease repeatedly flared up. Treatment with intravenous cyclophosphamide eventually led to remission. Post-treatment, her C4 titer increased to within the normal range. Although hypocomplementemia with TMA or hemophagocytosis has been reported in AOSD patients, our patient showed no sign of either at disease onset. Hypocomplementemia of AOSD may be a sign of high disease activity and could be a predictive marker for resistance to standard therapy.
RESUMEN
BACKGROUND: In recent years, advances in pharmacotherapy for rheumatoid arthritis have dramatically improved the control of disease activity. However, a significant number of patients still develop hand deformity and require surgical reconstruction. The objective of this study was to evaluate the long-term efficacy and drawbacks of the Swanson metacarpophalangeal joint arthroplasty for patients with rheumatoid arthritis over 10 years. METHODS: Clinical and radiological evaluations were performed for 87 joints of 29 hands in 27 patients who underwent metacarpophalangeal joint arthroplasty using the Swanson implant, and who were followed up for an average of 11.4 (10-14) years. RESULTS: The number of operated tender and swollen metacarpophalangeal joints decreased from 24 (27.6%) and 28 (32.2%) to 1 (1.1%) and 2 (2.3%), respectively. The patients' general health and disease activity score 28-erythrocyte sedimentation rate improved at the last survey. Mild recurrence of ulnar drift was observed, but the deformity was generally well-corrected. Implant fracture was noted in eight joints (9.2%), and revision surgery was performed in two joints (2.3%). The average active range of extension/flexion changed from -46.3°/65.9° to -32.3°/56.6°. While a significant change was not noted in grip or pinch strength, patients were satisfied with the operation especially in terms of pain relief and improved hand appearance. CONCLUSIONS: The long-term results of Swanson metacarpophalangeal joint arthroplasty were good in pain relief and correction of deformity, but some problems remain with regard to implant durability and mobility.