Adult-onset Still's disease (AOSD) is an uncommon inflammatory condition characterised by high fever, leukocytosis with neutrophilia, arthralgia and skin rash. Diagnosis is often clinical, and it is always necessary to exclude common disease mimics such as infections, malignancies and other rheumatic diseases. Ferritin and glycosylated ferritin are useful adjuncts to aid in the diagnosis of AOSD. Important complications to consider include fulminant hepatitis, disseminated intravascular coagulopathy and hemophagocytic syndrome. Mainstay treatment of AOSD is often with glucocorticoids. Other agents that can also be used include methotrexate, and biologic therapy, such as tumor necrosis factor-α (TNF-α) blockers and interleukin-1 (IL-1) inhibitors. A retrospective review of patients diagnosed with AOSD admitted to Singapore General Hospital (SGH) was performed, and their disease characteristics and laboratory findings were compared with that of previous published cohorts.
Systemic lupus erythematosus (SLE) is a chronic inflammatory disease with a relapsing and remitting course, which is often treatable with immunosuppressive therapy. However, we describe a patient with severe and refractory thrombotic thrombocytopaenic purpura (TTP) secondary to SLE that did not respond to high dose corticosteroids, cyclophosphamide (CyC), vincristine, rituximab, and plasmapheresis. She eventually required haematopoietic stem cell transplantation (HSCT) to bring the disease under control. She is in disease remission three years post-stem cell transplantation. HSCT is a possible treatment option for severe and refractory SLE when conventional therapy has failed. HSCT for other rheumatologic diseases is also reviewed.
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