Case Presentation: 61-year-old woman history of type 2 diabetes mellitus presented to medicine clinic with complaint of rash on her face and legs. Exam revealed an erythematous rash over both ear lobes, arms, and extensor surface of both knees. There was no mucocutaneous involvement and no palpable purpura. Drug reaction was suspected, therefore tramadol and atorvastatin were discontinued. Patient, however, then represented to PMD six days later with ongoing rash now with fevers, and bilateral knee and shoulder pain. She was not able to elevate either arm above shoulder level. She was admitted to the hospital for further evaluation. On admission the patient was febrile to 104, tachycardic to the 130s in atrial fibrillation. Labs were notable for leukocytosis to 16 and marked elevation of inflammatory markers. She was admitted to the intensive care unit, and treated with broad spectrum antibiotics without clinical improvement. Blood and urine cultures were negative. CK level within normal limits. Vasculitis and autoimmune work up including ANA, complement levels, hepatitis serologies and cryoglobulins were also negative. She underwent bone marrow biopsy which did not show evidence of hematologic malignancy nor hemophagocytic lymphohistiocytosis. Clinical concern for Adult Onset Still’s Disease raised, and serum ferritin was markedly elevated greater than 7500. She was started on systemic steroids and methotrexate with clinical improvement.
Discussion: The pathophysiology of Adult Onset Still’s Disease (ASD) remains unclear, however many interleukins and cytokines have been implicated. The clinical presentation of ASD may vary from primarily articular symptoms, such as arthritis and joint destruction, to systemic features including high grade fevers, serositis, hepatitis and reactive hemophagocytic lymphohistiocytosis. Diagnosis is based on the Yamaguchi Criteria which has been proven to be greater than 95% sensitive and specific. Once diagnosis of Adult Onset Stills Disease has been confirmed treatment should be initiated with prednisone. Studies have also demonstrated a role for methotrexate, as well as anti-interlukin-1, anti-interlukin-6, and anti-TNF-alpha for systemic disease. Our patient improved slowly on prednisone and methotrexate.
Conclusions: Adult Onset Still’s disease (ASD) is an inflammatory disorder typically characterized by fevers, arthritis, and truncal rash, associated with elevated ferritin levels. ASD can develop in older patients as well and should be considered in differential of the above symptoms. ASD is a diagnosis of exclusion. The Yamaguchi criteria is highly sensitive and specific for diagnosis.