Adult onset Still's disease (AOSD) is a rare systemic inflammatory disease of unknown etiology and pathogenesis that presents in 5 to 10% of patients as fever of unknown origin (FUO) accompanied by systemic manifestations. We report an interesting case of a 33-year-old African-American male who presented with one-month duration of FUO along with skin rash, sore throat, and arthralgia. After extensive workup, potential differential diagnoses were ruled out and the patient was diagnosed with AOSD based on the Yamaguchi criteria. The case history, incidence, pathogenesis, clinical manifestations, differential diagnoses, diagnostic workup, treatment modalities, and prognosis of AOSD are discussed in this case report.

The systemic vasculitides are rare conditions characterized by inflammation and necrosis of blood vessels. Those with predominately small vessel involvement, such as the ANCA-associated vasculitides (AAV), are the only ones to target the lungs with any regularity. However, these conditions can affect almost any organ system, including the cardiovascular system. Obtaining an accurate diagnosis is crucial before committing a patient to long-term immunosuppressive therapy. The choice of treatment is guided by the organ systems involved, the severity of disease, and the presence of comorbid conditions. Newer treatments are emerging which provide more options in the therapeutic armamentarium.

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