A 29‐year‐old white woman with a history of fever spikes for 1 month presented with complaints of fever, poor appetite, arthralgia, and an erythematous rash on her lower extremities. She had been seen in the emergency room twice in the past week. Her initial presentation with fever and sore throat was diagnosed as pharyngitis, and she was treated as an outpatient with penicillin VK. Two days later she presented with difficulty swallowing and a fever of 103°F. This time she was admitted to the hospital with a diagnosis of epiglottitis. ACT scan of the neck, however, was unremarkable. She was discharged home after treatment with intravenous antibiotics and Decadron. Several days later she returned with the above‐mentioned symptoms and was readmitted to the hospital with a diagnosis of fever of unknown origin. Her physical exam was significant for fever (101.3°F) and a maculopapular rash on the lower extremities. A CT scan showed hepatosplenomegaly and fatty liver. She also had leukocytosis (19,000/μL) with a high absolute neutrophil count (11,687 cells/μL), normocytic normochromic anemia, high ferritin (3030 ng/mL), and a high erythrocyte sedimentation rate (61 mm/h), and urine analysis was positive for blood. Blood and urine cultures obtained were negative; chest x‐ray was normal. A comprehensive autoimmune and infectious disease workup was negative. The rheumatology service was consulted to evaluate the possibility of an autoimmune condition. Adiagnosis of adult‐onset Still's disease was considered based on the presence of major and minor criteria. A biopsy of the rash also showed neutrophilic vasculitis. The patient improved significantly on prednisone and Naprosyn and was discharged home.
The challenge with adult Still's disease is diagnostic, not therapeutic. It is an extremely rare disease, with an incidence of 0.16 cases per 100,000 persons per year. These patients are often misdiagnosed with and treated for sepsis, without any clinical improvement on antibiotics. There is no definitive diagnostic test available; hence, classification criteria are helpful. Major criteria are fever of at least 39°C, arthralgias/arthritis for more than 2 weeks, maculopapular rash, and leukocytosis (10,000/μL or greater). Minor criteria are negative ANA and RF, sore throat, lymphadenopathy, hepatosplenomegaly, and abnormal results of liverfunction tests. The presence of 4 major or 3 major and 2 minor criteria has a sensitivity of 81% and a specificity of 99%. Infection, malignancy, and other rheumatologic diseases should be ruled out before diagnosing adult Still's disease.
Adult Still's disease should be considered in a patient with fever, sore throat, maculopapular rash, arthralgias, and liver function tests showing elevated enzymes. Physicians need to have a high clinical suspicion of adult Still's disease in patients with persistent, nonspecific flulike symptoms in order to decrease the morbidity associated with this disease.
R. Gambhir, None; M. Parker, None; H. Wilson, None.
To cite this abstract:Gambhir R, Parker M, Wilson H. “Still” A Mystery!. Abstract published at Hospital Medicine 2007, May 23-25, Dallas, Texas Abstract 119. Journal of Hospital Medicine. 2007; 2 (suppl 2). https://www.shmabstracts.com/abstract/still-a-mystery/. Accessed January 28, 2020.