Adult-onset Still's disease
Differential diagnosis in recurrent pharyngitis, fever of unknown original and excessive hyperferritinemia. Six weeks before admission a 43-year old previously healthy woman got right-sided pharyngitis, accompanied with Herpes labialis and oral candidiasis. Two weeks later she travelled to a holiday in the Caribbean. During the first week of holiday she developed pharyngitis again, this time accompanied with fever and arthralgies. Therapeutic trial with antibiotics, antimalaric drugs and antiamoebics, given at the holiday resort, did not reduce fever. Immediately after return to Germany a therapeutic trial with imipenem was was attempted, without any apparent improvement. At admission, the patient was febrile, had pinky patchy exanthema and arthralgias. At admission, abnormal findings included anaemia (Hb 8.8 mg/dl), severe leukocytosis (35.3/nl), increased ESR (43/89 mm), CRP (24.2 mg/dl) and ferritin (5751 micrograms/l). Ultrasound examination revealed mild splenomegaly. Computed tomography of the abdomen and chest were without apparent abnormalities. Infection, autoimmune diseases and neoplasia were ruled out. The diagnosis of adult onset Still's disease was established on the basis of a typical triad of symptoms (fever, exanthema, arthritis). Treatment with 100 mg/d prednisolon (started intravenously) was beneficial for fever and arthralgia, and resulted in the normalisation of laboratory findings. After gradual reduction in the corticosteroid dosage, maintenance treatment with 20 mg/d prednisolon was continued over following months. Recurrent prodromal pharyngitis and excessive hyperferritinaemia are, in addition to the triad fever-exanthema-arthritis, further important diagnostic criteria of adult onset Still's disease.