Adult onset Still's disease (AOSD) as a differential diagnosis of lymphadenitis colli with fever of unknown origin

Introduction Adult onset Still's disease is a rare autoinflammatory disease with an incidence of 5 per 100.000 and an equal ratio for men and women.

Case report: A 32-year-old female patient presented to our outpatient clinic. She reported a recurrent fever of 39°C since four days, a odynophagia, loss of appetite, persistent swelling of cervical lymph nodes on the right side as well as a new small-spotted, maculopapular exanthema. The ENT-examination as well as the laboratory findings were normal disregarding a slightly increased CRP. The patient was given an intravenous antibiotic(clindamycin 600mg 1-1-1) and a CT-Scan of the neck was performed which excluded an abscess. Symptoms persisted and additionally a painful swelling in the left ankle occurred. An ultrasound of the joints showed an effusion in the ankle joint with tendovaginitis. An advanced laboratory diagnostic showed an increasing CRP, a leukocytosis with neutrophilia, an increase of lactate dehydrogenase, an increase of liver parameters (AST, ALT and γ-GT), significantly increased ferritin, negative antinuclear antibodies as well as a normal C3 / C4 complement. Due to the above findings the patient was diagnosed AOSD. Therapy with the human interleukin 1 receptor antagonist anakinra was initiated. After four weeks the symptoms disappeared and since then the patient has been in complete remission.

Conclusion Autoinflammatory diseases like AOSD should be considered in case of recurrent or persisting fever for one week, exanthema and lymph node swelling especially with insufficient response to antibiotics when malignancis and other infections have been excluded.

Poster-PDF A-1204.pdf

Publication History

Article published online:
13 May 2021