Epidemiology and outcome of articular complications in adult onset still’s disease

Abstract

The adult onset Still's disease is a rare inflammatory pathology of unknown pathogeny. The clinical  features are variable. The diagnosis is difficult since exclusion of infectious, systemic and tumoral  pathologies should be done. The articular complications are frequent and can be revelatory of this  pathology. The articular prognosis depends on the diagnosis delay and the treatment efficiency. Our study aims to analyze different aspects of articular manifestations complicating adult onset Still disease to define epidemiological, clinical and evolving characteristics of these complications. It was a cross- sectional study concerning 18 cases of adult onset Still disease diagnosed from 1990 to 2014 in the  internal medicine A department of Charles Nicolle Hospital in Tunis, meeting Yamaguchi criteria. We identified clinical, radiological, evolving and therapeutic profile of the articular manifestations occurred in these patients. There were 11 women and 7 men. The average age was 27 years. The arthralgias were  reported in all cases; while, the arthritis interested thirteen patients. A hand deformation was found in  four patients. A wrist ankylosis was noted in one case and a flexion elbow in one patient. The Standard articular radiographs were normal in ten cases. The treatment associated essentially non-steroidal anti-inflammatory and/or corticosteroids and/or methotrexate. Concerning the evolving profile, the  monocyclic form was present in 25% of the cases, the intermittent form in 40% and the chronic articular form in 35% of our patients. The adult onset Still's disease is rare and heterogeneous. The articular disturbances are frequent and have various outcomes.

Key words: Adult onset still´s disease, arthritis, fever, corticosteroids, biotherapy, prognosis