Adult Onset Still’s Disease in Tropical Area: Illustration of Diagnostic and Therapeutic Difficulties from 3 Senegaleses Observations

Abstract

Introduction : The Adult Onset Still’s Disease (ASD) is a systemic auto-inflammatory affection of unknown cause seldom described in sub-Saharan Africa. We report 3 observations of ASD illustrating the diagnostic and therapeutic difficulties of this affection in our areas. Observation 1: Our first patient is a 56 years old schoolteacher presenting an ASD in its chronic articular form. She had been followed for an inflammatory arthralgia for 10 years and of the pharyngal pains without exact diagnosis. She presented ASD’s criteria of Yamaguchi and of Fautrel. The prednisone was begun at the dose of 0.8 mg/kg/day with fast appearance of a progressive muscular weakness. Use of methotrexate at a rate of 15 mg per week, associated with low dose of prednisone was effective in long-term without any flare of the disease so far. Observation 2: Our second patient is a 30 years old dressmaker presenting an ASD in its complicated systemic form of lymphohistiocytic activation syndrome. She validated the criteria of Yamaguchi and Fautrel for ASD. She also presented resistance to corticosteroid therapy. The evolution was marked by a hospital-acquired septicemia and a multi-organ failure leading to death. The diagnosis was retrospectively confirmed after that, with the low level of the glycosylated ferritin serum value. Observation 3: The third patient is a 22 years old Guinean student who presented prolonged fever and inflammatory polyarthralgia without articular deformation. He had been misdiagnosed for ASD with diagnostic wandering of several months. He was treated successfully with prednisone after set up of ASD diagnosis according common criteria. Corticosteroid therapy was stopped after 8 months without any relapse noted so far. Conclusion: Caring for ASD is difficult in our context mainly because of high cost of several explorations needed to set up its exact diagnosis while making differential one. Evolution under corticosteroid therapy is usually favorable but diagnostic delay may lead to severe complications and death.