Challenges on diagnosis and treatment of refractory adult-onset still disease in Sub-Saharan Africa: a case report

Abstract

BACKGROUND: Adult-onset-Still's disease is a rare, multi-systemic condition with difficult and delayed diagnosis, characterized by a diversity of signs and symptoms related to hyperactivation of macrophages and neutrophils. Although diagnosis in the classical form is easily realized, there are atypical presentations that can be challenging, particularly in the absence of diagnostic means. CASE PRESENTATION: We report the case of a 28-year-old single African woman who had been experiencing prolonged fever and diffuse inflammatory joint pain for months, and who had manifested odynophagia and a fleeting, salmon-pink skin rash in her upper limbs, in whom, after thorough evaluation, the diagnosis of adult-onset-Still's disease was made. This was based on the various symptoms presented by the patient, a positive acute phase reactant with elevation of C-reactive protein, erythrocyte sedimentation rate, and high serum ferritin with a low percentage of glycosylated ferritin (< 20%), an absence autoimmunity markers and comorbidities, and confirmed by both Fautrel and Yamaguchi diagnostic criteria, within the month following admission after excluding all differential diagnoses. CONCLUSION: Adult-onset-Still's disease is a rare pathology considered as a diagnosis of exclusion, which should be considered in the face of any fever of unknown origin. It is present in young adults over 16 years old, with an average peak at diagnosis of 38-39 years old and a sex ratio of 2:1 (female:male), with a female predominance of the disease. Rapid diagnosis and appropriate management with steroids, biologics and immunosuppressants are the keys to a favorable prognosis for this condition.