E59 Deforming juvenile idiopathic arthritis: a case report in Bouake (Cote D’ivoire)

Abstract

Abstract Introduction Juvenile Idiopathic Arthritis (JIA) is a group of conditions characterized by chronic inflammatory joint disease of unknown aetiology before the age of 16. This joint damage is complicated by joint deformities, amplitude limitations, muscle imbalances causing a reduction in the functional capacities of the child. The delayed diagnosis, the inaccessibility of biotherapies give rise to deformations which hinder the development of the physical capacities and the social, educational and then professional integration of the child. Objective Sensitize practitioners about early diagnosis and multidisciplinary management of JIA, in order to avoid severe deformities. Observation A 17-year-old girl, out of school, was admitted to a rheumatology unit for the evolution of chronic polyarthralgia with transient skin eruption evolving by inflammatory attack with partial remission since the age of 8 years. There was no notable story. On physical examination, the affected joints were painful. Deformities were observed such as valgus of the knees and forefeet, ulnar deviation of the wrists and nonspecific of the fingers (Figs. 1 and 2). The biological inflammatory syndrome was present with (CRP 90 mg/l, VS 48 mm/h) and a blood count (Hb 12.5 g/dl, Plq 205 000/μL, GB 14. 109/l). Serum ferritin was elevated at 197.7 μg/ml. The other assessments in particular (blood cultures, FAN, FR, blood smears) came back negative. The diagnosis of AJI in systemic form was retained. The X-ray showed osteoarticular destruction with fixed exostoses, Larsen's stage 4 (Figs. 3 and 4) and Steinbrocker's stage 3 with minimal functional capacity. The treatment included an NSAID (ibuprofen at 40 mg/kg/day), followed by corticosteroid therapy (Prednisolone at a dose of 1 mg/kg/day) and as background treatment methotrexate (10 mg/week). evolution at 3 months was favorable on the pain. The management of the deformities was the subject of a multidisciplinary consultation meeting (physiotherapists, rheumatologists, orthopedic surgeons and paediatricians) very expensive for the parents. Thus it benefited a physical treatment made up of joint maintenance physiotherapy and equipment for joints that are still mobile. Discussion In Africa, osteoarticular pathology in children most often evokes diagnoses of sickle cell disease and acute articular rheumatism. From the first symptoms, children are referred to traditional healers, especially in rural areas where there is no specialist, then to pediatrics [1]. These reasons could explain the long delay in diagnosis as in the patient whose delay was 9 years. The diagnostic age of our patient was 17 years, higher than elsewhere [2] but consistent with the results of Diomandé in Abidjan with an average delay of 10 years [3]. Most studies find a female predominance with an age of onset between 4 and 8 years [4, 5]. In our patient, the involvement was polyarticular from the outset and the FRs were negative, as in most studies [1, 3]. The presence of these deformities was a source of significant handicap in our patient, which led to her dropping out of school, as stipulated in the literature. In first intention, we used NSAIDs, however unsatisfactory. Faced with the persistence of pain, she received prednisolone at 1 mg/kg/d favorable. As basic treatment she received methotrexate, biotherapies being inaccessible in our context because of their high cost. The management of deformities was the subject of a multidisciplinary consultation meeting (physiotherapists, rheumatologists, orthopedic surgeons and paediatricians) whose costs were beyond the reach of indigent parents. Conclusion The diagnosis and the multidisciplinary treatment must be early and continuous of the disease in order to avoid the deformations, which once installed are irreversible.