50 The spectrum of pediatric lupus: data from the Kenya Pediatric Rheumatology Registry (KAPRI)

Abstract

BackgroundPediatric rheumatic diseases are chronic illnesses that impart a significant disease burden upon children and their families (1–3). Furthermore, they have the potential to cause physical disability, diminished quality of life and significant direct and indirect costs (1–3). Determination of the burden and clinical characteristics of pediatric lupus is a critical first step to improving access to care and optimizing use of existing health systems for the well-being of these patients (4). Childhood-onset systemic lupus erythematosus (cSLE) is a prototype autoimmune condition characterized by simultaneous or sequential organ and system involvement, with unpredictable flare and high levels of morbidity and mortality (5). A pediatric rheumatology registry is critical in defining the spectrum of pediatric lupus within the region, the clinical characteristics of the disease, the outcomes and responses to various interventions. The Kenya Pediatric Rheumatology Registry (KAPRI) registry offers a unique opportunity to pioneer and spearhead a systematic and organized format in collecting pertinent clinical information that will offer information on current clinical scenarios and offer a platform to conduct other research projects to improve pediatric lupus healthcare in sub-Sahara Africa. Our objective was to determine the clinic-epidemiological profile of paediatric lupus patients by describing their baseline patient characteristics and clinical features at the Aga Khan University Medical College East Africa who were enrolled into the KAPRI registry from inception in March 2019 to December 2021.MethodsAll patient records were selected from the KAPRI registry database using the ICD 10 code M32 that denotes Systemic Lupus Erythematosus (SLE) Age, gender, laboratory, clinical features at diagnosis, treatment options offered at time of diagnosis were extracted from the database.ResultsAmong the 207 patients enrolled thus far in the registry, 7 had a diagnosis of cSLE (3.4%). The commonest symptom among the patients were joint pain, fever and rash. Other clinical features and outcomes are highlighted in the table 1 below;ConclusionOver a 2-year period, 57% of our cSLE patients (4 of 7) achieved remission while 43% (3of 7) were lost to follow up. Further studies are required to elucidate predictors of good clinical response and reasons for loss to follow up among our cSLE patients.