AB0557 THE SPECTRUM OF PEDIATRIC LUPUS: DATA FROM THE KENYA PEDIATRIC RHEUMATOLOGY (KAPRI) REGISTRY

Abstract

BackgroundChildhood-onset systemic lupus erythematosus (cSLE) is a prototype autoimmune condition characterized by systemic organ involvement, high morbidity and mortality (1-5). A pediatric rheumatology registry is critical in defining the spectrum of pediatric lupus within the region 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.ObjectivesOur 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.Table 1.Clinico-epidemiological features of paediatric Lupus patientsPatientGenderAgeEnd Organ InvolvementAutoantibody profileTreatmentCurrent Clinical Status1Female14 yearsArthritisANA +ve Antiphospholipid antibody +ve (B2 glycoprotein IgA)Declined treatment of Hydroxychloroquine and Methotrexate to seek 2nd opinionLost to follow up2.Female14 years 9 monthsSkinANA +ve Anti ds DNA +veHydroxychloroquineLost to follow up3.Female11yearsArthritis Interstitial Lung DiseaseANA+ve >1:640 (fine granular) Anti Rib +ve Anti P protein +ve Anti SSA +veMycophenolate Mofetil Hydroxychloroquine Prednisone 1 dose of RituximabStable with minimally active disease4.Female15 years 2 monthsLupus GangreneANA +1:640 (Homogenpous) Anti Sm +ve Anti RNP +ve Anti ds DNA +ve Anti nucleosome+ve Anti ribosome +veCyclophosphamide Mycophenolate Mofetil AspirinLost to follow up5.Female8 years 7 monthsNeurolupus Lupus GangreneANA +VE 1:640(speckled) Anti Sm +ve Anti RNP +ve Anti Ribosome +veCyclophosphamide (6 courses) Rituximab (2 courses) Mycophenolate Mofetil Prednisone HydroxychloroquineMinimally Active Disease6.Female6 years 11 monthsArthritis Skin rash Class IV Lupus NephritisANA+VE (1:320 homogenous) Anti ds DNA +ve Anti nucleosome +veMycophenolate Mofetil Hydroxychloroquine PrednisoneMinimally Active Disease7.Female14 years 6 monthsClass II Lupus NephritisANA VE 1:320 (Homogenous)Mycophenolate Mofetil HydroxychloroquineRemissionConclusionOver 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.References[1]Brunner, H., Huggins J and Klein-Gitelman MS. Pediatric SLE—towards a comprehensive management plan Nat. Rev. Rheumatol. 7, 225–233 (2011).[2]Kamphuis, S. and Silverman, E. D. Prevalence and burden of pediatric-onset systemic lupus erythematosus Nat. Rev. Rheumatol. 6, 538–546 (2010).[3]Migowa A, Colmegna I, Hitchon C, Were E, Ng’ang’a E, Ngwiri T, et al. The spectrum of rheumatic in-patient diagnoses at a pediatric hospital in Kenya. Pediatric Rheumatology (2017)[4]Trindade, V.C., Carneiro-Sampaio, M., Bonfa, E. et al. An Update on the Management of Childhood-Onset Systemic Lupus Erythematosus. Pediatr Drugs 23, 331–347 (2021).[5]Scott C, Webb K. Pediatric rheumatology in sub-Saharan Africa. Rheumatology (Oxford). 2014;53(8):1357–8.Disclosure of InterestsNone declared