title: Severe disease presentation and poor outcomes among pediatric systemic lupus erythematosus patients in South Africa.
abstract: Background Systemic lupus erythematosus (SLE) is a life-threatening multisystem autoimmune disease that is more severe in patients of African ancestry and children, yet pediatric SLE on the African continent has been understudied. This study describes a cohort of pediatric SLE (PULSE) patients in South Africa. Methods Patients with a diagnosis of SLE (1997 American College of Rheumatology criteria) diagnosed prior to age 19 years in Cape Town, South Africa, were enrolled in this cross-sectional study from September 2013 to December 2014. Information on clinical and serological characteristics was extracted from medical records. Results were compared to a well-described North American pediatric SLE cohort. Results Seventy-two South African patients were enrolled in the study; mean age 11.5 years; 82% were girls. The racial distribution was 68% Coloured, 24% Black, 5% White and 3% Asian/Indian. Most patients presented with severe lupus nephritis documented by renal biopsy (61%). Of patients with lupus nephritis, 63% presented with International Society of Nephrology/Renal Pathology Society class III or IV. Patients in the PULSE cohort were more likely to be treated with cyclophosphamide, methotrexate and azathioprine. The PULSE cohort had high disease activity at diagnosis (mean Systemic Lupus Erythematosus Disease Activity Index-2K (SLEDAI-2K) 20.6). The SLEDAI-2K at enrolment in the PULSE cohort (5.0) did not differ from the North American pediatric SLE cohort (4.8). Sixty-three per cent of the PULSE cohort had end organ damage with Systemic Lupus International Collaborating Clinics Damage Index (SLICC-DI) score >0 (mean SLICC-DI 1.9), compared to 23% in a previously reported US cohort. Within the PULSE cohort, nine (13%) developed end-stage renal disease with six (8%) requiring transplant, strikingly higher than North American peers (transplant rate <1%). Conclusions The PULSE cohort had highly active multiorgan disease at diagnosis and significant disease damage at enrolment in the South African registry. South African patients have severe lupus nephritis and poor renal outcomes compared to North American peers. Our study revealed a severe disease phenotype in the PULSE cohort resulting in poor outcomes in this high-risk population.
authors.affiliation: 1 Pediatric Rheumatology, Duke University Medical Center, USA.|2 Duke Hubert Yeargan Global Health, Duke University Medical Center, USA.|3 Paediatric Rheumatology, Red Cross War Memorial Children's Hospital and University of Cape Town, Cape Town, South Africa.|9 National Institute of Arthritis, Musculoskeletal, and Skin Diseases, NIH, USA.
authors.forename: L B
authors.initials: LB
authors.lastname: Lewandowski
authors.affiliation: 1 Pediatric Rheumatology, Duke University Medical Center, USA.
authors.forename: L E
authors.initials: LE
authors.lastname: Schanberg
authors.affiliation: 2 Duke Hubert Yeargan Global Health, Duke University Medical Center, USA.
authors.forename: N
authors.initials: N
authors.lastname: Thielman
authors.affiliation: 3 Paediatric Rheumatology, Red Cross War Memorial Children's Hospital and University of Cape Town, Cape Town, South Africa.
authors.forename: A
authors.initials: A
authors.lastname: Phuti
authors.affiliation: 4 Rheumatology, Groote Schuur and University of Cape Town, Cape Town, South Africa.
authors.forename: A A
authors.initials: AA
authors.lastname: Kalla
authors.affiliation: 5 Division of Nephrology and Hypertension, Groote Schuur Hospital and University of Cape Town, South Africa.
authors.forename: I
authors.initials: I
authors.lastname: Okpechi
authors.affiliation: 6 Paediatric Nephrology, Red Cross War Memorial Children's Hospital and University of Cape Town, Cape Town, South Africa.
authors.forename: P
authors.initials: P
authors.lastname: Nourse
authors.affiliation: 6 Paediatric Nephrology, Red Cross War Memorial Children's Hospital and University of Cape Town, Cape Town, South Africa.
authors.forename: P
authors.initials: P
authors.lastname: Gajjar
authors.affiliation: 7 Paediatric Rheumatology, Chris Hani Baragwanath Academic Hospital and Charlotte Maxeke Johannesburg Academic Hospital, Johannesburg, South Africa.
authors.forename: G
authors.initials: G
authors.lastname: Faller
authors.affiliation: 7 Paediatric Rheumatology, Chris Hani Baragwanath Academic Hospital and Charlotte Maxeke Johannesburg Academic Hospital, Johannesburg, South Africa.
authors.forename: P
authors.initials: P
authors.lastname: Ambaram
authors.affiliation: 8 Winelands Rheumatology Centre, Stellenbosch and Department of Medicine, Stellenbosch University, South Africa.
authors.forename: H
authors.initials: H
authors.lastname: Reuter
authors.affiliation: 3 Paediatric Rheumatology, Red Cross War Memorial Children's Hospital and University of Cape Town, Cape Town, South Africa.
authors.forename: G
authors.initials: G
authors.lastname: Spittal
authors.affiliation: 3 Paediatric Rheumatology, Red Cross War Memorial Children's Hospital and University of Cape Town, Cape Town, South Africa.
authors.forename: C
authors.initials: C
authors.lastname: Scott
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D015415:Biomarkers / Q000097:blood; D002648:Child; D003430:Cross-Sectional Studies; D005260:Female; D006304:Health Status; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000208:ethnology; D008181:Lupus Nephritis / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000208:ethnology; D008297:Male; D010641:Phenotype; D011379:Prognosis; D012042:Registries; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D013019:South Africa / Q000453:epidemiology; D013997:Time Factors; D014481:United States / Q000453:epidemiology
chemical_list: D015415:Biomarkers; D007166:Immunosuppressive Agents
keywords: Africa; Systemic lupus erythematosus; lupus nephritis; pediatric lupus