Abstract
Background
Juvenile Systemic lupus erythematosus (JSLE) is a chronic multisystem autoimmune disease of childhood, which accounts for 10% to20% of systemic lupus erythematosus (SLE). It was initially thought that systemic lupus erythematosus (SLE), including JSLE, was rare in Blacks, this was eventually debunked with increasing reports from Africa. However, it is now known that SLE is more common among patients of African descent in western countries. While the estimated prevalence of JSLE in the developed countries is 0.36–2.5 per 100 000, data in Black Africans is scarce due to missed diagnosis, poor diagnostic capacity and under-reporting.
JSLE has protean manifestations similar to common paediatric conditions such as severe malaria, overwhelming septicaemia, hyper-haemolytic crisis in sickle cell anaemia etc., which often cause delayed or missed diagnosis. The objective is to describe the demographic and clinical characteristics, including outcome of children with JSLE, thus raising awareness on their occurrence and management in Nigerian children.
Methods
Retrospective review of records of children diagnosed with SLE at the Adult/paediatric Rheumatology Clinic and Paediatric Wards of Lagos State University Teaching Hospital (LASUTH) from May 2018 to May 2021.
Results
Twenty-two children, nineteen (n = 19) girls and three (n = 3) boys, aged 5–17 years, fulfilled the American College of Rheumatology (ACR)’s diagnostic criteria for JSLE out of 45 children newly diagnosed with paediatric rheumatic diseases during this period. The duration of symptoms before diagnosis ranged from two weeks to three years. The presentations included recurrent severe anaemia (n = 16), arthritis (n = 17), arthralgia (n = 17), malar rash (n = 17), neurologic symptoms (n = 5) oral ulcers (n = 17), cardiopulmonary symptoms (n = 5), photosensitivity (n = 10) and renal disease (n = 14). Laboratory findings included elevated ESR with a mean (±SD) of 99.68 ± 44.44, positive ANA (n = 22), positive anti-dsDNA (n = 12), low C3 & C4 (n = 2), positive anti-Smith antibody (n = 8) and massive proteinuria (n = 14). All patients were treated with steroids and disease modifying anti-rheumatic drugs (synthetics and biologics) based on disease severity and organ manifestations.
Sepsis (n = 4) was the most common preliminary diagnosis before a final diagnosis of JSLE was made, other preliminary diagnosis were pulmonary tuberculosis (n = 1), dermatitis (n = 1), acute glomerulonephritis (n = 1), Typhoid fever (n = 1), malaria (n = 1), deep vein thrombosis (n = 1), seizure disorder (n = 1), leukaemia (n = 1), meningitis (n = 1), meningoencephalitis (n = 1), hyper-haemolytic crisis in sickle cell anaemia (n = 1), Steven Johnson syndrome (n = 1), Juvenile Idiopathic Arthritis (n = 2), Eczema (n = 1), unexplained anaemia (n = 1) and acute rheumatic fever (n = 1).
One boy and three girls defaulted from clinic after commencement of treatment due to severe financial constraints of their parents and religious beliefs, however six girls died, four from an acute flare and two from end stage renal disease.
Conclusion
Our study has shown that JSLE has protean manifestations with a tendency to miss its diagnosis due to similarity of signs and symptoms with common childhood diseases in our environment. JSLE may not be as rare as commonly thought, thus its prompt diagnosis and treatment require a high index of clinical suspicion.
Therapeutic approaches for the treatment of new onset and flared juvenile systemic lupus erythematosus with active renal disease: an international multicenter PRINTO study
