Abstract
Background
Pediatric Systemic Lupus Erythematosus (pSLE) is a chronic mutisystemic autoimmune disease with complex clinical manifestations whose diagnosis is not always easy and the course is generally severe and the treatment is not very well codified and often extrapolated from that of adults. This study aims to describe the clinical, immunological, therapeutic characteristics and short outcome of systemic lupus erythematosus in Algerian children.
Methods
This was a prospective, multicentre and descriptive study 36 months (January 2015 - December 2018) at the department of Pediatrics of University Hospital Nefissa Hamoud ex Parnet Algiers. Children less than16 years of age fulfilling the American College of Rheumatology SLE criteria were included. Disease activity estimated by Systemic Lupus Erythematosus Disease Activity index (SLEDAI) whose use has been validated in children and damage index based on Systemic Lupus International Collaborating Clinics (SLICC) score were determined.
Results
Eighty-three (83) patients were studied. Female: male ratio was1:49. Mean ages at lupus onset and diagnosis were respectively: 10, 12 ± 3, 88 and 11, 3 ± 3, 62 years. All patients had skin involvement while constitutional signs including fever and asthenia were observed in (98.8%). Rheumatological, renal, neuropsychiatric, cardiac, hepato-digestive, pleuropulmonary and ocular disorders were observed respectively: 65, 1%, 44, 6%, 41%, 27, 7%, 41%, 19, 3% and 7, 2%.
All patients were positive for antinuclear antibodies. Anti-double-stranded DNA (75%) was the most frequently observed autoantibody profile.
Antiphospholipid antibody positivity was noted in 52% whereas hypocomplementemia in fractions C3, C4 was observed in 55% and 56% respectively.
In our study, the severe forms were more frequent (83%) than the mild ones (17%) with a significant difference (P = < 10–6).
Overall, the mean SLEDAI at disease onset was 22.11 ± 11.87 with high activity ≥ 20 in 59% of cases. The mean damage score was 1.8 ± 2.045 (interquartile range 0–8).
Among induction drugs, oral corticosteroids were the most frequently used (92%), and in a third of cases intravenously at high doses in combination with immunosuppressive therapy.
In induction therapy, cyclophosphamide (CYC) was the most used drug (23%) compared with mycophenolate mofetil (MMF) (14%). Unlike the maintenance phase where MMF observed an increase (28%) vs (8%) CYC.
The use of MMF was correlated with severe lupus nephritis with a significantly effective difference in the decrease in SLEDAI (P = 0.0001). The use of hydroxychloroquine (HCQ) was observed in 81% in induction and 89% in maintenance treatment. The correlation of HCQ use with survival was significantly positive (P = 0.04). Indeed, adherence to treatments and essentially HCQ was a protective factor, its odds ratio is < 1 with a significant p-value, [OR 0.016 95% CI (0.001–0.353)].
Mortality was estimated at 11%. Multivariable regression analysis showed that the neurological involvement (odds ratio = 6,093 95% confidence interval ((1,1 8 0 ∼ 31 446)) and macrophage activation syndrome were associated with a high risk of mortality.
Conclusion
we report a series of pSLE characterized by great clinical and biological heterogeneity. It follows a severe course of the disease with high disease activity at the diagnosis and therefore leads to high morbidity and mortality. However, these results must be confirmed by other pediatric studies which could form the basis of a diagnostic and therapeutic approach more adapted for children.
Keywords
Algeria, Child, Clinical features, Disease activity, lupus
SCARF1-induced efferocytosis plays an immunomodulatory role in humans, and autoantibodies targeting SCARF1 are produced in patients with systemic lupus erythematosus
