Abstract
Background
Juvenile Systemic Lupus Erythematous (JSLE) is a systemic autoimmune disorder with speckled manifestations that can emerge overstretched period of time and can affect any organ system, most frequently the skin, joints, kidneys, and the nervous, hematologic, and cardiovascular systems. The Aim is to examine the clinical features, serologic and laboratory characteristics associated with SLE. To probe and outline Clinical and Immunologic features of Incomplete Lupus Erythematous (ILE) patients who progressed from ILE to SLE.
Methods
The files of patients diagnosed as SLE in pediatric rheumatology clinic from 2001 to 5/2021 were retrospectively reviewed.
Result
Thirty SLE cases were included; Females were more prevalent with a female: male ratio of 14:1. Mean age at presentation 11 ± 4 years (range of 5 months-13 years), Disease onset was before sixth birthday in (7%), above twelve years in (40%) of the patients, and 53% of the patients was among 6 –12 years age group (31% & 37% of them fulfilled the SLICC & ACR criteria respectively at diagnosis) .The Mean duration between the onset of symptoms and SLE diagnosis was 6 months ±2 years. The Pre-pubertal age group presented early. At diagnosis, 50% of the patient got SLICC score criteria <4, on other hand 70% of the patients had ACR score criteria less than four Variable
The most common presenting feature was arthritis (83%) (polyarticular arthritis) followed by dermatological manifestations (46%) photosensitivity, malar rash, and discoid rash in order of most frequent, fatigability (37%), renal manifestation (23%) most commonly as hematuria, one case diagnosed as lupus nephritis by renal biopsy. ANA was positive in 87%, anti-ds-DNA positive in 40% and Anti sm positive in 17%. The most frequently used medications were steroids and hydroxychlorqiune, the most commonly used steroid sparing medications were azathioprine (43%), Mycophenolate mofetil (40%) and ciclosporin (13%), 26% were on antihypertensive, 3% required rituximab to control their disease and one patient received Eltromboag to treat refractory thrombocytopenia. the mean follow-up duration 46.9 ± 43.6m, 20% lost follow up, 13% died due to disease complications (renal system involvement, thrombocytopenia and neurological system involvements).
Conclusion
jSLE in Libya is very rare before the sixth birthday and presented early among 6–12 years age group with a delay of less than one year between the first presentation and time of diagnosis. SLICC criteria was sufficient to diagnose the disease in > 50% of patients. High index of suspension should be maintained because in some patient’s years may be passed before fulfilling the diagnostic criteria. The most common cause of death is renal involvement. Glucocorticoids are the backbone of jSLE treatment in the acute phase. Both azathioprine and Mycophenolate mofetil are sufficient to control the disease in most patients. The disease outcome is accepted in our cohort as most patients have mild disease activity with low dose steroid and a steroid-sparing agent.
Impacts of FcγRIIB and FcγRIIIA gene polymorphisms on systemic lupus erythematous disease activity index
