ABSTRACT
INTRODUCTION: Familial Mediterranean fever (FMF) is an autosomal recessive disease. It is characterized by recurrent crises of fever and serosal inflammation. Although FMF patients are symptom free in between attacks, subclinical inflammation continues during the attack-free period. Such patients with inflammatory status have an increased risk of atherosclerotic cardiovascular complications. We attempted to elucidate the role of arterial wall thickening as a predictor of early atherosclerosis in children affected by FMF and to clarify the links between carotid intima media thickness and the markers of subclinical inflammation serum amyloid A (SAA), erythrocyte sedimentation rate (ESR), neutrophil-to-lymphocyte ratio (NLR) and platelet lymphocyte ratio (PLR). MATERIAL AND METHODS: It is a case control study. The study comprised 45 Egyptian children diagnosed with FMF and 45 healthy children of matched age and sex who served as controls, without family history or clinical manifestations suggestive of FMF. Laboratory investigations included complete blood count, NLR, PLR, ESR, C-reactive protein and lipid profile. Serum amyloid A levels were determined in both groups using enzyme linked immunosorbent assay. Assessment of the common carotid artery intima media thickness (CIMT) in the FMF patients was carried out. RESULTS: The level of SAA was significantly higher in patients than the control subjects with a mean value of 38.30 ng/ml and 23.43 ng/ml respectively (p < 0.001). Our patients showed significantly higher PLR when compared to controls (p < 0.001). The mean right and left carotid intima media thickness in patient and control groups showed a highly significant difference (p = 0.005 and 0.036 respectively). CONCLUSIONS: The mean carotid intima media thickness is higher in cases than the control group. Hence carotid intima media thickness may be used as a tool in the prediction of any atherosclerotic burden in those children.
ABSTRACT
Lupus nephritis has been described as the most serious complication of systemic lupus erythematosus (SLE) and the strongest predictor of poor outcome. While the incidence of childhood SLE is relatively low, renal involvement appears to be more common and more severe in childhood SLE. This study aims to characterize the features and outcome of renal involvement in childhood-onset SLE based on a study of 100 Egyptian patients (mean age at diagnosis 10.1 years, range 2-17 years). Initial data regarding disease manifestations and biopsy findings were reviewed. Disease activity was assessed using SLEDAI scores. Follow-up data (mean duration 6 years) were noted regarding specific treatment, response, complications and renal survival. Initial renal involvement was present in 78 patients, including 66 with hypertension and 23 with renal impairment. Pathologically, class IV nephropathy was found in 18 patients, class V in 9 and low-grade lesions (class II-III) in 49. Twenty patients required follow-up biopsy, and all transformations were observed. SLEDAI scores significantly decreased from initial (mean ± SD) of 21.4 ± 7.3 to 13.4 ± 7.8, in association with response to therapy (P < 0.0001). Poor response was associated with initial hypertension and renal impairment but not with initial SLEDAI score or pathological class. The projected renal survival was 82.4 and 64.7% 5 and 10 years from diagnosis. Early renal involvement in childhood SLE is common, serious and requires proper evaluation and management.
