Serum interleukin-33 (IL-33) in children with active systemic lupus erythematosus: A cross-sectional study.

Interleukin-33 (IL-33) is a member of the IL-1 cytokine family and is associated with the development of different autoimmune diseases as systemic lupus erythematosus (SLE). So, the purpose of this cross-sectional study was to measure the serum IL-33 in children with SLE (c-SLE) in relation to their SLE disease activity index. This study was conducted upon 50 c-SLE patients in comparison to 50 normal matched children as a control group. Disease activity was assessed according to SLE Disease Activity Index (SLEDAI-2K). Serum IL-33 was measured by an Enzyme-linked immunosorbent assay (ELISA). Serum IL-33 was significantly higher in c-SLE patients (median: 157.47, IQR:64.49-237.57ng/l) than controls (median: 10.9, IQR: 10.04-12.51ng/L) (P= 0.001) and negatively correlated with serum C3 and C4 levels. Serum IL-33 levels were significantly higher in high disease activity status (HDAS) patients (SLEDAI-2K ≥ 10) (298.47 ± 78.84ng/l) than lupus low disease activity status (LLDAS) patients (SLEDAI-2K < 10) (112.18 ± 16.23ng/l) (P= 0.001). The receiver operating characteristic (ROC) curve analysis revealed that the best cutoff of serum IL-33 level to predict the disease activity was ≥141.3 ng/l with a sensitivity of 93%, a specificity of 90% and accuracy 97%. We concluded that serum IL-33 was higher in c-SLE patients and positively related to the disease activity index so could be used as a disease activity marker.

Hemolytic Uremic Syndrome in Egyptian Children: A Single Center Experience across 10 Years.

Hemolytic uremic syndrome (HUS) is a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI) and is the most common cause of AKI in children. We aimed to demonstrate the clinical patterns, laboratory findings, management, and outcomes of HUS in Egyptian children. This was a retrospective cohort study carried out in the Nephrology Unit of the Pediatric Department at Tanta University Hospitals. Hospital-based records of HUS cases between January 2009 and January 2019 were used to obtain the disease history, clinical manifestations, investigations, treatment, and outcomes. Sixty-eight children were included in the study: 63 (96.56%) with Shiga-toxin-producing Escherichia coli (STEC) HUS and five (7.53%) with atypical HUS. The boy-to-girl ratio was 1.19:1. The age at the onset of the disease ranged from 0.5 to 13 years, with a median of 2.25 years. The main presenting manifestations were pallor (80.88%), diarrhea (67.65%), oliguria (54.41%), and convulsions (19.21%). The survival rate was 85.29%, whereas the mortality rate was 14.71%. Thirty-seven patients (54.41%) recovered completely, 17 (25%) patients survived but with chronic kidney disease, and four patients (5.88%) progressed to end-stage renal disease and are currently maintained on dialysis. The risk factors for mortality were female gender, age <5 years, anuria, and an affected central nervous system (CNS). STEC-HUS had a higher incidence than atypical HUS with better outcomes. Early dialysis improved the outcome in terms of mortality in young patients, females, and those with an affected CNS.

Is podocytopathy another image of renal affection in p-SLE?

BACKGROUND: Lupus podocytopathy (LP) is a renal affection described in systemic lupus erythematosus (SLE) patients with nephrotic range proteinuria, characterized by diffuse foot process effacement without immune deposits and glomerular proliferation. This study describes LP, its pathological features and outcomes of pediatric (p-SLE) patients in comparison to the usual lupus nephritis (LN) cases. METHODOLOGY: A retrospective cohort study conducted on a 10-year registration (2010-2019) of 140 p-SLE patients at the Pediatric Department, Tanta University. Histopathological analysis with light microscopy (LM) and immunofluorescence (IF) of all renal biopsies were evaluated according to the International Society of Nephrology Renal Pathology Society (ISN/RPS) grading system. In addition, some biopsies were examined with electron microscopy (EM). RESULTS: Eighty-six p-SLE cases (61.4%) had renal involvement; seventy-nine biopsies (91.86%) of them met the classification criteria of LN as defined by ISN/RPS system. Five biopsies were normal (MCD) and two showed focal segmental sclerosis (FSGN) that did not meet any known classification of LN. Hence, they were reevaluated using EM that revealed diffuse effaced podocytes without glomerular sub-epithelial, endocapillary or basement membrane immune deposits, and were classified as having lupus podocytopathy, representing (8.14%) of all LN biopsies. Those seven cases showed good response to steroids with a complete remission duration of 3.40 ± 1.95 weeks. However, some case had 1-3 relapses during the duration of follow up. CONCLUSIONS: LP is a spectrum of p-SLE, not an association as it is related to disease activity and its initial presentation.