IgA vasculitis (Henoch – Schönlein Purpura) as the first manifestation of juvenile Systemic Lupus Erythematosus: Case-control study and systematic review

Abstract

BackgroundWe have recognized 15 children with jSLE and the antecedent of IgA vasculitis (HSP). This association is not broadly present in the literature.AimTo know the age and gender distribution of children with IgA vasculitis (HSP), compare it to our IgA vasculitis (HSP) + jSLE cases, and identify prognostic factors to develop jSLE within our case series, IgA vasculitis (HSP) vs. IgA vasculitis (HSP) + jSLE.MethodsA systematic review was carried out to know the age and gender distribution of children with IgA vasculitis (HSP). The information obtained plus data from 110 children with IgA vasculitis (HSP) from the Instituto Nacional de Pediatría were used to compare groups and identify prognostic factors. We performed a case-control study in patients < 18 years, consisting of 15 cases retrospectively identified with IgA vasculitis (HSP) + jSLE, and 110 IgA vasculitis (HSP) control subjects.ResultsThe information of 12,819 IgA vasculitis (HSP) subjects from the systematic review and 110 IgA vasculitis (HSP) controls was obtained and compared to our 15 IgA vasculitis (HSP) + jSLE cases. The mean age of IgA vasculitis (HSP) was 7.1-years vs. 10.4-years of IgA vasculitis (HSP) + jSLE at the HSP diagnosis. Female to male ratio of IgA vasculitis (HSP) was 1:1.33 vs. 1:0.25 of IgA vasculitis (HSP) + jSLE. Patients with IgA vasculitis (HSP) + jSLE had lower levels of Hemoglobin (Hb) compared to patients with IgA vasculitis (HSP) 109 g/L vs. 141 g/L. For the development of jSLE, we found older age and lower levels of Hb as prognostic factors with OR [95% CI]: 1.37 [1.06, 1.89] and 5.39 [2.69, 15.25], respectively.ConclusionIgA vasculitis (HSP) + jSLE patients are older and have lower levels of Hb than patients with IgA vasculitis (HSP). It is necessary to confirm these findings through a prospective study.

Análisis del gen Fas como molécula causante del lupus eritematoso sistémico en pacientes con vasculitis IgA (púrpura de Henoch-Schönlein)

“…The researchers collected data from 110 children with HSP who attended the Instituto Nacional de Pediatría. 18 Based on the information obtained from the systematic review, the investigators found that 12,819 subjects with HSP and 110 controls were exposed to particular risk factors, including a childhood age of approximately 10 years, female sex, and anemia. The study identified older age and lower hemoglobin levels as prognostic factors, with respective odds ratios of 1.37 [1.06, 1.89] and 5.39 [2.69, 15.25]; however, they could not determine the molecular point of union between both diseases.…”

“…The study identified older age and lower hemoglobin levels as prognostic factors, with respective odds ratios of 1.37 [1.06, 1.89] and 5.39 [2.69, 15.25]; however, they could not determine the molecular point of union between both diseases. 18 In this study based on case-control analysis, the primary article under review emphasized the necessity of conducting experimental research to elucidate the association between the two diseases beyond their clinical interconnection. Nonetheless, the article asserts that a molecular insufficiency in the apoptotic clearance pathway is plausibly a pathophysiological occurrence for which we aim to furnish a response, given the extant literature.…”

“…Nonetheless, the article asserts that a molecular insufficiency in the apoptotic clearance pathway is plausibly a pathophysiological occurrence for which we aim to furnish a response, given the extant literature. 18…”

“…Throughout the duration of this study, the cases were assessed by the respective Departments of Rheumatology and Immunology; however, the precise number of rheumatologists who confirmed both diagnoses remains undisclosed. 18 Apoptosis, a biological process of cell death, is characterized by three stages: stimuli, pathways, and effector mechanisms that execute the death program. [13][14][15] The apoptotic process being deficient in autoimmune diseases like SLE and HSP causes the perpetuation of the lymphoproliferative syndrome and the inability of the organism to eliminate it.…”

Nefropatía por IgA en una adolescente con lupus eritematoso sistémico juvenil. Reporte de caso

“…La Vasculitis por IgA (anteriormente denominada Púrpura de Henoch-Schonlein) representa la etiología más común de vasculitis sistémica en la (7) población infantil y raramente se asocia con LES . En un estudio de Glomerulopatías en el Paraguay, se reportaron 1072 biopsias renales, con 1072 (75.5%) glomerulopatías y, a diferencia de la mayoría de los p a í s e s , s e o b s e r v ó u n p r e d o m i n i o d e glomerulonefritis secundarias (GNS) (57.3%), de las cuales 85.9% fueron nefritis lúpica (NL), y entre las glomerulonefritis primarias (GNP), la Nefropatía (8) por IgA (NIgA) fue rara (1.9%) .…”