IgA vasculitis (Henoch \u2013 Sch\xf6nlein Purpura) as the first manifestation of juvenile Systemic Lupus Erythematosus: Case-control study and systematic review

Cecilia Solís-Galicia,Hayde Guadalupe Hernández-Huirache,Ana Luisa Rodríguez-Lozano,Miriam Martínez-Pérez,Francisco Eduardo Rivas-Larrauri,Esmeralda Nancy Jiménez-Polvo,Chiharu Murata,Laura Andrea Rincón-Arenas

doi:10.1186/s12887-019-1829-4

Cecilia Solís-Galicia, Hayde Guadalupe Hernández-Huirache + Show 6 more

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https://doi.org/10.1186/s12887-019-1829-4

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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.

Highlights

We have recognized 15 children with juvenile systemic lupus erythematosus (SLE) (jSLE) and the antecedent of IgA vasculitis (HSP)
Skin involvement is the mandatory criterion in patients with IgA vasculitis (HSP) [7]; this is a nonpathognomonic sign of the disease [8]
Systematic review A Systematic Review of the literature was conducted on PubMed from 1977 to 2016; we include the different terms for naming IgA vasculitis

Summary

Introduction

We have recognized 15 children with jSLE and the antecedent of IgA vasculitis (HSP). This association is not broadly present in the literature. Et al [3] reported a general incidence of 6.1 per 100,000 children/year, while in the age group between 4 and 7 years-old, the incidence is 70.3 per 100,000 children/year [2] It is frequently reported in the literature a distinction by group of age, one study including 120 children with IgA vasculitis (HSP) found that children under 10-years represented the 88.3% from the total [4], another study of 107 children, reported that 73% of them were ≤ 10-years [5] and, in a smaller study of 78 patients the percentage of subjects under 10-years reached 90% [6]. There are few reports in which IgA vasculitis (HSP) and juvenile SLE (jSLE) are somehow associated [13,14,15,16]

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