Abstract

ObjectiveTo characterize scrotal involvement in children and adolescents with IgA vasculitis.MethodsA cross-sectional retrospective study included 296 IgA vasculitis (EULAR/PRINTO/PRES criteria) patients, 150/296 (51%) were males and assessed by demographic/clinical/laboratory and treatments. Scrotal involvement was defined by the presence of scrotal edema and/or pain/tenderness in physical examination and/or testicular Doppler ultrasound abnormalities.ResultsScrotal involvement was observed in 28/150 (19%) IgA vasculitis patients. This complication was evidenced at IgA vasculitis diagnosis in 27/28 (96%). Acute recurrent scrotal involvement was observed in 2/150 (1%) and none had chronic subtype. Further analysis of patients with scrotal involvement at first episode (n = 27) compared to those without this complication (n = 122) revealed that the median age at diagnosis [4.0 (2.0–12) vs. 6 (1.3–13) years, p = 0.249] was similar in both groups. The frequency of elevated serum IgA was significantly lower in IgA vasculitis patients with scrotal involvement versus without this manifestation (18% vs. 57%, p = 0.017), whereas glucocorticoid (93% vs. 49%, p < 0.0001) and ranitidine use (63% vs. 30%, p = 0.003) were significantly higher in the former group.ConclusionsThe scrotal involvement occurred in almost one fifth of IgA vasculitis patients and was commonly evidenced as acute subtype at diagnosis. Scrotal signs/symptoms improved after a prompt use of glucocorticoid and was associated with low frequency of elevated IgA serum levels.

Highlights

  • Immunoglobulin A (IgA) vasculitis previously known as Henoch-Schönlein purpura (HSP), is the most frequent systemic vasculitis of small vessels with IgA dominant immune complexes deposits [1,2,3,4,5,6,7]

  • Data of scrotal involvement in IgA vasculitis patients are limited due to the small representation of this complication in previous case reports or case series [1, 3, 5, 8,9,10,11,12] precluding an accurate analysis of associated factors and outcomes in patients with and without this complication, using the European League Against Rheumatism (EULAR)/Paediatric Rheumatology International Trials Organisation (PRINTO)/PRESS IgA vasculitis classification criteria [9]

  • None of the patients had scrotal edema as the first sign/symptom of IgA vasculitis. This complication was evidenced at IgA vasculitis diagnosis in 27/28 (96%)

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Summary

Introduction

Immunoglobulin A (IgA) vasculitis previously known as Henoch-Schönlein purpura (HSP), is the most frequent systemic vasculitis of small vessels with IgA dominant immune complexes deposits [1,2,3,4,5,6,7]. In 2010, validated classification criteria for IgA vasculitis were established and proposed by European League Against Rheumatism (EULAR), Paediatric Rheumatology International Trials Organisation (PRINTO) and Paediatric Rheumatology European Society (PRES) [13]. Data of scrotal involvement in IgA vasculitis patients are limited due to the small representation of this complication in previous case reports or case series [1, 3, 5, 8,9,10,11,12] precluding an accurate analysis of associated factors and outcomes in patients with and without this complication, using the EULAR/PRINTO/PRESS IgA vasculitis classification criteria [9]

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