Persistence and Severity of Cutaneous Manifestations in IgA Vasculitis Is Associated with Development of IgA Vasculitis Nephritis in Children

Abstract

<b><i>Background/Objective:</i></b> The purpose of this study was to evaluate the prevalence and severity of skin involvement in children with IgA vasculitis (IgAV) and its relationship with clinical and biochemical parameters and the risk of developing IgA vasculitis nephritis (IgAVN), the only cause of long-term morbidity and the main prognostic factor in IgAV patients. <b><i>Methods:</i></b> This national multicenter retrospective study included 611 patients under the age of 18 years with IgAV referred to five Croatian tertiary hospitals between 2009 and 2019. Patient data were collected from a database with systematic analysis of IgAV patients in the Croatian population. <b><i>Results:</i></b> Among the 611 children, 205 (33.55%) had purpura on the lower extremities, in 207 (33.88%) the rash extended on the trunk, in 149 (24.39%) it extended to the upper extremities, in 32 (5.24%) the rash was generalized, while 15 (2.47%) had the most severe skin symptoms: bullae, ulcerations, and necroses. IgAVN developed in 130 (21.28%) and persistent IgAVN (present for >3 months) in 48 (7.86%) children. Multivariate logistic regression found that presence of ulcerations and necroses (OR 3.20 [95% CI 1.03–9.91]), persistent purpura (OR 2.89 [95% CI 1.71–4.88]), and higher age (OR 1.16 [95% CI 1.09–1.23]) were significant predictors of IgAVN, whereas persistent purpura (OR 20.11 [95% CI 1.09–372.52]), male sex (OR 3.32 [95% CI 1.13–9.80]), and higher age (OR 1.15 [95% CI 1.00–1.30]) were predictors of persistent IgAVN. Among the laboratory parameters, higher serum urea (OR 1.43 [95% CI 1.03–2.00]) and reduction in activated partial thromboplastin time (OR 0.83 [95% CI 0.74–0.93]) were shown to have a significant impact on increasing the risk of persistent IgAVN. <b><i>Conclusion:</i></b> With increasing severity and duration of cutaneous manifestations in IgAV, the risk of developing IgAVN increases, making the prognosis worse, with a greater likelihood to need more aggressive treatment.

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“…Such skin alterations developed in 2-3% of patients with IgAV and recently it was shown that with increasing severity and duration of cutaneous manifestations in IgAV, the risk of developing IgAVN increases, making the prognosis worse with a greater likelihood to have required more aggressive treatment. 32 Furthermore, it was found that the presence of ulcerations and necroses, persistent purpura (more than 1 month) and age were significant predictors of IgAVN, while persistent purpura, male gender and age were predictors of persistent IgAVN (hematuria and/or proteinuria more than 3 months). 32 Musculoskeletal manifestations are the second most common feature and up to 70-90% of patients will have arthralgia or arthritis.…”

“…32 Furthermore, it was found that the presence of ulcerations and necroses, persistent purpura (more than 1 month) and age were significant predictors of IgAVN, while persistent purpura, male gender and age were predictors of persistent IgAVN (hematuria and/or proteinuria more than 3 months). 32 Musculoskeletal manifestations are the second most common feature and up to 70-90% of patients will have arthralgia or arthritis. 31 The affected joints have painful ranges of movement due to periarticular swelling, but the characteristic findings of warmth, erythema and effusion are often absent.…”

“…It has not yet been defined whether the follow-up time should be extended for some groups of patients, ie, older children with the onset of gastrointestinal symptoms before other IgAV symptoms and severe GI form of IgAV, as well as those who develop ulcerations and necroses and persistent purpura, since they may be at higher risk for the later development of nephritis. 32,33 There are different suggestions for monitoring patients. According to one strategy, the initial urinalysis should be repeated three times.…”

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“…It is proposed to follow-up patients with IgAV for at least 6–12 months even if the initial blood pressure measurements and urinalysis are normal ( 74 ), measuring regularly blood pressure and performing urinalyses to detect presence of haematuria, and quantification of albuminuria and/or proteinuria. Bearing in mind that recently published research has indicated that a certain group of patients (such as older children with the onset of gastrointestinal symptoms before other IgAV symptoms and severe GI form of IgAV, as well as those who develop ulcerations and necroses and persistent purpura) may be at higher risk for the later development of nephritis, the question arises whether some children should be monitored longer than recommended ( 54 , 59 ).…”

“…Furthermore, hemorrhagic bullae, ulcerations and necrotic lesions can be seen in the most severe cases. Recently, several papers have been published on severe skin changes in patients with IgAV, questioning whether these changes are associated with more severe disease, persistent sequelae, and discussing how to treat patients with such manifestations (52)(53)(54). The results of these studies are not uniform, and according to some authors the presence of ulcerations and necroses, persistent purpura (≥1 month) and older age were significant predictors of IgAVN.…”