Epidemiological and clinical aspects of immunoglobulin A vasculitis in childhood: a retrospective cohort study

Abstract

Background A retrospective study was conducted in order to investigate and describe the characteristics of Immunoglobulin A vasculitis (IgAV), previously known as Henoch-Schӧnlein purpura, in the paediatric population of a community-based healthcare delivery system in the Italian region of Abruzzo. Methods This is a population-based retrospective chart review of the diagnosis of IgAV in children ages 0 to 18, admitted to the Department of Paediatrics of Chieti and Pescara between 1 January 2000 and 31 December 2016. All children enrolled presented with clinical symptoms and laboratory findings and met the EULAR/PRINTO/PRES 2008 criteria. Results Two-hundred-eight children met the criteria for IgAV, with the highest incidence reported among children below 7-years of age. A correlation with recent infections was found in 64% of the cohort; the onset was more frequently during the winter and fall. Purpura had a diffuse distribution in the majority of patients; joint impairment was the second most frequent symptom (43%), whereas the gastrointestinal tract was involved in 28% of patients. Conclusions Hereby, we confirm the relative benignity of IgAV in a cohort of Italian children; with regards to renal involvement, we report a better outcome compared to other studies. However, despite the low rate of renal disease, we observed a wide use of corticosteroids, especially for the treatment of persistent purpura.

Comparison of clinical characteristics and complication predictors of IgA vasculitis between pediatric and adult patients in China

“…IgAV often develops in autumn and winter [10]. It was found in our study that IgAV developed in autumn and winter in 61.4% of the patients.…”

Management of Henoch-Schonlein Purpura with Gastrointestinal Manifestations in Children

“…The etiology of HSP remains unknown, but genetic predisposition and environmental factors may impact pathogenesis of the disease. Some hypotheses suggest a connection between HSP and infection, 50% of patients with HSP have acute upper respiratory infections (AURI) with the common cause Group A beta-hemolytic streptococcus (GAS) found in 20-50% of patients with serologic culture or bacterial culture (2,11,13).…”

“…Henoch Schoenlein purpura or IgA vasculitis (IgAV) is the most common systemic vasculitis in children, characterized by inflammatory infiltration of polymonuclear leukocytes in small blood vessels, followed by predominance of IgA1 immune deposits (1,2). The inflammatory process that occurs in the small blood vessels in this disease causes an ischemia and/or hemorrhagic process, characterized by purpura without a decrease in platelet levels, arthralgia, gastrointestinal problems, and kidney impairment, so this process is called leukocytoclastic vasculitis (3).…”

The neutrophil: A key resourceful agent in immune‐mediated vasculitis

“…59 As IgA is produced in response to external stimuli, many bacteria and viruses as well as drugs are thought to act as triggers for IgAV 60 and a history of infection is reported in 30-63% of cases. [61][62][63] Although vaccination has been proposed as a trigger for IgAV, a recent study could not confirm this as a major etiological factor in childhood IgAV. 64 IgAV is characterized by IgA1 immune deposits and neutrophil infiltrates damaging the small vessels.…”

“…An association between interleukin (IL)‐1 receptor antagonist allele 2 (ILRN*2) and renal involvement has also been shown 59 . As IgA is produced in response to external stimuli, many bacteria and viruses as well as drugs are thought to act as triggers for IgAV 60 and a history of infection is reported in 30–63% of cases 61–63 . Although vaccination has been proposed as a trigger for IgAV, a recent study could not confirm this as a major etiological factor in childhood IgAV 64 …”