IgA vasculitis (IgAV) is the most common childhood-vasculitis in which more than 50% of children develop gastrointestinal (GI) symptoms. In 10-20% of patients serious complications such as intussusception, bowel perforation, and massive bleeding may occur. The most important complication is the development of nephritis with progression to chronic renal failure in about 3% of children. The aim of the research was to analyze clinical and biochemical parameters in patients with IgAV and GI manifestations.
This retrospective study included children with IgAV reviewed in five Croatian University Centers for pediatric rheumatology in the period 2009 to 2019.
Out of 611 children with IgAV, 320 were males and 291 were females. The overall GI symptoms prevalence was 45.9% and the median (range) age at diagnosis was 6.42 (4.5-8.83) years. Among patients with GI symptoms there were 1.44 times more males (N=166) than females (N=115), which was statistically significant (p=0.003). Patients with GI symptoms had less infections before the appearance of purpura (59.8% vs. 70.9%, p=0.005) and were found to be significantly more likely to have rash distributed on the trunk (61.9% vs. 48.5%, p=0.001), and upper extremities (35.2% vs. 24.7%, p=0.006), as well as generalized rash (38.8% vs. 28.3%, p=0.008). These patients also had significantly higher values of C-reactive protein, leukocyte count, erythrocytes and platelets, hemoglobin, hematocrit and D-dimer concentrations and lower levels of IgG and IgM. In our cohort 42 out of 281 children (14.9%) had the most severe GI manifestations (intussusception and/or massive GI bleeding) with significantly higher values of 24-hour urine protein levels and D-dimer concentrations and lower total serum protein, albumin, IgG, IgM and C3 levels in comparison with children whose GI manifestations were not severe. Predictors of severe GI involvement were: relapse of the disease, generalized rash, rash extended on upper extremities, rash extended to the face, recurrent rash and renal involvement, as well as lower values of prothrombin time, fibrinogen and IgM among the laboratory parameters. Patients with GI symptoms were 1.68 times more likely to develop nephritis, and this probability was 2.58 times higher if GI symptoms occurred before other symptoms. Other predictors of nephritis were: severe and moderate GI manifestations, recurrent rash, one or more relapses of IgAV, and older age.
Older children with IgAV and severe GI manifestations in whom IgAV begun with GI symptoms had a higher risk of acute and chronic complications of the disease.
SUPPORT: Croatian Science Foundation project IP-2019-04-8822
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