Rare diseases

IgAV childhood patients have long-term risk of infections

A WA study of children presenting to hospital with IgA vasculitis has found that their severe infection rates are raised for decades compared to other children.

The infection rate – including sepsis, pneumonia, throat and skin infections – was 2.51/1,000 months for children with vasculitis compared to 1.13/1,000 for other children during 20 years of follow up.

On the question of whether infections may trigger the condition, the study found that URTI and throat infections were the only infections occurring at higher rates in IgAV patients in a two-year period prior to diagnosis

The study, led by Professor Johannes Nossent from the Sir Charles Gairdner Hospital and the University of Western Australia, said steroid use may confound the relationship between infections and IgA vasculitis (IgAV).

“In spite of this, we demonstrate that IgAV patients remain a higher risk for further infections for at least twenty years post diagnosis,” the study authors said.

“Treatment for IgA vasculitis is usually very short term because it is considered self limiting but the fact that the immune response has been altered in producing a vasculitis indicates there has been antigenic change … that seems to lead to a continued deficiency in clearing, especially, respiratory infections for which IgA is the major player,” Professor Nossent told the limbic.

He said immunosuppression for other conditions such as giant cell arteritis was often for several years or more. However in all types of vasculitis, having better biomarkers of response could help optimise treatment and potentially minimise steroid use.

“Most of them [patients with vasculitis] are difficult to treat because we have very few specific biomarkers to see how they are going. We use non-specific inflammatory markers to see if there is an increase in inflammation in the blood vessels. We use PET scans to look at the larger vessels but PET scans have only limited resolution so we can’t look at the smaller vessels.”

“We are doing the best we can with the limited options in terms of seeing how active the inflammation in the blood vessels is.”

He said alternative treatments would also be difficult to identify until there was a better understanding of the conditions, for example how IgA molecules and mucosal structures are altered in IGA vasculitis.

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