News

A recent case report highlights the difficulties that can occur in diagnosing granulomatosis with polyangiitis (GPA), a form of ANCA-associated vasculitis, after a patient was initially diagnosed and treated for tuberculosis. The male patient had a mass in the mediastinum — the space between the lungs — initially attributed…

The National Organization for Rare Disorders (NORD) has updated its State Report Card to make it more digitally friendly and added telehealth to its categories of rare disease policy issues in a nod to its increased use during the ongoing COVID-19 pandemic. NORD’s report card project began seven…

Significantly high levels of the inflammatory protein pentraxin-3 (PTX3) were found in the bloodstream and urine of people with ANCA-associated vasculitis (AAV), particularly those with kidney involvement, a study revealed. Unlike C-reactive protein (CRP), a widely used inflammatory biomarker, PTX3 correlated with standard measures of AAV disease activity before…

Infliximab successfully led to the remission of respiratory symptoms in a 15-year-old boy with steroid-resistant granulomatosis with polyangiitis (GPA), a type of ANCA-associated vasculitis (AAV), according to a case report. More studies are needed to determine the treatment’s safety profile in children, the researchers noted. The report, “…

Antimicrobial webs produced by neutrophils — immune cells that respond quickly to bodily injury — may be implicated in the development of ANCA-associated vasculitis (AAV) accompanied by lung scarring (fibrosis), a recent review of several published studies revealed. These webs of DNA and antimicrobial proteins are known as neutrophil…

Stellate ganglion block, a procedure where a pain-relieving medicine is injected into the neck to block certain nerves, might be a successful therapeutic approach for eosinophilic granulomatosis with polyangiitis (EGPA), a recent case study suggests. The procedure helped control symptoms and lower corticosteroid doses, which are associated with detrimental…

Compared with standard glucocorticoid regimen, a reduced-dose regimen may lower the risk of death and serious infections, while not increasing the risk of kidney failure, in people with ANCA-associated vasculitis (AAV), according to a review study. No major differences between regimens were detected in terms of disease remission, relapse,…

Adding plasma exchange to standard treatment reduces the risk of kidney failure after one year, but does not improve survival in people with ANCA-associated vasculitis (AAV), a review study reports. Plasma exchange was also associated with an increased risk of serious infections — a previously unrecognized effect that may explain…

Healthcare providers involved in diagnosing and treating rare diseases believe that increased physician education and collaboration with specialized facilities will have the greatest positive impact on treating these conditions over the next five years, according to results from a 2021 survey. Definitive Healthcare, a healthcare commercial intelligence company, conducted…

The body’s complement system — comprised of blood proteins that fight infection — is specifically activated in individuals with microscopic polyangiitis and ANCA-associated vasculitis (AAV) with myeloperoxidase (MPO) antibodies years before a patient’s symptoms begin, new research shows. “These findings support differential pathogenic [disease-causing] mechanisms behind” these two AAV…