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, “…
News
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…
Patient registries are a hot topic of rare disease research and many organizations are taking advantage of this resource by signing up their patient communities and connecting with researchers. Eric Sid, MD, program officer for the Office of Rare Diseases Research (ORDR), said it is difficult to estimate how…
A team of scientists in Europe has developed a novel mouse model of the type of kidney damage that commonly occurs in ANCA-associated vasculitis (AAV), and identified the receptor CXCR4 as a potential target for future therapies. “This model may be a considerable benefit to research aimed at ameliorating…
Horizon Therapeutics has launched its #RAREis Representation program aimed at increasing diversity, equity, and inclusion among patients with rare diseases. There are about 400 million people worldwide living with a rare disease; for many of them, access to diagnosis, care, and treatments can be challenging. Accessing better care depends on…
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