Organ Involvement in EGPA Patients Impacted by ANCA Status, Review Finds

The presence or absence of ANCA antibodies appears to influence which organs are affected by eosinophilic granulomatosis with polyangiitis (EGPA), a rarer subtype of ANCA-associated vasculitis, a recent review of studies suggests. While EGPA patients carrying these antibodies will more likely develop nervous, renal, and skin manifestations…

Raremark, an online rare disease patient community, has launched a digital platform called Xperiome, aimed at streamlining the search for new medicines for rare disorders and incorporating more patient input into research. The goal is to help the pharmaceutical industry deliver innovative new therapies faster and smarter, the…

The EveryLife Foundation for Rare Diseases has launched a scholarship fund in the U.S. to support individuals with rare disorders who are pursuing personal goals through training and education. The initial phase of the five-year, $1-million #RAREis Scholarship Fund will include 32 scholarships — each totaling $5,000 —…

Extracellular vesicles, or the tiny membrane-bound particles that are released from cells, could be used as a blood biomarker of disease activity in people with ANCA-associated vasculitis (AAV), a new study suggests. The study, “Microparticles expressing myeloperoxidase as potential biomarkers in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides…

The complement system, a part of the immune system, is overactive in people with active ANCA-associated vasculitis (AAV), but achieving remission appears to lower most of its components to normal levels, a study reports. The study, “The alternative complement pathway in ANCA‐associated vasculitis: further evidence…

When the COVID-19 pandemic forced the postponement of a rare disease film festival originally slated for May, its organizers set out to find a new way to bring the films to an audience.  Co-founders Daniel DeFabio and Bo Bigelow, who are both fathers of children with…

Plasma exchange does not prolong the survival of patients with severe ANCA-associated vasculitis connected with diffuse alveolar hemorrhage, a potentially life-threatening condition in which blood starts to build up in the lungs, a study has found. The results challenge the notion that plasma exchange therapy might improve…

The presence of detectable ANCA antibodies and B-cells after initial treatment with rituximab — used to induce remission in people with ANCA-associated vasculitis (AAV) — predicts a greater risk of relapse, a new study shows. The study, “Proteinase-3-anti-neutrophil cytoplasmic antibodies (PR3-ANCAs) predict relapses…

The complement system, a component of the immune system, is overactive in people with necrotizing glomerulonephritis, or inflammation in the tiny blood vessels of the kidneys, caused by ANCA-associated vasculitis (AAV), a study reports. Some complement proteins seem to correlate with more severe kidney disease in these…

Fingolimod (FTY720), a medication used to treat multiple sclerosis, also appears to ease many of the symptoms of ANCA-associated vasculitis (AAV), a new study in rats suggests. The study, “Sphingosine-1-phosphate receptor modulator FTY720 attenuates experimental myeloperoxidase-ANCA vasculitis in a T cell-dependent manner,” was published…