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Doctors should suspect granulomatosis with polyangiitis (GPA) in cases of isolated, acute sinusitis that does not improve with treatment, especially in young patients, a case report suggests. The study, “Very painful acute frontal sinusitis revealing granulomatosis with polyangiitis,” was published in the journal European Annals…

Researchers have developed a specific index for ANCA-associated vasculitis (AAV) that uses laboratory results at the time of diagnosis to help assess disease activity and predict death by any cause. The index could supplement currently-used indexes such as the Birmingham Vasculitis Activity Score (BVAS) to guide treatment course…

Rare diseases deeply affect not only the children who experience them, but also their healthy brothers and sisters, as their parents can attest.    Two entries in November’s “Disorder: The Rare Disease Film Festival” will focus on what siblings go through, according to the San Francisco festival’s co-founder,…

After examining 12 established methods for detecting antineutrophil cytoplasmic antibodies (ANCAs), researchers in China identified those likely best for diagnosing patients there suspected of having granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA). Their study, “Evaluation of 12 different assays for detecting ANCA in…

Developing gene therapies for rare diseases is one thing. Creating gene-edited “designer babies” is quite another. German legal expert Timo Minssen outlined the potentially explosive ethical landmines surrounding such issues during a recent talk at the New York Genome Center. Minssen directs the Center for Advanced Studies in…

Cocaine and levamisole, a chemical substance commonly used to cut the drug, can trigger ANCA production and mimic the symptoms of ANCA-associated vasculitis (AAV), researchers said. Most cases affect the skin and the nose, but they also can involve internal organs like the kidneys, a study including…

More people with ANCA-associated vasculitis (AAV), including those with kidney involvement, also have connective tissue diseases (CTD) than is generally thought, especially among female patients. But the co-existence of a CTD was unlikely to worsen kidney disease or change overall disease outcomes, a study reports. AAV patients with connective…

The autoimmune condition ANCA-associated vasculitis (AAV) needs improved classification systems that can better predict disease outcomes, a Spanish study says. Disease management should be based more on individual patient characteristics and levels of disease severity than on general classifications or ANCA specificities, the researchers suggest. The study, “…

Intravenous immunoglobulin (IVIG) therapy leads to a fast and significant reduction in disease activity, as well as in the levels of inflammatory biomarkers, in people with ANCA-associated vasculitis (AAV), a review of several studies found. The study, “The therapeutic efficacy of intravenous immunoglobulin in anti-neutrophilic cytoplasmic antibody-associated…

Imagine living your whole life with a painful disease so rare that only 25 others worldwide have what you have. And that you’re one of just six such people who’ve made it to adulthood. Neena Nizar doesn’t have to imagine. The 41-year-old English professor at Metro Community College in Elkhorn,…