Some Patients with Hydralazine-related AAV Develop Advanced Kidney Disease, Study Finds

Some patients with ANCA-associated vasculitis (AAV) caused by hydralazine — a medication used to treat high blood pressure — develop end-stage kidney disease, even after discontinuing the medicine and receiving immunosuppressive therapy, a small study found. The risk for hydralazine-induced AAV appears to be higher among African-Americans, according to the Johns Hopkins…

Isotretinoin, a medication used to treat severe acne, may in rare instances lead to ANCA-associated vasculitis (AAV), according to the case report of a 15-year-old boy. The study, “Perinuclear antineutrophil cytoplasmic antibody-positive vasculitis, oligoarthritis, tendinitis, and myositis associated with isotretinoin in a 15-year-old boy: Case report and review…

Low levels of two immune system proteins increase the risk that a person who has just been diagnosed with ANCA-associated vasculitis will die or have a serious kidney problem, a study shows. The findings on the complement proteins C3 and C4 applied to two forms of ANCA-associated vasculitis — granulomatosis with polyangiitis (GPA) and…

Patients with ANCA-associated vasculitis (AAV) have fewer adverse events and better quality of life when treated with avacopan over standard therapy, results from two Phase 2 clinical trials show. ChemoCentryx, which is developing the therapy, presented the findings at the 2018 Spring Clinical Meetings of the National Kidney…

Compared to patients with inflammatory bowel disorders, the safety and efficacy of azathioprine treatment in ANCA-associated vasculitis (AAV) patients is not affected by lower TPMT protein activity, researchers found. The study, “Thiopurine methyltransferase genotype and activity cannot predict outcomes of azathioprine maintenance therapy for antineutrophil cytoplasmic antibody…

A little-known government entity within the National Institutes of Health (NIH) is helping to lead U.S. efforts to speed up the development of therapies for some 7,000 rare diseases. The Office of Rare Diseases Research (ORDR), headquartered in Bethesda, Maryland, was established in 1993 within the NIH Office of the…

A faulty immune system component in patients with active ANCA-associated vasculitis (AAV) is not equipped to stop ANCA-induced neutrophil activation, leading to kidney damage, a Chinese study shows. The component is called complement factor H. The study, titled “Complement Factor H Inhibits Anti-Neutrophil Cytoplasmic Autoantibody-Induced Neutrophil Activation by…

The more white blood cell precursors that an ANCA-associated vasculitis (AAV) patient has when diagnosed, the worse their disease, a South Korean study reports. Levels of the precursors, known as immature granulocytes, can help doctors identify which patients with two forms of AAV are likely to have relapses, the researchers…

The U.S. Food and Drug Administration has approved two automated diagnostic tests that AESKU.Group developed for ANCA-associated vasculitis. AESKU used its HELIOS technology to develop the tests. HELIOS is the only diagnostic tool capable of both processing and analyzing a patient’s blood sample via an immunofluorescence assay (IFA) in a single run, the…

ANCA-associated vasculitis (AAV) may be associated with rare mutations in the alpha-1 antitrypsin (AAT) protein, a new case report suggests. The study, “Fulminant hepatic failure in the setting of progressive ANCA-associated vasculitis associated with a rare alpha-1 antitrypsin phenotype, ‘PiEE‘,” was published in the journal BMJ…