News

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…

Patients with ANCA-associated vasculitis carry a threefold higher risk for cardiovascular disease, and a eightfold greater risk for cerebrovascular accidents, compared to the general population, a long-term study shows. The study, “Risk of Cardiovascular Disease and Venous Thromboembolism Among Patients With Incident ANCA-Associated Vasculitis: A 20-Year Population-Based Cohort…

Patients with ANCA-associated vasculitis might develop acute thrombocytopenia — extremely low levels of platelets circulating in the blood — after being treated with Rituxan (rituximab), a case study from Japan reported for a first time. While rare, physicians should be aware of this potential side effect so as to detect…

Patients with ANCA-associated vasculitis (AAV) have a higher prevalence of thyroid disease than the general population, a single-center retrospective analysis shows. But the increase in thyroid disease is not related to the use of anti-thyroid medications, researchers said. The study, “Increased Prevalence of Thyroid Disease in…

Eosinophilic granulomatosis with polyangiitis (EGPA) may manifest as as a painful, progressive skin rash, and be associated with secondary cryoglobulinemia — a condition where abnormal proteins in the blood clump together at low temperatures — a new case report shows. While rare, it is critical that these conditions are recognized…

Chest manifestations and disease activity at diagnosis may help predict the risk of  infections during hospital stays in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), a retrospective study suggests. The South Korean study, “Birmingham vasculitis activity and chest manifestation at diagnosis can predict hospitalised infection in ANCA-associated vasculitis,”…