Link Exists Between EGPA and Clotting Disease in Small Blood Vessels, Report Suggests

In rare cases, eosinophilic granulomatosis with polyangiitis (EGPA) may serve as a trigger for thrombotic microangiopathy (TMA), a disease that affects the smallest blood vessels in the body and often involves blood clots, a case report suggests. It remains unclear why these two rare disorders may be interconnected. However, a common underlying…

The world’s biggest gathering of rare disease researchers, patient groups, pharmaceutical executives, and government officials is planned for April 10–12 in a Washington, D.C., suburb. Some 1,200 people have already registered to attend the World Orphan Drug Congress (WODC) USA 2019, set to take place at the Gaylord National Harbor…

Adding intravenous methylprednisolone pulses to standard treatment for inducing remission in severe cases of ANCA-associated vasculitis does not confer clinical benefits and increases the risk of infection and diabetes, a study suggests. The study, “Intravenous pulse methylprednisolone for induction of remission in severe ANCA associated Vasculitis: a multi-center retrospective…

ANCA-associated vasculitis (AAV) diagnoses increased and overall and renal survival improved over a period of 23 years, a real-world study in the U.K regions of Lancashire and South Cumbria reports. These improved outcomes may be due to earlier diagnosis and better management of AAV and chronic kidney disease in recent…

A new test measuring the relative abundance of immunoglobulin G4 (IgG4) RNA could be an indicator of granulomatosis with polyangiitis (GPA) activity and distinguish active disease from remission, a study suggests. The study, “IgG4:IgG RNA ratio differentiates active disease from remission in granulomatosis with polyangiitis: a…

About 100 scientists, researchers, pharmaceutical executives, and others will converge on Austria’s capital city early next month for the 2nd International Congress on Advanced Treatments in Rare Diseases. The March 4-5 meeting, to take place at the Hilton Am Stadtpark Vienna, features 27 speakers on a variety of disorders…

A significant proportion of patients who test positive for antibodies associated with ANCA-associated vasculitis (AAV) do not have the disease, according to a study. Its findings also suggested that a higher threshold of these antibodies could be used to improve diagnosis of the condition, since people with higher levels…

Pain and swelling in the testes, in rare cases, may be the first symptoms of ANCA-associated vasculitis, even among patients without other symptoms associated with this disease, researchers reported, noting that testicular vasculitis is often misdiagnosed. The study, “Testicular granulomatous vasculitis mimicking testicular torsion in an anti-neutrophil…

Long-term disease and treatment-related damage reduce the muscle strength and physical capacity of people with ANCA-associated vasculitis. The resulting reduction in physical activity diminishes the quality of life, a study suggests. The study, “Leg muscle strength is reduced and is associated with physical quality of life…

Abnormal antimicrobial traps found in inflammatory lesions from ANCA-associated vasculitis patients might be correlated with disease development, a study suggests. The study, “The formation and disordered degradation of neutrophil extracellular traps in necrotizing lesions of anti-neutrophil cytoplasmic antibody–associated vasculitis,” was published in The American Journal of Pathology.