New Index May Better Predict All-cause Mortality at AAV Diagnosis

A novel index that computes five simple laboratory results at the time of diagnosis may improve predictions of all-cause mortality among patients with ANCA-associated vasculitis (AAV), a South Korean study reports. These lab results include the levels of immune neutrophils, lymphocytes, and monocytes, and of proteins C-reactive protein and…

More than 600 people participated in the 10th annual Rare Disease Week on Capitol Hill 2021, held virtually July 14–22, to advocate for the rare disease community. Hosted by the EveryLife Foundation’s Rare Disease Legislative Advocates (RDLA) program, the event brings together community members from across the U.S. to…

Rituximab is safe and effective for inducing remission in two types of ANCA-associated vasculitis in children and adolescents: granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA), according to data from a Phase 2a trial. After one and a half years on the trial, all patients achieved remission, and safety…

In 2004, when I was a teenager who hadn’t yet been diagnosed with granulomatosis with polyangiitis, I suffered a severe onset that required a drug-induced coma for treatment. I’d only been awake a few days when I heard a cheerful voice outside my ICU room. “Would your patient like…

A reduced dosage of rituximab maintenance therapy may be effective at preventing relapses among people with ANCA-associated vasculitis (AAV), a new study reports. Specifically, the researchers said 500 mg infusions every six months “appear to be an effective and safe option” for maintaining AAV remission. “This study provides real-world…

Symptoms differ among people with the rare disorder eosinophilic granulomatosis with polyangiitis (EGPA), depending on whether they test positive or negative for antineutrophil cytoplasmic antibodies (ANCAs). A recent case study revealed that ANCA-positive patients tended to have more blisters, body-wide inflammatory symptoms, and to receive higher doses of oral…

Today is Lab Day. It’s been highlighted on my calendar for weeks, two words, stern and unavoidable. On the surface, it’s only a quick task: Show up, give samples, leave. But realistically, these visits demand a lot of my mental and physical energy due to the myriad little details. My…

A two-dose rituximab regimen is as safe and works as well as the standard four doses in inducing remission in adults with severe granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA), according to a review study. While these findings support the current use of either regimen in clinical practice…

The American College of Rheumatology and the Vasculitis Foundation have published new guidelines to improve the treatment and management of systemic vasculitis, a group of inflammatory disorders that includes ANCA-associated vasculitis (AAV). The rarity of these disorders can make them challenging to diagnose and treat. The new…

We were 600 feet above sea level as we traversed a dormant volcano rim in Hawaii. We followed a narrow ledge with nothing on the trail to guide us. My friend, a Honolulu resident and avid hiker, told me that if we got lost, the only safety recourse was to…