Rare Case of GPA/EGPA Overlap Syndrome Highlights Importance of Early Diagnosis

Early identification of granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA) overlap is critical for prognosis, long-term treatment, and management of these two distinct diseases, a case report shows. Although few instances of this rare event have been reported, clinicians should be aware of this possibility…

Patients with ANCA-associated vasculitis (AAV) carrying specific variants of the glucocorticoid receptor (GR) gene NR3C1 have a greater risk of death and of developing end-stage renal disease, according to a large study. Also, patients with a variant of the gene coding for an enzyme that regulates glucocorticoids —…

InflaRx will soon launch a Phase 2 clinical trial in Europe to assess the safety and effectiveness of IFX-1 — an investigational inhibitor of the complement system — in patients with ANCA-associated vasculitis (AAV). Now that European authorities have approved it, the randomized, placebo-controlled Phase 2 trial is expected to recruit approximately…

Azathioprine an oral immunosuppressant used as a maintenance therapy for ANCA-associated vasculitis (AAV) patients — may in rare cases cause a hypersensitive reaction of tender, red bumps on the skin, called Sweet’s syndrome, a case report shows. The report, “Sweet syndrome: a rare feature of ANCA-associated vasculitis or unusual…

Cash-strapped governments across the 28-member European Union are struggling to control runaway healthcare expenditures — at exactly the same time as the promise of new but expensive therapies to treat rare diseases has never been greater. That’s the paradox faced by pharmaceutical companies as well as patient advocacy groups in…

Hydralazine, a medication commonly used to lower blood pressure, may in rare cases cause ANCA-associated vasculitis (AAV), a new case report shows. The study, “Hydralazine-induced MPO-ANCA Renal-Limited Vasculitis Masquerading as Lupus Nephritis,” was published in the Journal of the…

Elevated levels of the CD64 protein on the surface of neutrophils – a type of immune cell – accurately distinguishes bacterial infection from disease flares in patients with ANCA-associated vasculitis (AAV) and systemic lupus erythematosus (SLE), a study has found. The study, “Utility of neutrophil…

In rare cases, ANCA-associated vasculitis (AAV) presents as an interstitial lung disease with a pattern of usual interstitial pneumonia several years before the clinical onset of vasculitis, a case report suggests. The report, “Interstitial lung disease with usual interstitial pneumonia pattern preceding the presentation of ANCA-associated vasculitis…

The occurrence of a severe infection during the first month of care is a strong predictor of one-year mortality among patients 65 and older with ANCA-associated renal vasculitis — a form of kidney inflammation caused by ANCA antibodies — a retrospective study reports. The study, “Early predictors of one-year…

Disease status at three and six months seems to predict long-term survival and kidney failure in patients with ANCA-associated vasculitis (AAV), a study shows. These findings suggest that early disease control could be used as a surrogate endpoint, or objective, for AAV clinical trials, potentially shortening their duration. The study, “…