Wegener’s Granulomatous (WG) now known as Granulomatous Polyangiitis (GPA) is an anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). GPA is a complex disease that can affect multiple organ systems. There are high morbidity and mortality associated with GPA if left untreated or undertreated. GPA is characterized by a multitude of signs and symptoms such as necrotizing granulomas of the upper and lower respiratory tracts, renal system, and small blood vessels. It is associated with myeloperoxidase (MPO) ANCA and proteinase 3 (PR3) ANCA however; PR3 is more sensitive and specific to GPA. PR3 does not explicitly diagnose or distinguish GPA alone; however; data shows it can predict the severity of the disease and or relapse probabilities. Thirty years ago, the life expectancy for a patient with GPA was one year or less. Improvement of disease outcomes improved with the addition of Corticosteroids, Cyclophosphamide, and other conventional disease modifying anti-rheumatic drugs (DMARDs).
"In Adults with Granulomatous Polyangiitis is Systemic Corticosteroid’s More Effective than Cyclophosphamide in Reducing Disease Activity?,"
Lynchburg Journal of Medical Science: Vol. 1
, Article 61.
Available at: https://digitalshowcase.lynchburg.edu/dmscjournal/vol1/iss4/61
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