emDocs - October 11, 2017 - Authors: Hadley N and Powell J
Edited by: Koyfman A and Long B
Background
Granulomatosis with Polyangiitis (GPA, formerly Wegener’s granulomatosis) is a rare vasculitis affecting both arterial and venous small vessels. Incidence is estimated at 12 cases per 1 million individuals per year. GPA, along with microscopic polyangiitis and Churg-Strauss syndrome, is a vasculitis associated with antineutrophil cytoplasmic antibodies. GPA, as the name indicates, is characterized by granulomatous formation of lesions in the upper and lower respiratory tract along with glomerular nephritis. Onset of disease is usually gradual over the course of 4-12 months. The disease affects men and women equally, has a Caucasian predominance, and almost never affects African American males. It most commonly occurs in the 4th and 5th decade, but the disease may occur at any age with 15% of patients diagnosed <19 years of age. Prior to development of effective treatment, GPA was universally fatal from a couple months to a year after diagnosis. GPA is also associated with a 7% annual risk for venous thromboembolism...
Summary
- Granulomatosis with polyangiitis is classically a triad of:
- Upper respiratory symptoms
- Lower respiratory symptoms
- Renal involvement
- Complaints are commonly non-specific so look for refractory upper and lower respiratory symptoms with or without renal involvement
- Most common in 4th and 5th decade but can occur at any age
- Respiratory collapse can occur quickly
- GPA associated with 7% annual risk for VTE
- Previous GPA frequently have relapses