GPA (Granulomatosis With Polyangiitis) Flashcards
What is the triad of GPA?
- VASCULITIS
- GRANULOMAS (necrotizing, granulomatous inflammation of upper/lower repiratory tracts)
- GN
What is the serologic marker of GPA?
ANCA
GPA Is more common in what gender?
MALE
What is the peak incidence of GPA?
4th-6th decade
What is the most common form of crescentic glomerulonephritis in older adults?
PAUCI-IMMUNE RPGN
(including GPA and MPA)
What is the classic histopathologic finding in GPA?
FOCAL SEGMENTAL NECROTIZING & CRESCENTIC GN
True vasculitis in GPA involves what size of renal arteries?
SMALL & MEDIUM-SIZED
What 3 types of vessels is affected in true vasculitis in GPA?
- RENAL ARTERIES (small &medium-sized)
- VEINS
- CAPILLARIES
C-ANCA is directed against?
PR3
P-ANCA is directed against?
MPO
What is the specificity of C-ANCA for GPA?
98-99%
What 3 medications have been associated with ANCA, usually anti-MPO, and at very high titers?
- PTU
- HYDRALAZINE
- MINOCYCLINE
What drug is associated with unique ANCA anti-MPO and anti-PR3 positivity, often with high titers of ANCA in association with positive ANAs, APL antibody titer, and other serologic tests?
LEVIMASOLE
A rise in this titer correlated with relapse in the patients with renal disease but only weakly in those without kidney disease
ANCA
a rise in this titer is associated with severe relapses, especially in those with renal disease and pulmonary hemorrhage
PR3 ANCA