Pulmonary Renal Syndromes Flashcards
Pulmonary-Renal Syndrome
development of diffuse alveolar hemorrhage and GN
2 main categories of Pulmonary-Renal Syndrome
- ANCA-associated Vasculitis (AAV)
2. Anti-GBM disease (Goodpasture)
3 ANCA associated Vasculitides that have pulmonary-renal involvement
- Granulomatosis with Polyangiitis (GPA aka The nazi)
- Eosinophilic Granulomatosis with Polyangiitis (EGPA aka Churg-Strauss)
- Microscopic Polyangiitis (MPA)
GPA characteristics
Necrotizing vasculitis with granuloma formation with NO asthma or eosinophilia
PR3-Ab +
C-ANCA
Cytoplasmic pattern on IF
EGPA characteristics
Necrotizing vasculitis with granulomas with asthma and eosinophilia
MPO Ab +
P-ANCA
Perinuclear pattern on IF
MPA characteristics
Necrotizing vasculitis without granulomas.
MPO-Ab +
P-ANCA
Perinuclear pattern on IF
Age group for presentation of most AAV
50-70 y/o
Pathogenesis of an AAV
Environmental or infectious trigger primes MPO/pr3 + Neutrophils, MPO/pr3 moves to surface where Ab’s attach and cause inflammatory response
Symptoms of AAV
Fever
Malaise
Myalgias
Migratory Arthralgias
Which AAV is a saddle nose deformity seen in?
GPA (pr3)
Renal symptoms of AAV
Hematuria, proteinuria, and RF
Are purpura associated with AAV palpable or not?
Palpable.
Non-palpable purpura is seen more in thrombocytopenia
Which AAV has a more acute presentation
GPA (PR3)
What pattern of glomerular damage is seen in AAV?
Crescentic pattern RPGN
Induction Treatment for AAV
High dose glucocorticoids + Rituximab
OR
glucocorticoids + Cyclophosphamide
Maintenance Therapy for AAV
1: Azathioprine + Rituximab
2: Methotrexate
Anti-GBM age group
Bimodal: 20’s and 60’s
What part of the GBM does Goodpasture affect?
Lamina Densa a3 chain of Type IV collagen
What type of Glomerular damage is seen in Anti-GBM?
RPGN with crescent formation and Nephritic Syndrome
What type of pattern is seen on IF in Anti-GBM?
Diffuse Linear
Treatment for Anti-GBM
Plasmapheresis + Glucocorticoids + Cyclophosphamide
