Vasculitis and Alveolar Hemorrhage Flashcards
3 ANCA associated vasculitises
MPA - Microscopic polyangiitis
GPA - Granulomatosis with polyangiitis (Wegener’s)
EGPA - Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
4 Immune complex associated vasculitises
Anti-GBM (Goodpastures)
IgA-vasculitis (Henoch-Shoenlein)
Cryoglobulinemic vasculitis
Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)
Most common type of vasculitis
Giant cell arteritis
Rarely effects lungs but may have cough and nodules
3 “NO’s” of polyarteritis Nodosa
No ANCA
No glomerulonephritis
No pulmonary capillaritis
2 large to medium vessel vasculitises that can cause pulmonary artery aneurysms
Takayasu (young female)
Behcet’s disease
What percentage of GPA or MPA will have develop diffuse alveolar hemorrhage?
25%
Which ANCA version is more likely to relapse
C-ANCA/PR3-ANCA
C-ANCA is associated with PR3 or MPO
PR3
Is there a correlation of the level of ANCA positivity and the severity of disease?
NO
Saddle Nose deformity
Necrotizing granulomatous inflammation of GPA
Strawberry gums
Necrotizing granulomatous inflammation of GPA
Remission induction regimen in limited GPA
MTX and GCS
Trial that compared rtixumab and cyclophosphamide
RAVE trial
NEJM 2010
Remission induction regimen for SEVERE GPA or MPA
GCS and cyclophosphamide or rituximab
Subset of severe patients with MPA that will benefit from cyclophosphamide OVER rituximab in remission induction
MPO+ patients with severe renal disease
Remission maintenance therapy for GPA or MPA following cyclophosphamide
Methotrexate or azithioprine
Cellcept can be used but not as good
Remission maintenance therapy for GPA or MPA following rituximab
Rtiuximab
Mainstay of therapy in churg strauss
glucocorticoids
Steroid sparing agents in churg strauss
MTX, AZA, Anti-IL5
Situations where CYC is required for severe churg strauss
Heart, CNS, mononeuritis multiplex, severe renal involvement
Mepolizumab dosing difference in Asthma vs Churg Strauss
Asthma = 100 mg q 4 wks
Churg Strauss = 300 mg q 4 wks
Biologic Asthma Meds (name, brand, Mechanism, dosing)
Percentage of hemosiderin-laden macrophages that suggests DAH
> 20%
