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2021 ACR/VF Guidelines for Management of Eosinophilic Granulomatosis with Polyangiitis (EGPA)

#EGPA #Eosinophilic #Granulomatosis #Polyangiitis #ACR2021 #Management
Polyangiitis (EGPA ... ) #EGPA #Eosinophilic
Rapidly Progressive Glomerulonephritis (RPGN)
RPGN has three primary pathophysiologic causes differentiated by immunofluorescence

Immune Complex Mediated (granular staining):
ANCA (C-ANCA): GPA ... >>> MPA > eGPA ... MPO-ANCA (P-ANCA): eGPA ... , MPA >>> GPA Stanford
Pulmonary Renal Syndromes - OnePager Summary
Autoimmune ANCA vasculitis (AAV): GPA (granulomatous with polyangiitis), EGPA (eosinophilic granulomatosis
polyangiitis), EGPA ... DAH), asthma in EGPA ... nodules, asthma (EGPA ... • Cardiac (in EGPA ... if concern for EGPA
ILD of Known Cause - Differential Diagnosis
Exposure:
 • Occupational: Asbestosis, Silicosis, Hypersensitivity, Pneumonitis
 • Treatment Related:
with Vasculitis: GPA ... , EGPA • Granulomatous
Granulomatous Lung Disease - Differential Diagnosis Algorithm
OP: organising pneumonia; EGPA: eosinophilic granulomatosis with polyangiitis; NSG: necrotising
organising pneumonia; EGPA ... granulomatosis; GPA
Vasculitis Management - ACR/VF 2021 Guidelines for Treatment & Management
 • Giant Cell Arteritis (GCA)
 •
Polyangiitis (GPA ... Polyangiitis (EGPA
Eosinophilic Granulomatosis with Polyangiitis (EGPA) - ANCA Vasculitis Management Algorithm - ACR/VF 2021 Guidelines
 • Active
Polyangiitis (EGPA ... @MithuRheum #EGPA
ANCA Associated Vasculitis (AAV) - Auto-antibodies and Differential Diagnosis

MPA → usually p-ANCA
GPA → usually c-ANCA
EGPA →
usually p-ANCA GPA ... usually c-ANCA EGPA
ANCA-Associated Vasculitis
There are many definitions, but most come down to the presence of granulomas (pathologically or
polyangiitis (GPA ... polyangiitis (eGPA
Dermatological semiology of Cutaneous Vasculitis 

Clinicopathologic correlations
 • Purpura, papules, pustules, necrosis: involvement of small vessels
vasculitis (5%): PAN > GPA ... , EGPA • Solid