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A System for Vasculitides 

#Diagnosis #IM #Rheum #Vasculitis #Small #Medium #Large #Vessel #Size #Classification #ANCA #Differential
A System for Vasculitides ... Diagnosis #IM #Rheum #Vasculitis ... #Vessel #Size #Classification ... #ANCA #Differential ... #Algorithm #Ddxof
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... Vasculitis: + MPO-ANCA ... against MPO • Complement ... #rheumatology #classification
Rapidly Progressive Glomerulonephritis (RPGN)
RPGN has three primary pathophysiologic causes differentiated by immunofluorescence

Immune Complex Mediated (granular staining):
staining): • Low Complement ... Idiopathic • Normal Complement ... (C-ANCA): GPA > ... Glomerulonephritis #Vasculitis ... differential #diagnosis #algorithm
Classification of Vasculitis 
Vasculitis of large vessels 
 - Arteritis temporalis (giant-cell arteritis) 
 - Takayasu
Classification of ... central nervous system ... small vessels - ANCA-associated ... disease - systemic ... dermatomyositis #Size #Classification
Vasculitis - Differential Diagnosis Framework

When to Consider Vasculitis:
• Purpura, ischemic skin lesions
• Mononeuritis multiplex
• Hematuria, proteinuria,
Diagnosis: • Systemic ... Presentation: Systemic ... Central Nervous System ... Elevated AST ALT • Hypocomplementemia ... ) • Hypocomplementemic
Aortitis - Differential Diagnosis Framework
Non-Infectious Aortitis:
 • Vasculitides: Giant Cell Arteritis (GCA), Takayasu's arteritis, Cogan's Syndrome,
Nodosa (PAN), ANCA ... associated vasculitis ... Rheumatic Diseases: IgG4 ... Related Disease (IgG4 ... Arthritis (RA), Systemic
IgG4-Related Disease
Clinical history:  Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor
4 phenotypes:
 •
IgG4-Related Disease ... female, asian • Systemic ... node), high serum IgG4 ... rheumatoid factor), ± hypocomplementemia ... No necrosis, No vasculitis
Cryoglobulinemia Summary

Cryoglobulins:
 • Cryoglobulins are Igs in the serum that precipitate in vitro at temperatures below
activate the immune system ... • Low serum C4 complement ... DDX: • ANCA (Antineutrophil ... disorder (e.g. systemic ... diagnosis #types #classification
Hypersensitivity Types and Their Mechanisms
Type I - lgE - Soluble antigen
 - Allergen-specific lgE antibodies bind
- Local and systemic ... antigen, leading to complement ... Complement activation ... arthritis, and systemic ... Hypersensitivity #Types #Classification
EULAR/ACR Classification Criteria for Systemic Lupus Erythematosus
Clinical Domains:
 • Constitutional domain: Fever
 • Cutaneous domain: Non-scarring
EULAR/ACR Classification ... Criteria for Systemic ... Anticardiolipin IgG ... or anti-ß2GPI IgG ... anticoagulant • Complement