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Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
Vasculitis and Vasculitides ... often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... against MPO • Complement ... #rheumatology #classification
Cryoglobulinemia Summary

Cryoglobulins:
 • Cryoglobulins are Igs in the serum that precipitate in vitro at temperatures below
Cryoglobulins: - Single monoclonal ... - Composed of monoclonal ... • Low serum C4 complement ... • Vasculitis ... diagnosis #types #classification
Recommended initial testing for patients being evaluated for glomerular disease 

24-hour urine collection - Quantify proteinuria.
chains - Screen for monoclonal ... for detection of monoclonal ... or alternative complement ... for ANCA-related vasculitis ... glomerular #testing #workup
IgG4-Related Disease
Clinical history:  Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor
4 phenotypes:
 •
rheumatoid factor), ± hypocomplementemia ... No necrosis, No vasculitis ... Disease #diagnosis #management ... #phenotypes #workup
Recommended initial testing for patients being evaluated for glomerular disease
1) 24-hour urine collection - Quantify proteinuria.
or alternative complement ... chains - Screen for monoclonal ... for detection of monoclonal ... for ANCA-related vasculitis ... glomerular #disease #workup
IGG-4 RELATED DISEASE

WHAT?
 • A chronic, immune-mediated fibroinflammatory disease with tumefactive infiltration of IgG4+ plasma cells
aortitis, mimics vasculitis ... - ↑ IgE, ↓ complement ... • Classification ... mimics excluded MANAGEMENT ... IGG4 #Diagnosis #Management
Monoclonal Gammopathy of Renal Significance (MGRS)

MGRS
• MGRS refers to kidney disease caused by nephrotoxic monoclonal immunoglobulins
Monoclonal Gammopathy ... Diagnostic Workup ... guide subtype classification ... tubulopathy) - Complement ... formation causing vasculitis