4 results
IgG4-Related Disease
Clinical history:  Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor
4 phenotypes:
 •
IgG4-Related Disease ... node), high serum IgG4 ... rheumatoid factor), ± hypocomplementemia ... No necrosis, No vasculitis ... management #phenotypes #workup
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
IGG-4 RELATED DISEASE

WHAT?
 • A chronic, immune-mediated fibroinflammatory disease with tumefactive infiltration of IgG4+ plasma cells
IGG-4 RELATED DISEASE ... infiltration of IgG4 ... aortitis, mimics vasculitis ... - ↑ IgE, ↓ complement ... • Classification
Monoclonal Gammopathy of Renal Significance (MGRS)

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