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Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
abnormalities SMALL ... pain, infarct • Small ... against MPO • Complement ... , and SLE) • Urinalysis ... #rheumatology #classification
IgG4-Related Disease
Clinical history:  Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor
4 phenotypes:
 •
IgG4-Related Disease ... false negative (small ... rheumatoid factor), ± hypocomplementemia ... No necrosis, No vasculitis ... Disease #diagnosis #management
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 ... #Management #Rheumatology
Cryoglobulinemia Summary

Cryoglobulins:
 • Cryoglobulins are Igs in the serum that precipitate in vitro at temperatures below
diseases: - 10% SLE ... • Low serum C4 complement ... • Cutaneous small-vessel ... erythematosus (SLE ... diagnosis #types #classification
SLE (Systemic Lupus Erythematosus)

Suspect:
Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
anti-Smith Ab, low complements ... Skin Disease Classification ... ANCA-associated vasculitis ... inflammation of the small ... RNP antibodies, Complement
Sjogren's Syndrome Overview

Epidemiology:
• F > M: 9:1
• 5-6th Decades (can be any age)

Autoimmune exocrinopathy multisystemic disease
overlaps with RA, SLE ... Cytopenia: 2% • Hypocomplementemia ... nervous system: small-fiber ... ) • Cutaneous vasculitis ... Rheumatology #Diagnosis #Management