9 results
Diagnosis and Management of Hypocomplementemic Urticarial Vasculitis (HUV)

Diagnosis of HUV
1) Chronic urticaria 
2) Leukocytoclastic vasculitis 
3)
Diagnosis and Management ... of Hypocomplementemic ... vasculitis 3) Hypocomplementemia ... Therapeutic management ... TerrierBen #Hypocomplementemic
Causes of Hypocomplementemia - CHAMPS Mnemonic
C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis
H - Heavy Chain deposition
Causes of Hypocomplementemia ... autoimmune (RA, Sjogren's ... @AnnKumfer #Hypocomplementemia ... #Low #Complement
Pathophysiology of Hypocomplementemic Urticarial Vasculitis

C1q/anti-C1q antibiody ->
Immune Complex Formation - Complement Pathway Activation (C3a and C5a)
Pathophysiology of Hypocomplementemic ... Complex Formation - Complement ... TerrierBen #Hypocomplementemic
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... against MPO • Complement ... #rheumatology #classification
IgG4-Related Disease
Clinical history:  Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor
4 phenotypes:
 •
rheumatoid factor), ± hypocomplementemia ... Disease #diagnosis #management
Sjogren's Syndrome Overview

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

Autoimmune exocrinopathy multisystemic disease
Sjogren's Syndrome ... enlargement Sjogren's ... Cytopenia: 2% • Hypocomplementemia ... or 3rd line #Sjogrens ... Rheumatology #Diagnosis #Management
Cryoglobulinemia Summary

Cryoglobulins:
 • Cryoglobulins are Igs in the serum that precipitate in vitro at temperatures below
RA - 5 to 20% Sjögren's ... • Low serum C4 complement ... rheumatoid arthritis, Sjögren's ... 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 ... dermatomyositis, Sjogren ... RNP antibodies, Complement
IGG-4 RELATED DISEASE

WHAT?
 • A chronic, immune-mediated fibroinflammatory disease with tumefactive infiltration of IgG4+ plasma cells
- ↑ IgE, ↓ complement ... • Classification ... mimics excluded MANAGEMENT ... IGG4 #Diagnosis #Management