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Diagnosis and Management of Hypocomplementemic Urticarial Vasculitis (HUV)

Diagnosis of HUV
1) Chronic urticaria 
2) Leukocytoclastic vasculitis 
3)
Diagnosis and Management ... of Hypocomplementemic ... Urticarial Vasculitis ... 3) Hypocomplementemia ... TerrierBen #Hypocomplementemic
Pathophysiology of Hypocomplementemic Urticarial Vasculitis

C1q/anti-C1q antibiody ->
Immune Complex Formation - Complement Pathway Activation (C3a and C5a)
Pathophysiology of Hypocomplementemic ... Urticarial Vasculitis ... Complex Formation - Complement ... Formation -> Vasculitis ... TerrierBen #Hypocomplementemic
Causes of Hypocomplementemia - CHAMPS Mnemonic
C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis
H - Heavy Chain deposition
Causes of Hypocomplementemia ... antiphospholipid syndrome ... @AnnKumfer #Hypocomplementemia ... #Low #Complement
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... : • Behçet Syndrome ... • Cogan Syndrome ... against MPO • Complement
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 ... Disease #diagnosis #management
Vasculitis - Differential Diagnosis Framework

When to Consider Vasculitis:
• Purpura, ischemic skin lesions
• Mononeuritis multiplex
• Hematuria, proteinuria,
Mucocutaneous lymph node syndrome ... Elevated AST ALT • Hypocomplementemia ... ) • Hypocomplementemic ... • Behcet syndrome ... • Cogan syndrome
IGG-4 RELATED DISEASE

WHAT?
 • A chronic, immune-mediated fibroinflammatory disease with tumefactive infiltration of IgG4+ plasma cells
IGG-4 RELATED DISEASE ... aortitis, mimics vasculitis ... - ↑ IgE, ↓ complement ... mimics excluded MANAGEMENT ... #Management #Rheumatology
Sjogren's Syndrome Overview

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

Autoimmune exocrinopathy multisystemic disease
Sjogren's Syndrome ... Cytopenia: 2% • Hypocomplementemia ... ) • Cutaneous vasculitis ... : 2% Systemic Vasculitis ... Rheumatology #Diagnosis #Management
Cold Agglutinin Disease

3 Types of Cold Sensitive Antibodies:
  1. Cold Agglutinins (CAD)
  2. Donath-Landsteiner
Cryoglobulins (Vasculitic ... Cold Agglutinin Syndrome ... Donath-Landsteiner (IgG ... temperatures by fixing complement
TAFRO

TAFRO syndrome was first described in 2010, standing for:
- Thrombocytopenia
- Anasarca
- Fever
- Reticulin fibrosis
- Organomegaly

TAFRO syndrome
TAFRO TAFRO syndrome ... nodes TAFRO syndrome ... ANCA-associated vasculitis ... - IGG4 - Hepatic ... TAFRO #diagnosis #management