7 results
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 ... Complex Formation - Complement ... TerrierBen #Hypocomplementemic ... #HUV #Pathophysiology
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
Vasculitis and Vasculitides ... When to Consider Vasculitis ... often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... against MPO • Complement
Rapidly Progressive Glomerulonephritis (RPGN)
RPGN has three primary pathophysiologic causes differentiated by immunofluorescence

Immune Complex Mediated (granular staining):
three primary pathophysiologic ... staining): • Low Complement ... Idiopathic • Normal Complement ... Glomerulonephritis #Vasculitis ... differential #diagnosis #algorithm
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
Sjogren's Syndrome Overview

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

Autoimmune exocrinopathy multisystemic disease
Cytopenia: 2% • Hypocomplementemia ... ) • Cutaneous vasculitis ... : 2% Systemic Vasculitis ... Rheumatology #Diagnosis #Management
IGG-4 RELATED DISEASE

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