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

Diagnosis of HUV
1) Chronic urticaria 
2) Leukocytoclastic vasculitis 
3)
Management of Hypocomplementemic ... Urticarial Vasculitis ... 3) Hypocomplementemia ... TerrierBen #Hypocomplementemic ... #Urticarial #Vasculitis
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 ... TerrierBen #Hypocomplementemic ... #Urticarial #Vasculitis
Causes of Hypocomplementemia - CHAMPS Mnemonic
C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis
H - Heavy Chain deposition
Causes of Hypocomplementemia ... @AnnKumfer #Hypocomplementemia ... #Low #Complement
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
often involved • Hypocomplementemic ... Urticarial Vasculitis ... ): - Urticaria ... and hypocomplementemia ... against MPO • Complement
Vasculitis - Differential Diagnosis Framework

When to Consider Vasculitis:
• Purpura, ischemic skin lesions
• Mononeuritis multiplex
• Hematuria, proteinuria,
Elevated AST ALT • Hypocomplementemia ... : Urticarial Vasculitis ... ) • Hypocomplementemic ... Urticarial Vasculitis ... maculopapular lesions, urticaria
Rapidly Progressive Glomerulonephritis (RPGN)
RPGN has three primary pathophysiologic causes differentiated by immunofluorescence

Immune Complex Mediated (granular staining):
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
Complement 1 esterase inhibitor (C1-INH) Deficiency Testing Algorithm
INDICATIONS FOR TESTING:
 • Recurrent angioedema (without urticaria)
 •
Complement 1 esterase ... Deficiency Testing Algorithm ... angioedema (without urticaria ... ARUPLabs #C1INH #Complement ... Deficiency #Testing #Algorithm
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