11 results
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 ... Chain deposition disease ... @AnnKumfer #Hypocomplementemia ... #Low #Complement
Vasculitis and Vasculitides - Differential Diagnosis Framework

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

When to Consider Vasculitis:
• Purpura, ischemic skin lesions
• Mononeuritis multiplex
• Hematuria, proteinuria,
Systemic rheumatic diseases ... atherosclerotic disease ... most important diseases ... Elevated AST ALT • Hypocomplementemia ... ) • Hypocomplementemic
IgG4-Related Disease
Clinical history:  Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor
4 phenotypes:
 •
IgG4-Related Disease ... rheumatoid factor), ± hypocomplementemia ... No necrosis, No vasculitis ... IgG4 #Related #Disease
Rapidly Progressive Glomerulonephritis (RPGN)
RPGN has three primary pathophysiologic causes differentiated by immunofluorescence

Immune Complex Mediated (granular staining):
staining): • Low Complement ... Idiopathic • Normal Complement ... involvement: Anti-GBM Disease ... Glomerulonephritis #Vasculitis ... differential #diagnosis #algorithm
Hemolytic Anemia - Differential Diagnosis Algorithm
 • Sickle cells - Consider sickle cell disease (diverse genotypes):
Differential Diagnosis Algorithm ... Consider sickle cell disease ... cardiac valve, vasculitis ... Differential #Diagnosis #Algorithm ... workup #hematology #testing
Cold Agglutinin Disease

3 Types of Cold Sensitive Antibodies:
  1. Cold Agglutinins (CAD)
  2. Donath-Landsteiner
Cold Agglutinin Disease ... Cryoglobulins (Vasculitic ... Positive Coombs testing ... post infectious setting ... temperatures by fixing complement
Recommended initial testing for patients being evaluated for glomerular disease 

24-hour urine collection - Quantify proteinuria.
Recommended initial testing ... for glomerular disease ... or alternative complement ... of glomerular disease ... for ANCA-related vasculitis
Sjogren's Syndrome Overview

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

Autoimmune exocrinopathy multisystemic disease
multisystemic disease ... Cytopenia: 2% • Hypocomplementemia ... demyelinating disease ... ) • Cutaneous vasculitis ... : 2% Systemic Vasculitis