15 results
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 #HUV #Pathophysiology
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
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 ... involvement: Anti-GBM Disease ... differential #diagnosis #algorithm
IgG4-Related Disease
Clinical history:  Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor
4 phenotypes:
 •
IgG4-Related Disease ... rheumatoid factor), ± hypocomplementemia ... IgG4 #Related #Disease
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 ... Vasculitis) • Hypocomplementemic
C5-C9 Deficiency - Pathogenesis and Clinical Findings
Note:
 • Risk of invasive meningococcal disease is 1000-10000x higher
meningococcal disease ... is rare Normal complement ... response: The complement ... activation -> Complement ... #pathophysiology
A schema of the pathophysiology of the inborn errors of metabolism, excluding complex storage diseases. Helps
schema of the pathophysiology ... complex storage diseases ... connections and pathophysiology ... MetabolicEmergency #Genetics #Pathophysiology ... #Diagnosis #Algorithm
Hypersensitivity Types and Their Mechanisms
Type I - lgE - Soluble antigen
 - Allergen-specific lgE antibodies bind
antigen, leading to complement ... during hemolytic disease ... Complement activation ... Classification #Pathophysiology
Sjogren's Syndrome Overview

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

Autoimmune exocrinopathy multisystemic disease
multisystemic disease ... associated with other diseases ... Cytopenia: 2% • Hypocomplementemia ... demyelinating disease ... Tracheobronchial disease