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diagnosis rheumatology differential management classification hematology agglutinin allergy anemia champs cold glomerulonephritis hemolytic huv hypersensitivity low mnemonic myelitis neurology phenotypes
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
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
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 ... #Related #Disease
IGG-4 RELATED DISEASE WHAT? • A chronic, immune-mediated fibroinflammatory disease with tumefactive infiltration of IgG4+ plasma cells
IGG-4 RELATED DISEASE ... fibroinflammatory disease ... dysfunction PATHOPHYSIOLOGY ... aortitis, mimics vasculitis ... - ↑ IgE, ↓ 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 ... involvement: Anti-GBM Disease ... Glomerulonephritis #Vasculitis ... differential #diagnosis #algorithm
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
Hypersensitivity Types and Their Mechanisms Type I - lgE - Soluble antigen - Allergen-specific lgE antibodies bind
antigen, leading to complement ... during hemolytic disease ... Type Ill - IgG and ... Complement activation ... Classification #Pathophysiology
Cold Agglutinin Disease 3 Types of Cold Sensitive Antibodies: 1. Cold Agglutinins (CAD) 2. Donath-Landsteiner
Cold Agglutinin Disease ... Cryoglobulins (Vasculitic ... Cold Agglutinin Disease ... Donath-Landsteiner (IgG ... temperatures by fixing complement
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
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