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diagnosis rheumatology differential management hematology renal agglutinin anemia champs classification cold gammopathy glomerulonephritis hemolytic huv low mgrs mimics mnemonic monoclonal
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 •
proteinuria, rapidly progressing ... often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... (Granulomatous 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
Rapidly Progressive Glomerulonephritis (RPGN) RPGN has three primary pathophysiologic causes differentiated by immunofluorescence Immune Complex Mediated (granular staining):
Rapidly Progressive ... 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,
proteinuria, Rapidly progressing ... is 50 years • Progressive ... immune GN (rapidly progressive ... Elevated AST ALT • Hypocomplementemia ... ) • Hypocomplementemic
Pulmonary Renal Syndromes and Mimics - Differential Diagnosis Framework Diffuse alveolar hemorrhage + rapidly progressive glomerulonephritis 90%: -
hemorrhage + rapidly progressive ... - Anti-GBM Disease ... anti-GBM) 10%: - Low Complement ... - Normal Complement ... , Behcet's 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 ... inflammation -> progressive ... aortitis, mimics vasculitis ... - ↑ IgE, ↓ 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
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
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