11 results
diagnosis rheumatology differential management igg4 champs cryoglobulinemia cryoglobulins erythematosus gammopathy glomerulonephritis hematology huv low lupus mgrs mnemonic monoclonal pathophysiology phenotypes
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 ... against MPO • Complement ... #rheumatology #classification
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
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
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
Cryoglobulinemia Summary Cryoglobulins: • Cryoglobulins are Igs in the serum that precipitate in vitro at temperatures below
• Autoimmune diseases ... • Low serum C4 complement ... • IgA vasculitis ... • Vasculitis ... diagnosis #types #classification
SLE (Systemic Lupus Erythematosus) Suspect: Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
anti-Smith Ab, low complements ... Classification: ... ANCA-associated vasculitis ... • Mesenteric vasculitis ... RNP antibodies, 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
IGG-4 RELATED DISEASE WHAT? • A chronic, immune-mediated fibroinflammatory disease with tumefactive infiltration of IgG4+ plasma cells
IGG-4 RELATED DISEASE ... fibroinflammatory disease ... aortitis, mimics vasculitis ... - ↑ IgE, ↓ complement ... • Classification
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