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rheumatology diagnosis differential huv igg4 urticarial classification disease glomerulonephritis pathophysiology phenotypes rapidly related rpgn sjogrens syndrome treatment vasculitides workup
Diagnosis and Management of Hypocomplementemic Urticarial Vasculitis (HUV) Diagnosis of HUV 1) Chronic urticaria 2) Leukocytoclastic vasculitis 3)
Diagnosis and Management ... of Hypocomplementemic ... Urticarial Vasculitis ... 3) Hypocomplementemia ... TerrierBen #Hypocomplementemic
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
Vasculitis and Vasculitides - Differential Diagnosis Framework When to Consider Vasculitis: • Purpura, ischemic skin lesions •
Vasculitis and Vasculitides ... proteinuria, rapidly progressing ... often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... against MPO • Complement
Rapidly Progressive Glomerulonephritis (RPGN) RPGN has three primary pathophysiologic causes differentiated by immunofluorescence Immune Complex Mediated (granular staining):
Rapidly Progressive ... staining): • Low Complement ... RPGN #Rapidly #Progressive ... 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
IgG4-Related Disease Clinical history: Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor 4 phenotypes: •
rheumatoid factor), ± hypocomplementemia ... No necrosis, No vasculitis ... Disease #diagnosis #management
IGG-4 RELATED DISEASE WHAT? • A chronic, immune-mediated fibroinflammatory disease with tumefactive infiltration of IgG4+ plasma cells
inflammation -> progressive ... aortitis, mimics vasculitis ... - ↑ IgE, ↓ complement ... mimics excluded MANAGEMENT ... IGG4 #Diagnosis #Management
Sjogren's Syndrome Overview Epidemiology: • F > M: 9:1 • 5-6th Decades (can be any age) Autoimmune exocrinopathy multisystemic disease
Cytopenia: 2% • Hypocomplementemia ... ) • Cutaneous vasculitis ... : 2% Systemic Vasculitis ... Rheumatology #Diagnosis #Management
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