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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 ... Membranoproliferative glomerulonephritis ... Post/pauci immune glomerulonephritis ... @AnnKumfer #Hypocomplementemia ... #Low #Complement
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
, proteinuria, rapidly ... often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... , Glomerulonephritis ... 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 ... Idiopathic • Normal Complement ... #Vasculitis #differential ... #diagnosis #algorithm
Pulmonary Renal Syndromes and Mimics - Differential Diagnosis Framework
Diffuse alveolar hemorrhage + rapidly progressive glomerulonephritis
90%:
 -
alveolar hemorrhage + rapidly ... progressive glomerulonephritis ... ANCA Associated Vasculitis ... anti-GBM) 10%: - Low Complement ... - Normal Complement
Vasculitis - Differential Diagnosis Framework

When to Consider Vasculitis:
• Purpura, ischemic skin lesions
• Mononeuritis multiplex
• Hematuria, proteinuria,
, proteinuria, Rapidly ... Pauci immune GN (rapidly ... (Glomerulonephritis ... Elevated AST ALT • Hypocomplementemia ... ) • Hypocomplementemic
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 ... acidosis • NAGMA • Glomerulonephritis ... : 2% Systemic Vasculitis