16 results
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
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 ... rheumatoid factor), ± hypocomplementemia ... No necrosis, No vasculitis ... #Related #Disease ... #diagnosis #management
IGG-4 RELATED DISEASE

WHAT?
 • A chronic, immune-mediated fibroinflammatory disease with tumefactive infiltration of IgG4+ plasma cells
IGG-4 RELATED DISEASE ... aortitis, mimics vasculitis ... - ↑ IgE, ↓ complement ... mimics excluded MANAGEMENT ... #Management #Rheumatology
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
Polyarteritis Nodosa (PAN) - Vasculitis Management Algorithm - ACR/VF 2021 Guidelines
 • Severe Disease → Remission
Nodosa (PAN) - Vasculitis ... Management Algorithm ... Guidelines • Severe Disease ... • Non-Severe Disease ... #Management #Treatment
Treatment of Non-hepatitis C virus Cryoglobulinemia Vasculitis - French Vasculitis Study Group

MONOCLONAL CRYOGLOBULINEMIA (TYPE 1) 
IgM
Cryoglobulinemia Vasculitis ... - French Vasculitis ... chloraminophene IgG ... /or refractory disease ... #Management #Rheumatology