15 results
Diagnosis and Management of Hypocomplementemic Urticarial Vasculitis (HUV)

Diagnosis of HUV
1) Chronic urticaria 
2) Leukocytoclastic vasculitis 
3)
Management of Hypocomplementemic ... (HUV) Diagnosis ... of HUV 1) Chronic ... 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 ... TerrierBen #Hypocomplementemic ... #HUV #Pathophysiology
Causes of Hypocomplementemia - CHAMPS Mnemonic
C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis
H - Heavy Chain deposition
Causes of Hypocomplementemia ... Chain deposition disease ... A - Atypical HUS ... @AnnKumfer #Hypocomplementemia ... #Low #Complement
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... against MPO • Complement ... Cryoglobulins • HIV ... • HBV HCV •
Pulmonary Renal Syndromes and Mimics - Differential Diagnosis Framework
Diffuse alveolar hemorrhage + rapidly progressive glomerulonephritis
90%:
 -
- Anti-GBM Disease ... anti-GBM) 10%: - Low Complement ... - Normal Complement ... , Behcet's Disease ... , atypical HUS,
Vasculitis - Differential Diagnosis Framework

When to Consider Vasculitis:
• Purpura, ischemic skin lesions
• Mononeuritis multiplex
• Hematuria, proteinuria,
atherosclerotic disease ... most important diseases ... HTN • Check: HBV ... 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
• Infections: HCV ... infection, HBV ... infection, HIV infection ... • Autoimmune diseases ... • Low serum C4 complement
Hemolytic Anemia - Differential Diagnosis Algorithm
 • Sickle cells - Consider sickle cell disease (diverse genotypes):
Differential Diagnosis Algorithm ... Consider sickle cell disease ... DIC, TTP, HELLP, HUS ... cardiac valve, vasculitis ... Differential #Diagnosis #Algorithm