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Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
Vasculitis and Vasculitides ... tract, and often causes ... often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... against MPO • Complement
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
Diagnosis and Management of Hypocomplementemic Urticarial Vasculitis (HUV)

Diagnosis of HUV
1) Chronic urticaria 
2) Leukocytoclastic vasculitis 
3)
Management of Hypocomplementemic ... Urticarial Vasculitis ... Leukocytoclastic vasculitis ... 3) Hypocomplementemia ... #Urticarial #Vasculitis
Causes of Hypocomplementemia - CHAMPS Mnemonic
C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis
H - Heavy Chain deposition
Causes of Hypocomplementemia ... @AnnKumfer #Hypocomplementemia ... #Low #Complement
Rapidly Progressive Glomerulonephritis (RPGN)
RPGN has three primary pathophysiologic causes differentiated by immunofluorescence

Immune Complex Mediated (granular staining):
pathophysiologic causes ... staining): • Low Complement ... Idiopathic • Normal Complement ... Glomerulonephritis #Vasculitis
Pulmonary Renal Syndromes and Mimics - Differential Diagnosis Framework
Diffuse alveolar hemorrhage + rapidly progressive glomerulonephritis
90%:
 -
ANCA Associated Vasculitis ... anti-GBM) 10%: - Low Complement ... Syndrome, Rheumatoid Vasculitis ... - Normal Complement ... Renal Crisis, IgA Vasculitis
ANCA-Associated Vasculitis
A group of necrotizing, small-vessel vasculitis without immune complex deposition. Complement levels are normal due
ANCA-Associated Vasculitis ... , small-vessel vasculitis ... Complement levels ... Levamisole-induced vasculitis ... ANCA #Associated #Vasculitis
Causes of Hemolytic Anemia - Differential Diagnosis
Inherited
 • Membrane
 • Internal
    - Hemoglobin
Causes of Hemolytic ... Antibody/DAT+ - Complement ... Differential #Diagnosis #Causes
Vasculitis - Differential Diagnosis Framework

When to Consider Vasculitis:
• Purpura, ischemic skin lesions
• Mononeuritis multiplex
• Hematuria, proteinuria,
Vasculitis - Differential ... When to Consider Vasculitis ... tract, and often causes ... Elevated AST ALT • Hypocomplementemia ... ) • Hypocomplementemic
Cryoglobulinemia Summary

Cryoglobulins:
 • Cryoglobulins are Igs in the serum that precipitate in vitro at temperatures below
• They can cause ... Cryoglobulins Cause ... • Low serum C4 complement ... • IgA vasculitis ... • Vasculitis