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Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... , such as systemic ... against MPO • Complement ... , and SLE) • Urinalysis
Causes of Hypocomplementemia - CHAMPS Mnemonic
C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis
H - Heavy Chain deposition
Causes of Hypocomplementemia ... Chain deposition disease ... glomerulonephritis (>90%) S - Systemic ... @AnnKumfer #Hypocomplementemia ... #Low #Complement
Pulmonary Renal Syndromes and Mimics - Differential Diagnosis Framework
Diffuse alveolar hemorrhage + rapidly progressive glomerulonephritis
90%:
 -
- Anti-GBM Disease ... anti-GBM) 10%: - Low Complement ... Levels: SLE, Cryoglobulinemic ... - Normal Complement ... , Behcet's Disease
Systemic Lupus Erythematosus (SLE) - Diagnosis and Management Summary
 • Epidemiology: 10-180/100,000, Typically Age 20-40, F:M
Systemic Lupus Erythematosus ... • Immunologic Workup ... 80% - Low complement ... Evolution: Chronic disease ... #lupus #Systemic
Vasculitis - Differential Diagnosis Framework

When to Consider Vasculitis:
• Purpura, ischemic skin lesions
• Mononeuritis multiplex
• Hematuria, proteinuria,
Diagnosis: • Systemic ... , such as SLE, atherosclerotic ... Presentation: Systemic ... Elevated AST ALT • Hypocomplementemia ... ) • Hypocomplementemic
Drug Induced Lupus vs SLE
Drug Induced Lupus (DIL):
 • Epidemiology: -10% of all lupus cases, drug-dependent,
• Immunologic Workup ... 90% - Low complement ... months to years) Systemic ... • Immunologic Workup ... Evolution: Chronic disease
Clinical Features of Systemic Lupus (SLE)
General: Fever (50%), Depression, Fatigue (75%), Weight loss (50%)
Eye: Sjögrens (15%)
Skin
Clinical Features of Systemic ... Lupus (SLE) General ... Butterfly rash, Vasculitis ... pain (20%) Renal disease ... Thrombocytopenia #Lupus #SLE
Cryoglobulinemia Summary

Cryoglobulins:
 • Cryoglobulins are Igs in the serum that precipitate in vitro at temperatures below
activate the immune system ... • Autoimmune diseases ... : - 10% SLE or ... • Low serum C4 complement ... erythematosus (SLE
SLE (Systemic Lupus Erythematosus)

Suspect:
Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
SLE (Systemic Lupus ... anti-Smith Ab, low complements ... ANCA-associated vasculitis ... RNP antibodies, Complement ... #Systemic #Lupus
ANA (Antinuclear Antibody) and ANCA (Antineutrophil cytoplasmic antibodies)
 • Systemic lupus erythematosus
   1. Anti-dsDNA
antibodies) • Systemic ... in drug-induced SLE ... ) • Systemic sclerosis ... ANCA-Associated Vasculitis ... Renal-limited vasculitis