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Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
Urticaria and hypocomplementemia ... inflammation Others: • Lupus ... such as systemic lupus ... against MPO • Complement ... , and SLE) • Urinalysis
Systemic Lupus Erythematosus (SLE) - Diagnosis and Management Summary
 • Epidemiology: 10-180/100,000, Typically Age 20-40, F:M
Systemic Lupus Erythematosus ... and Management ... , oral ulcers, Lupus ... 80% - Low complement ... #lupus #Systemic
Drug Induced Lupus vs SLE
Drug Induced Lupus (DIL):
 • Epidemiology: -10% of all lupus cases, drug-dependent,
vs SLE Drug Induced ... 90% - Low complement ... 80% - Low complement ... Usual therapeutic management ... rheumatology #diagnosis #management
Rapidly Progressive Glomerulonephritis (RPGN)
RPGN has three primary pathophysiologic causes differentiated by immunofluorescence

Immune Complex Mediated (granular staining):
staining): • Low Complement ... Post-strep GN, Lupus ... Idiopathic • Normal Complement ... Glomerulonephritis #Vasculitis ... differential #diagnosis #algorithm
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 ... • Mesenteric vasculitis ... RNP antibodies, Complement
Cryoglobulinemia Summary

Cryoglobulins:
 • Cryoglobulins are Igs in the serum that precipitate in vitro at temperatures below
diseases: - 10% SLE ... • Low serum C4 complement ... • Vasculitis ... (e.g. systemic lupus ... erythematosus (SLE
Algorithm for the Treatment of Membranous Lupus Nephritis
Sub-nephrotic-range proteinuria (<3.5g/d, normal serum albumin) and Preserved kidney
Algorithm for the ... of Membranous Lupus ... treat non-renal SLE ... treat extra-renal SLE ... #Treatment #management
Sjogren's Syndrome Overview

Epidemiology:
• F > M: 9:1
• 5-6th Decades (can be any age)

Autoimmune exocrinopathy multisystemic disease
overlaps with RA, SLE ... Cytopenia: 2% • Hypocomplementemia ... ) • Cutaneous vasculitis ... : 2% Systemic Vasculitis ... Rheumatology #Diagnosis #Management
Drug Induced Lupus (DIL)
 • Epidemiology: -10% of all lupus cases, drug-dependent, 4:1 to 1:1 F:M
Drug Induced Lupus ... Epidemiology: -10% of all lupus ... is rare in DIL, SCLE-DIL ... 90% - Low complement ... diagnosis #treatment #management
TAFRO

TAFRO syndrome was first described in 2010, standing for:
- Thrombocytopenia
- Anasarca
- Fever
- Reticulin fibrosis
- Organomegaly

TAFRO syndrome
including systemic lupus ... erythematosus (SLE ... ANCA-associated vasculitis ... TAFRO #diagnosis #management