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Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
- Skin, glomeruli ... Urticaria and hypocomplementemia ... : - Kidneys: glomerular ... against MPO • Complement ... #rheumatology #classification
Vasculitis - Break down based on vessel size 
Distribution of vessel involvement by large vessel vasculitis,
Vasculitis - Break ... by large vessel vasculitis ... can affect any type ... Anti-GBM = anti–glomerular ... Medium #Large #Classification
Cryoglobulinemia Summary

Cryoglobulins:
 • Cryoglobulins are Igs in the serum that precipitate in vitro at temperatures below
Types: • Type ... • Type III Often ... • Low serum C4 complement ... • Vasculitis ... #classification
Recommended initial testing for patients being evaluated for glomerular disease 

24-hour urine collection - Quantify proteinuria.
Recommended initial testing ... or alternative complement ... for ANCA-related vasculitis ... cryoglobulinemias (types ... #testing #workup
Recommended initial testing for patients being evaluated for glomerular disease
1) 24-hour urine collection - Quantify proteinuria.
Recommended initial testing ... or alternative complement ... for ANCA-related vasculitis ... cryoglobulinemias (types ... diagnosis #initial #testing
Cold Agglutinin Disease

3 Types of Cold Sensitive Antibodies:
  1. Cold Agglutinins (CAD)
  2. Donath-Landsteiner
Agglutinin Disease 3 Types ... Cryoglobulins (Vasculitic ... • Symptoms of anemia ... Positive Coombs testing ... temperatures by fixing complement
Clinical Classification of Pulmonary Hypertension

1. Pulmonary arterial hypertension from pulmonary vasculopathy
Idiopathic pulmonary arterial hypertension
Heritable gene mutations
Clinical Classification ... protein receptor type ... activin A receptor type ... Chronic hemolytic anemia ... neurofibromatosis, vasculitis
SLE (Systemic Lupus Erythematosus)

Suspect:
Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
anti-Smith Ab, low complements ... Skin Disease Classification ... Kikuchi disease • Type-I ... lymphopenia, low PLT • Anemia ... RNP antibodies, Complement
IGG-4 RELATED DISEASE

WHAT?
 • A chronic, immune-mediated fibroinflammatory disease with tumefactive infiltration of IgG4+ plasma cells
• Pancreas: Type ... aortitis, mimics vasculitis ... - ↑ IgE, ↓ complement ... • Classification
Monoclonal Gammopathy of Renal Significance (MGRS)

MGRS
• MGRS refers to kidney disease caused by nephrotoxic monoclonal immunoglobulins
guide subtype classification ... tubulopathy) - Complement ... formation causing vasculitis ... Types of MGRS-Associated