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Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... (Granulomatous disease ... against MPO • Complement ... #rheumatology #classification
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
Polyarteritis Nodosa (PAN) - Vasculitis Management Algorithm - ACR/VF 2021 Guidelines
 • Severe Disease → Remission
Nodosa (PAN) - Vasculitis ... Management Algorithm ... Guidelines • Severe Disease ... • Non-Severe Disease ... #Management #Treatment
IgG4-Related Disease
Clinical history:  Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor
4 phenotypes:
 •
IgG4-Related Disease ... with response to treatment ... 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
• Autoimmune diseases ... • Low serum C4 complement ... • IgA vasculitis ... • Vasculitis ... diagnosis #types #classification
SLE (Systemic Lupus Erythematosus)

Suspect:
Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
anti-Smith Ab, low complements ... Classification: ... ANCA-associated vasculitis ... • Mesenteric vasculitis ... RNP antibodies, Complement
Approach to Thrombocytosis - Elevated Platelet Count - Differential Diagnosis Algorithm

Peripheral Smear concerning for malignancy?
Differential Diagnosis Algorithm ... Rebound effect from treatment ... Rheumatologic, Vasculitides ... , IBD, Celiac disease ... Differential #Diagnosis #Algorithm
IGG-4 RELATED DISEASE

WHAT?
 • A chronic, immune-mediated fibroinflammatory disease with tumefactive infiltration of IgG4+ plasma cells
IGG-4 RELATED DISEASE ... fibroinflammatory disease ... aortitis, mimics vasculitis ... - ↑ IgE, ↓ complement ... • Classification
Sjogren's Syndrome Overview

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

Autoimmune exocrinopathy multisystemic disease
multisystemic disease ... Cytopenia: 2% • Hypocomplementemia ... ) • Cutaneous vasculitis ... : 2% Systemic Vasculitis ... Adenocarcinoma Treatment
Monoclonal Gammopathy of Renal Significance (MGRS)

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