24 results
Diagnosis and Management of Hypocomplementemic Urticarial Vasculitis (HUV)

Diagnosis of HUV
1) Chronic urticaria 
2) Leukocytoclastic vasculitis 
3)
Management of Hypocomplementemic ... vasculitis 3) Hypocomplementemia ... TerrierBen #Hypocomplementemic ... Diagnosis #Management #Rheumatology
Pathophysiology of Hypocomplementemic Urticarial Vasculitis

C1q/anti-C1q antibiody ->
Immune Complex Formation - Complement Pathway Activation (C3a and C5a)
Pathophysiology of Hypocomplementemic ... Complex Formation - Complement ... TerrierBen #Hypocomplementemic ... Pathophysiology #Rheumatology
Causes of Hypocomplementemia - CHAMPS Mnemonic
C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis
H - Heavy Chain deposition
Causes of Hypocomplementemia ... @AnnKumfer #Hypocomplementemia ... #Low #Complement ... #differential #hematology
IgG4-Related Disease
Clinical history:  Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor
4 phenotypes:
 •
IgG4-Related Disease ... node), high serum IgG4 ... rheumatoid factor), ± hypocomplementemia ... with increased IgG4 ... workup #treatment #rheumatology
Warm vs Cold Autoimmune Hemolytic Anemia
Warm Autoimmune Hemolytic Anemia:
 • Mechanism: IgG and Complement-mediated
 • Etiology:
• Mechanism: IgG ... and Complement-mediated ... Coombs positive for IgG ... Mechanism: IgM and Complement-mediated ... Hemolytic #Anemia #hematology
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... against MPO • Complement ... differential #diagnosis #rheumatology
IGG-4 RELATED DISEASE

WHAT?
 • A chronic, immune-mediated fibroinflammatory disease with tumefactive infiltration of IgG4+ plasma cells
IGG-4 RELATED DISEASE ... infiltration of IgG4 ... lesions - IgG4 ... - ↑ IgE, ↓ complement ... #Management #Rheumatology
EULAR/ACR Classification Criteria for Systemic Lupus Erythematosus
Clinical Domains:
 • Constitutional domain: Fever
 • Cutaneous domain: Non-scarring
pericarditis • Hematologic ... Anticardiolipin IgG ... or anti-ß2GPI IgG ... anticoagulant • Complement ... Erythematosus #diagnosis #rheumatology
Rapidly Progressive Glomerulonephritis (RPGN)
RPGN has three primary pathophysiologic causes differentiated by immunofluorescence

Immune Complex Mediated (granular staining):
staining): • Low Complement ... Idiopathic • Normal Complement ... differential #diagnosis #algorithm ... #rheumatology
Causes of Extravascular and Intravascular Hemolysis - Differential Diagnosis Algorithm
Extravascular - Spleen and RES-mediated hemolysis
 -
Differential Diagnosis Algorithm ... perfringens - Complement-Mediated ... Differential #Diagnosis #Algorithm ... #Causes #Hematology