89 results
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 ... Mettler_K1000 #IgG4
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
Pathophysiology of Hypocomplementemic Urticarial Vasculitis

C1q/anti-C1q antibiody ->
Immune Complex Formation - Complement Pathway Activation (C3a and C5a)
Pathophysiology of Hypocomplementemic ... TerrierBen #Hypocomplementemic
Causes of Hypocomplementemia - CHAMPS Mnemonic
C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis
H - Heavy Chain deposition
Causes of Hypocomplementemia ... @AnnKumfer #Hypocomplementemia
IgG4-associated conditions

Diagnosis of IgG4 disease is depending on BIOPSY. While IgG4 levels can be informative (and
IgG4-associated ... Diagnosis of IgG4 ... While IgG4 levels ... inflammation of IgG4 ... #IgG4 #conditions
Manifestations of IgG4-related disease by organ system.
 The most common primary disease features are indicated in
Manifestations of IgG4 ... #IgG4 #Manifestations
Expected DAT Results in Autoimmune Hemolysis
DAT Negative Hemolysis
 • DAT Poly ( - ), IgG (
DAT Poly ( - ), IgG ... DAT Poly ( + ), IgG ... DAT Poly ( + ), IgG ... Poly ( +/- ), IgG ... DAT Poly ( + ), IgG
Autoimmune Hemolytic Anemia (AIHA) Pathophysiology - Warm vs Cold AIHA
Warm AIHA:
 - 70%; IgG mediated
 -
AIHA: - 70%; IgG ... mediated - DAT: IgG ... mediated - DAT: IgG
Aortitis - Differential Diagnosis Framework
Non-Infectious Aortitis:
 • Vasculitides: Giant Cell Arteritis (GCA), Takayasu's arteritis, Cogan's Syndrome,
Rheumatic Diseases: IgG4 ... Related Disease (IgG4
Eosinophils (Eos) and hypereosinophilia (HE)
Caused by eosinophilopoietic cytokines
Common causes:
 - Drug reaction
 - Helminth infection
 -
rheumatoid arthritis, IgG4