10 results
Pathophysiology of Hypocomplementemic Urticarial Vasculitis

C1q/anti-C1q antibiody ->
Immune Complex Formation - Complement Pathway Activation (C3a and C5a)
Pathophysiology of Hypocomplementemic ... Urticarial Vasculitis ... Complex Formation - Complement ... Formation -> Vasculitis ... TerrierBen #Hypocomplementemic
Causes of Hypocomplementemia - CHAMPS Mnemonic
C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis
H - Heavy Chain deposition
Causes of Hypocomplementemia ... Chain deposition disease ... @AnnKumfer #Hypocomplementemia ... #Low #Complement
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
, proteinuria, rapidly ... often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... (Granulomatous disease ... against MPO • Complement
IgG4-Related Disease
Clinical history:  Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor
4 phenotypes:
 •
IgG4-Related Disease ... pseudotumor 4 phenotypes ... rheumatoid factor), ± hypocomplementemia ... No necrosis, No vasculitis ... IgG4 #Related #Disease
Rapidly Progressive Glomerulonephritis (RPGN)
RPGN has three primary pathophysiologic causes differentiated by immunofluorescence

Immune Complex Mediated (granular staining):
Rapidly Progressive ... staining): • Low Complement ... involvement: Anti-GBM Disease ... Glomerulonephritis #Vasculitis ... differential #diagnosis #algorithm
Vasculitis - Differential Diagnosis Framework

When to Consider Vasculitis:
• Purpura, ischemic skin lesions
• Mononeuritis multiplex
• Hematuria, proteinuria,
, proteinuria, Rapidly ... atherosclerotic disease ... Pauci immune GN (rapidly ... Elevated AST ALT • Hypocomplementemia ... ) • Hypocomplementemic
Pulmonary Renal Syndromes and Mimics - Differential Diagnosis Framework
Diffuse alveolar hemorrhage + rapidly progressive glomerulonephritis
90%:
 -
alveolar hemorrhage + rapidly ... - Anti-GBM Disease ... anti-GBM) 10%: - Low Complement ... - Normal Complement ... , Behcet's Disease
Hemolytic Anemia - Differential Diagnosis Algorithm
 • Sickle cells - Consider sickle cell disease (diverse genotypes):
Differential Diagnosis Algorithm ... Consider sickle cell disease ... (diverse genotypes ... cardiac valve, vasculitis ... Differential #Diagnosis #Algorithm
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 ... demyelinating disease ... ) • Cutaneous vasculitis ... : 2% Systemic Vasculitis
Monoclonal Gammopathy of Renal Significance (MGRS)

MGRS
• MGRS refers to kidney disease caused by nephrotoxic monoclonal immunoglobulins
refers to kidney disease ... end-stage kidney disease ... proteinuria with rapid ... tubulopathy) - Complement ... formation causing vasculitis