9 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 ... @AnnKumfer #Hypocomplementemia ... #Low #Complement
Rapidly Progressive Glomerulonephritis (RPGN)
RPGN has three primary pathophysiologic causes differentiated by immunofluorescence

Immune Complex Mediated (granular staining):
Rapidly Progressive ... staining): • Low ... With associated pulmonary ... Glomerulonephritis #Vasculitis ... differential #diagnosis #algorithm
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
proteinuria, rapidly progressing ... often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... against MPO • Complement ... (Low levels mixed
Pulmonary Renal Syndromes and Mimics - Differential Diagnosis Framework
Diffuse alveolar hemorrhage + rapidly progressive glomerulonephritis
90%:
 -
Pulmonary Renal ... hemorrhage + rapidly progressive ... anti-GBM) 10%: - Low ... Complement Levels ... - Normal Complement
Vasculitis - Differential Diagnosis Framework

When to Consider Vasculitis:
• Purpura, ischemic skin lesions
• Mononeuritis multiplex
• Hematuria, proteinuria,
proteinuria, Rapidly progressing ... is 50 years • Progressive ... immune GN (rapidly progressive ... Elevated AST ALT • Hypocomplementemia ... ) • Hypocomplementemic
Pulmonary Renal Syndromes - OnePager Summary
Autoimmune ANCA vasculitis (AAV): GPA (granulomatous with polyangiitis), EGPA (eosinophilic granulomatosis
Autoimmune ANCA vasculitis ... palpable purpura 2/2 vasculitis ... non-specific, ESR usually low ... hematuria, RBC casts, low ... Path: rapidly progressive
Sjogren's Syndrome Overview

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

Autoimmune exocrinopathy multisystemic disease
Cytopenia: 2% • Hypocomplementemia ... Disease: • 10%-15% Pulmonary ... , INC risk PE, Pulmonary ... ) • Cutaneous vasculitis ... : 2% Systemic Vasculitis
Monoclonal Gammopathy of Renal Significance (MGRS)

MGRS
• MGRS refers to kidney disease caused by nephrotoxic monoclonal immunoglobulins
Pathogenesis: How ... Damages Kidneys • Low ... tubulopathy) - Complement ... formation causing vasculitis ... function and prevent progression