11 results
Causes of Hypocomplementemia - CHAMPS Mnemonic
C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis
H - Heavy Chain deposition
Causes of Hypocomplementemia ... Membranoproliferative glomerulonephritis ... Post/pauci immune glomerulonephritis ... @AnnKumfer #Hypocomplementemia ... #Low #Complement
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
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... , Glomerulonephritis ... against MPO • Complement ... (Low levels mixed
Rapidly Progressive Glomerulonephritis (RPGN)
RPGN has three primary pathophysiologic causes differentiated by immunofluorescence

Immune Complex Mediated (granular staining):
staining): • Low ... Complement: Post-strep ... Idiopathic • Normal Complement ... #Vasculitis #differential ... #diagnosis #algorithm
Pulmonary Renal Syndromes and Mimics - Differential Diagnosis Framework
Diffuse alveolar hemorrhage + rapidly progressive glomerulonephritis
90%:
 -
rapidly progressive glomerulonephritis ... ANCA Associated Vasculitis ... anti-GBM) 10%: - Low ... Complement Levels ... - Normal Complement
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 ... No necrosis, No vasculitis ... with increased IgG4
Vasculitis - Differential Diagnosis Framework

When to Consider Vasculitis:
• Purpura, ischemic skin lesions
• Mononeuritis multiplex
• Hematuria, proteinuria,
, pauci-immune glomerulonephritis ... (Glomerulonephritis ... Elevated AST ALT • Hypocomplementemia ... ) • Hypocomplementemic ... Mononeuropathy, Glomerulonephritis
Cryoglobulinemia Summary

Cryoglobulins:
 • Cryoglobulins are Igs in the serum that precipitate in vitro at temperatures below
Skin ulcers - Glomerulonephritis ... factor level • Low ... serum C4 complement ... • IgA vasculitis ... • Vasculitis
Recommended initial testing for patients being evaluated for glomerular disease 

24-hour urine collection - Quantify proteinuria.
in monitoring low-level ... or alternative complement ... for ANCA-related vasculitis ... workup #diagnosis #glomerulonephritis
Sjogren's Syndrome Overview

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

Autoimmune exocrinopathy multisystemic disease
Cytopenia: 2% • Hypocomplementemia ... ) • Cutaneous vasculitis ... acidosis • NAGMA • Glomerulonephritis ... : 2% Systemic Vasculitis