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
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
Vasculitis and Vasculitides ... often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... against MPO • Complement ... #rheumatology #classification
Recommended initial testing for patients being evaluated for glomerular disease 

24-hour urine collection - Quantify proteinuria.
Recommended initial testing ... or alternative complement ... for ANCA-related vasculitis ... #glomerular #testing ... #workup #diagnosis
IgG4-Related Disease
Clinical history:  Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor
4 phenotypes:
 •
rheumatoid factor), ± hypocomplementemia ... No necrosis, No vasculitis ... management #phenotypes #workup
Recommended initial testing for patients being evaluated for glomerular disease
1) 24-hour urine collection - Quantify proteinuria.
Recommended initial testing ... or alternative complement ... for ANCA-related vasculitis ... glomerular #disease #workup ... #nephritis
SLE (Systemic Lupus Erythematosus)

Suspect:
Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
anti-Smith Ab, low complements ... Skin Disease Classification ... ANCA-associated vasculitis ... • Mesenteric vasculitis ... RNP antibodies, Complement
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 ... • Interstitial nephritis ... : 2% Systemic Vasculitis
IGG-4 RELATED DISEASE

WHAT?
 • A chronic, immune-mediated fibroinflammatory disease with tumefactive infiltration of IgG4+ plasma cells
aortitis, mimics vasculitis ... Tubulointerstitial nephritis ... - ↑ IgE, ↓ complement ... • Classification
Monoclonal Gammopathy of Renal Significance (MGRS)

MGRS
• MGRS refers to kidney disease caused by nephrotoxic monoclonal immunoglobulins
Clinical Clues: • Nephrotic-range ... Diagnostic Workup ... guide subtype classification ... tubulopathy) - Complement ... formation causing vasculitis