section name header

Info


A. Characteristicsnavigator

  1. Recurrent urticarial vasculitis
  2. Arthralgias / Arthritis
  3. Hypocomplementemia
  4. Other Findings
    1. Angioedema
    2. Ocular inflammation
    3. Glomerulonephritis
    4. Obstructive lung disease - often with dyspnea, worsened with smoking
    5. Anemia - probably of chronic disease (inflammatory) type

B. Pathologynavigator

  1. Unknown Cause
  2. Serum anti-C1q Antibodies
    1. Mainly in hypocomplementemic urticarial vasculitis
    2. May be associated with SLE
  3. Disease Associations
    1. Systemic Lupus Erythematosus (SLE)
    2. Hepatitis B or C Virus Infection
    3. Inflammatory Bowel Disease
    4. Plasma cell dyscrasia (paraprotein associated urticarial vasculitis)
  4. Relationship to SLE
    1. Patients often ANA+
    2. Complement levels depressed, especially C4 levels
    3. Renal dysfunction is present in many patients

C. Diagnosisnavigator

  1. Skin biopsy required
  2. Reduced serum complement levels

D. Treatment navigator

  1. Intravenous Immunoglobulin (IVIg) 1-2gm/kg iv
  2. High dose glucocorticoids: prednisone 1-2mg/kg/day initially
  3. Cytotoxic Agents - may be used for severe renal or ocular disease

References navigator

  1. Wisnieski JJ, Baer AN, Christensen J, et al. 1995. Medicine. 74(1):24 abstract
  2. Greaves M. 2000. J Allergy Clin Immunol. 105(4):664 abstract