Hypocomplementemic urticarial vasculitis
ORPHA:36412· ICD-10 M31.8
Definition
A rare immune complex-mediated small vessel vasculitis characterized by urticaria and hypocomplementemia (low C3, C4 and/or C1q), and usually associated with circulating anti-C1q autoantibodies. Arthritis, pulmonary disease, ocular inflammation are common systemic manifestations.
- Prevalence
- Unknown
- Inheritance
- Autosomal recessive, Not applicable
- Age of onset
- Adult, Childhood