Hypocomplementaemic urticarial vasculitis, angio-oedema and 'lupus-like' disease: association with C4B null allele.
A patient who presented with urticarial vasculitis, recurrent angio-oedema and gastro-intestinal symptoms in association with a 'lupus-like' disorder is documented. Other 'auto-immune' phenomena such as Sjogren's-like syndrome and polyarthritis were present in association with antibodies to RNP, low C4 and a null allele was demonstrated at the C4B locus. Response to varied therapies was poor and unsustained.
D000799:Angioedema / Q000235:genetics; D001327:Autoimmune Diseases / Q000235:genetics* / Q000628:therapy; D003131:Combined Modality Therapy; D003181:Complement C4 / Q000235:genetics*; D015935:Complement C4b; D003165:Complement System Proteins / Q000032:analysis; D003240:Connective Tissue Diseases / Q000235:genetics* / Q000628:therapy; D005260:Female; D006801:Humans; D008875:Middle Aged; D014581:Urticaria / Q000235:genetics; D014657:Vasculitis / Q000235:genetics
D003181:Complement C4; D015935:Complement C4b; D003165:Complement System Proteins