The 2 patients presented here showed the typical signs of hypocomplementemi
c urticarial vasculitis syndrome (HUVS), During follow-up, there was an inv
erse correlation between anti-C1q autoantibody titer and C1q antigen concen
tration in serum in both patients over a period of 2 years, The first patie
nt had nephritis characterized by immune deposits in glomeruli and around t
he tubules, The histological findings, C1q deposits, and presence of tubulo
reticular inclusions in capillary endothelial cells suggested a disease pro
cess identical to systemic lupus erythematosus (SLE), The second patient, a
fter a lag phase of 2 years, fulfilled a fourth American College of Rheumat
ology criteria for SLE when she developed anti-double-stranded DNA antibodi
es. HUVS and SLE overlap, and the criteria for identifying HUVS as an entit
y distinct from SLE are lacking. (C) 1999 by the National Kidney Foundation
, Inc.