Review Article
Shizuyo Tsujimura, Akio Kawabe
Abstract
True renal lupus vasculitis (TRLV), a rare form of vascular lesion usually associated with proliferative lupus nephritis (LN), is resistant to conventional treatments and associated with poor renal outcome among renal vasculopathies. Evidence suggests the involvement of P-glycoprotein (P-gp) expressing-activated B cells in the pathogenesis and treatment resistance of TRLV through the production of autoantibodies and direct infiltration into the inflammatory lesion of small vessels. Therapies targeting activated B cells might overcome refractory TRLV. Identification of the subsets of peripheral activated B cells that express P-gp in TRLV patients might help the selection of suitable treatment strategy.