Progressive interstitial renal fibrosis due to Chinese herbs in a patient with calcinosis Raynaud esophageal sclerodactyly telangiectasia (CREST) syndrome

A 58-year-old woman with calcinosis Raynaud esophageal sclerodactyly telang iectasia (CREST) syndrome presented with slowly progressive renal dysfuncti on. She was normotensive with normal plasma renin activity and lacking symp toms of vasculitis. Mild proteinuria was of tubular origin, but serological tests and an absence of sicca symptoms excluded the possibility of Sjogren 's syndrome. Light microscopic study of renal biopsy showed interstitial fi brosis with ectasia and degeneration of proximal tubule and lymphocyte infi ltration. There were no remarkable changes in the glomeruli. Chromatographi c analysis of the Chinese herbs regimen that she had been taking for severa l years demonstrated aristolochic acid. She was diagnosed as Chinese herbs nephropathy. Therapy with oral prednisolone was markedly effective in impro ving renal function and anemia. To our knowledge, this is the first report of Chinese herbs nephropathy complicating connective tissue disease. It is important to consider the possibility of Chinese herbs nephropathy when pat ients treated with Chinese herbs develop renal dysfunction.