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PEDIATRICS Vol. 107 No. 6 June 2001, pp. 1443-1446

EXPERIENCE AND REASON:
Tubulointerstitial Nephritis and Uveitis Syndrome With Autoantibody Directed to Renal Tubular Cells

Received Oct 2, 2000; accepted Dec 11, 2000.

Hitoshi Wakaki

Hisato Sakamoto*

Midori Awazu

* Second Department of Internal Medicine Tokyo Medical and Dental University Department of Pediatrics Keio University School of Medicine Tokyo 160-8582 Japan

The pathogenesis of tubulointerstitial nephritis and uveitis (TINU) syndrome remains unknown, but T cell-mediated immune response has been postulated to play a role. On the other hand, TINU syndrome is characterized by hypergammaglobulinemia and high serum immunoglobulin G (IgG) levels, suggesting an involvement of humoral immunity. We describe a case of TINU syndrome in a 13-year-old girl with multiple tubular dysfunctions including renal glucosuria, tubular proteinuria, phosphaturia, uricosuria, and concentrating and acidifying defect. IgG antibody from her serum was reactive against 125-kDa human kidney protein. Immunofluorescence study using mouse kidney revealed that the antibody was against cortical renal tubular cells. The antibody disappeared as the renal symptoms resolved. We suggest that IgG antibody may contribute to tubular dysfunction in some patients with TINU syndrome.

 Key words:  tubulointerstitial nephritis, uveitis, antibody, kidney, renal tubule, hypergammaglobulinemia.


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