A Japanese girl aged 8 years who presented with a 2-month history of uveitis subsequently developed tubulointerstitial nephritis. A percutaneous renal biopsy revealed massive interstitial mononuclear cell infiltrates consisting of CD4-positive T cells. Despite administration of topical corticosteroids, the ocular symptoms persisted. Systemic corticosteroid therapy dramatically reduced the ocular symptoms and urinary beta2-microglobulin (beta 2MG) concentration. However, reducing the prednisolone dosage induced recurrence of uveitis associated with increased levels of urinary beta 2MG. The CD4-positive T cell infiltration persisted in the second renal biopsy performed 6 months after the first renal biopsy. These observations suggest that the interstitial cell infiltration persists for a relatively long time in a proportion of patients with tubulointerstitial nephritis and uveitis syndrome (TINU). Although the renal outcome of TINU has been reported to be favorable, prolonged interstitial cell infiltration may affect long-term renal outcome. Selected patients with TINU should be followed with close observation.

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Mizoribine (MZR), a novel selective inhibitor of inosine monophosphate dehydrogenase in the de novo pathway, whose mode of action is very similar to that of mycophenolate mofetil, has been successfully applied without serious adverse effects for the treatment of renal diseases. We have previously reported the efficacy and safety of a new MZR treatment regimen, namely, oral MZR intermittent pulse therapy, which we examined based on the observation that it might show superior efficacy to the conventional daily low-dose MZR regimen on account of the higher peak serum MZR levels, in selected patients with lupus nephritis. Two Japanese patients with severe lupus nephritis (WHO class IV-G) who were administered long-term intermittent MZR pulse therapy, and in whom pre- and post-treatment renal biopsies were reported. Post-treatment renal biopsy confirmed the marked attenuation of histologic progression by the treatment. These clinical observations may lend further support, from the histologic standpoint, to the efficacy of long-term MZR intermittent pulse therapy for selected patients with active lupus nephritis.

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