Interstitial nephritis syndrome and uveitis]

A nine year old male patient was admitted to a metropolitan general hospital at Santiago, Chile, because of unexplained severe anemia (hematocrit 18%) and redness of the right eye. Uveitis was confirmed by ophthalmological examination. Laboratory work up showed an erythrocyte sedimentation rate of 101 mm.h, marked nitrogen retention (BUN 91 mg/dl), creatinine clearance was 9 ml/min.1,73 sq m, serum IgG and IgM immune globulin concentrations were 2,368 mg/dl and 263 mg/dl respectively, over the normal range for age, with almost absent serum IgA and reduced T cell populations. Negative rheumatoid factor, antinuclear antibodies and anti smooth muscle antibodies reactions were obtained, together with normal levels of C3 and C4 fractions of complement and normal urine sediment. Renal biopsy specimen included 25 glomerular, one of them was wholly sclerotic, another two had periglomerular fibrosis and the remainder showed only slightly increased mesangial matrix. Heavy mononuclear inflammatory interstitial infiltration, with granulomatous structures including giant multinuclear cells as those seen in foreign body reactions (fig 1) and PAS positive intraluminal tubular crystals were also observed. In spite of steroidal treatment, which was complicated by an inflammatory brain granuloma that healed in coincidence with parenteral antibiotic therapy, the patient evolved toward chronic renal failure. We think that the available evidence strongly suggests an immunological disorder as a very likely explanation for this case.