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Hereditary nephritis is a well recognized disease, but its clinical manifestations and patterns of inheritance are variable. We report a family with hereditary renal disease in which the morphological lesion is interstitial nephritis and the pattern of inheritance autosomal dominant. In contrast with previous reports, microscopic/macroscopic hematuria was not a consistent marker for renal involvement. The family also demonstrates the inheritance of rheumatoid arthritis in an autosomal dominant pattern, not temporally related to the interstitial renal disease.