Fanconi症候群を合併したSjögren症候群の1例

Abstract

A 73-year-old female with Sjögren's syndrome, Fanconi's syndrome, distal and proximal tubular acidosis is reported. She was admitted to The Orthopedic Department of our hospital for arthralgia and was diagnosed as having osteomalacia. She was later transferred to our department for the investigation of proteinuria associated with elevation of serum ALP, TTT and ZTT. Laboratory tests at that time revealed alkaluria, isosthenuria, hypokalemia, hyperchloremia and metabolic acidosis. Renal tubular acidosis, with both distal and proximal tubular dysfunction, was found in this case. In view of glucosuria without hyperglycemia, hypophosphatemia, hyperphosphaturia, and aminoaciduria, the diagnosis of Fanconi's syndrome was made. The diagnosis of Sjögren's syndrome was confirmed on the basis of a positive Schirmer's test, marked corneal staining from rose bengal dye, sialograph showing the destructive stage and marked infiltration by lymphocytes of the salivary gland. The renal biopsy showed a diffuse interstitial mononuclear cell infiltration, an interstitial fibrosis and tubulitis. From this case, it is concluded that the renal manifestation of Sjögren's syndrome can be associated with variable renal interstitial abnormalities.