[A case of Sjögren's syndrome at the age of 91 with a lymphoepithelial lesion].

A 91 year old woman with Sjögren's syndrome who developed a lymphoepithelial lesion and showed an active state of the disease is described. Since June, 1990, the patient had been complaining of dry eyes and mouth and a left submandibular tumor (1.0 x 1.5 cm in diameter). A biopsy of the tumor revealed lymphoepithelial lesions in the salivary gland. Mild anemia (Hb 9.6 g/dl) and an elevated erythrocyte sedimentation rate (80 mm/hr) were noted. The gamma-globulin level was 2.7 g/dl, IgG 2789 mg/dl, IgA 469 mg/dl, RAHA x80, antinuclear antibody x20, anti-SS-A x256, thyroid test x400 and microsome test x102400. The Schirmer's test showed decreased tear secretion (Lt. 3mm, Rt. 9mm) and keratoconjunctivitis sicca were noticed by an ophthalmologist. Salivary scintigraphy revealed decreased uptake and slow excretion of the isotope (grade II). A biopsy of a minor salivary gland showed periductal lymphocytic infiltration and acinar cell destruction. Immunohistochemical analyses revealed the cross-reactive idiotype of a monoclonal rheumatoid factor which is associated with a patient with Sjögren's syndrome, in the infiltrating lymphocytes and plasma cells of the minor salivary glands, but not in the lymphoepithelial lesions of the left submandibular gland. This was a rare case concerning a 91-year old patient with Sjögren's syndrome who developed a lymphoepithelial lesion and showed high activity in the serum and gives us valuable information on the relationship between aging and autoimmunity.