Autopsy Findings of Primary Sjögren’s Syndrome with End-Stage Renal Failure

Shingo Kubo, MD, 1-1 Iseigaoka, Yahatanishi-ku, Kitakyushu 807 (Japan) Dear Sir, We describe a case of primary Sjögren’s syndrome (pSS) with end-stage renal failure. A 51year-old woman experienced dry mouth, bone pain and general fatigue. She was admitted to another hospital, where pSS was diagnosed on the basis of Shirmer’s test and salivary gland biopsy. Tests showed deterioration of renal function, with a serum creatinine level of 2.3 mg/dl. She refused to accept specific forms of medication such as steroid therapy, and no renal biopsy was performed. She was admitted to our hospital 3 years later, suffering from the same complaints. She exhibited marked metabolic acidosis and hypo-calcemia. Laboratory parameters included hemoglobin 7.6 mg/dl, white blood cell count 8,600/mm3 (eosinophilia 11%), blood urea nitrogen 27 mg/dl, creatinine 3.6 mg/dl, calcium 6.9 mg/dl, inorganic phosphate 2.9 mg/dl, IgG 3,090 mg/dl, ß2-microglobulin (ß2MG) 29.1 mg/l (0.6-2.1), base excess -12.4 mmol/l. Complement components were normal. Rheumatoid factor was positive, while anti-nuclear antibody, lupus erythematosus (LE) cell, LE test, and anti-DNA antibodies were negative. Urine protein excretion was 0.5 g/ day with renal tubular proteins. Creatinine clearance was 9.6 ml/min. A bone marrow biopsy revealed normocellular marrow with slight enhancement of eosinophils. The patient received oral drugs to resolve the renal tubular acidosis and hypocalcemia. Administration of prednisolone 5 mg/day improved eosinophilia, erythrocyte sedimentation rate, and symptoms of bone pain. Renal function remained stable for 5 years, but then acute exacerbation of renal insufficiency occurred with a fever of unknown etiology, severe ileus, and pathological bone fractures. Maintenance hemodialysis (mHD) was continued for 2 years. However, polyuria was persistent because of renal tubule dysfunction during mHD. Finally, the patient died of bacterial peritonitis caused by perforation of the small intestine, which was rich in lymphatic glands. Autopsy revealed multiple ulcers on the tongue and caries of the teeth, keratoconjunc-tivitis, atrophic salivary glands, chronic eso-phagitis, chronic hepatitis with hepatomegaly (1,740 g), old pulmonary tuberculosis, and atrophic kidneys (30 g/30 g). A microscopy study revealed that almost all extraglandular organs were replaced by fatty tissue. Renal tubules were almost totally destroyed and marked infiltration of inflammatory cells, predominantly lymphocytes, plasma