S-477 Primary sjogren``s syndrome diagnosed from extraglandular manifestation = S-477 Primary sjogren``s syndrome diagnosed from extraglandular manifestation

다국어 초록 (Multilingual Abstract)

Primary Sjogren's syndrome (pSS) is a autoimmune disorder that primarily affects epithelial tissue characterized by lymphoplasmacytic infiltration of the exocrine glands. The patients may develop a extraglandular manifestation such as tubulointerstitial nephritis, hepatitis, or myositis due to systemic involvement of pSS which mark disease prognosis and can cause mortality. Recently, we experienced a patient who had acute kidney injury due to tubulointerstitial nephritis, autoimmune hepatitis and myositis, finally diagnosed as pSS. The 73 year-old female presented with oliguria, abdominal distension and edema. She had a diabetic nephropathy and hypertension. Abdomen CT scan showed advanced liver cirrhosis with ascites. Hepatocellular pattern of hepatitis and acute kidney injury had not improved with conservative care, therefore we biopsied the liver and kidney. Kidney showed tubulointerstitial nephritis, infiltrated with CD3, CD8, CD68, CD138 positive cells in immunohistochemistry. Liver showed chronic hepatitis with cirrhosis, marked plasma cell infiltration in portal area and serologic markers of hepatitis B and C were all negative, hence, we presumably diagnosed her as autoimmune hepatitis and started oral prednisolone. She also complained progressive weakness of lower extremities and bone scan showed increased uptake in both thighs and the symptom accompanied with elevated muscle enzyme. We also biopsied thigh muscle and it showed mild inflammatory cell infiltration in the endomysium, suggestive of inflammatory myopathy. Anti SS-A (Ro) and SS-B (La) which were checked during the work up of autoimmune hepatitis, were strong positive, therefore we started to have a suspicion for pSS. Salivary gland scan showed markedly impaired function of both submandibular glands, Shirmer test was positive, and lip biopsy showed focal lymphocytic sialadenitis. Finally, we diagnosed her as pSS with kidney, liver, and muscle involvement.She was discharged with fully recovered renal function, normalized hepatic enzyme and partially recovered muscle power continued on steroid treatment.