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1 ; IgA 1 A bs tract Primary Biliary Cirrhosis Associated with Multiple Renal Abscess and IgA Nephropathy : A Case Report Jung Woo Shin, M.D., Il Han Song, M.D., Myoung Ju Ki, M.D., Chang Young Lim, M.D., and Jong Tae Cho, M.D. Department of Internal Medicine, Dankook University College of Medicine, Cheonan, Korea Primary biliary cirrhosis is a chronic progressive disease most often affecting women. It is characterized by the progressive destruction of the small intrahepatic bile duct s with portal inflammation leading to hepatic fibrosis and cirrhosis. Autoimmune diseases such as Sjogren' s syndrome, rheumatoid arthritis, dermatomyositis, CREST syndrome, and autoimmune thyroiditis may be found in up to 80% of the patients. Primary biliary cirrhosis is also associated with renal diseases such as distal renal tubular acidosis, bacteriuria and glomerulopathy, but there is no case report of primary biliary cirhosis with renal manifestations in Korea. We are reporting a case of primary biliary cirrhosis with multiple renal abscess and IgA nephropathy with a review of the literature.(kor ean J Hepat ol 2000;6: ) Key Words : Primary biliary cirrhosis, IgA nephropathy, Renal abscess ; ; Abbreviations: ALT, alanine aminotransferase; AST, aspartate aminotransferase; C4, complement 4; - GTP, -glutamyltranspeptidase; IgA, immunoglobulin A; IgM, immunoglobulin M; IgG, immunoglobulin G; HBs Ag, hepatitis B surfase antigen; anti- HBs, anti- hepatitis B surfase antibody; anti- HCV, anti- hepatitis C virus antibody; UDCA, ursodeoxycholic acid; CREST, calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia. :,, 16-5, ( ) , Phone: ; Fax: ; E- mail Kuihs@chollian.net

224 The Korean Journal of Hepatology : Vol. 6. No. 2. 2000.. 11.6 g/ dl, 15,050/, 102,000/, AST/ ALT 60/ 37 IU/L, alkaline phosphatase 187 IU/L,.

2 224 The Korean Journal of Hepatology : Vol. 6. No g/ dl, 15,050/, 102,000/, AST/ ALT 60/ 37 IU/L, alkaline phosphatase 187 IU/L,. - glutamyltranspeptidase ( - GTP) 311 IU/L, bilirubin (total/ direct) 5.47/ 4.16 mg/ dl,,,, CREST ,, (. 1) (upper pole) 3.5 cm, (lower pole) 4.5 cm,.,,. 2 Klebsiella pneumoniae. 5%,, Goodpasture bilirubin, alkaline phosphatase. 3,.,,, HBs Ag, anti- HBs anti- HCV,. 4,5 (anti- mitochondrial Ab) IgA (anti- nuclear Ab), 1 (anti- microsomal Ab). (anti- smooth muscle Ab) ,. 130/80 mmhg, 80 /, 38.7, 24 /.. 2 Figure 1. Abdominal CT showed multiple renal abscess in upper and lower pole of the left kidney.

264 mg/ dl (60-370 mg/ dl), 1- antitrypsin 425 mg/ dl (140-470 mg/ dl), ceruloplasmin 26.9 mg/ dl (15-60 mg/ dl).,, 30 65, Mallory 100 54 3. ( 2A, 2B).

3 Jung Woo Shin, et al. Primary Biliary Cirrhosis Associated with Multiple Renal Abscess and IgA Nephropathy : A Case Report 225 IgG 2,469 mg/ dl (800-1,700 UDCA mg/ dl), IgA 1,344 mg/ dl ( mg/ dl), IgM. 264 mg/ dl ( mg/ dl), 1- antitrypsin 425 mg/ dl ( mg/ dl), ceruloplasmin 26.9 mg/ dl (15-60 mg/ dl).,, 30 65, Mallory ( 2A, 2B). ursodeoxycholic acid (UDCA) bilirubin alkaline phosphatase..,,, 50 60% ,, 1 g,, immunoglobulin bilirubin, alkaline 2 phosphatase - GTP. AST ALT. IgA IgM ( 3),, IgA.. bilirubin Figure 2A. Microscopic findings of the liver revealed expansion of periportal fibrosis into a network of connective tissue scars and proliferation of smaller bile ductules (Masson-Trichrome stain, X20). Figure 2B. Microscopic findings of the liver revealed intensely eosinophilic intracellular Mallory bodies in hepatocytes (large arrows) and less prominent lymphocytic infiltrations in the portal tract (small arrow) (H&E stain, X200).

226 6 2 2000 alkaline phosphatase. bilirubin, alkaline phosphatase - GTP.. 3,. IgM 80%,, CREST,,. 1,,.. 10 Figure 3.

4 alkaline phosphatase. bilirubin, alkaline phosphatase - GTP.. 3,. IgM 80%,, CREST,,. 1,,.. 10 Figure 3. Immunofluorescent study revealed positive staining for IgA in the glomerular mesangial area (IgA stain, 200).. 11,12 Morreale % C4 factor B.. E. coli 1 35%. 95% pyruvate dehydrogenase complex E2 2 57% CD8 T. A.. 8, Scheuer Ludwig , 2 (membranous glomerulonephritis), (focal glomeru-. 3, 4 lonephritis) IgA

5 4. IgA IgA. IgA IgA IgA IgA. 13 IgA IgA 60% IgM IgA. cyclosporin, colchicine, methotrexate, UDCA %. 1. Neuberger J. Primary biliary cirrhosis. Lancet 1997;350: Kaplan MM. Primary biliary cirrhosis. N Engl J Med 1996;335: Henry CB, Bodenheimer JR. Primary biliary cirrhosis. In : Haubrich WS, Schaffner N, Beck JE, eds. Bockus gastroenterology, Vol 3. 5th ed. Philadelphia: WB Saunders, 1995; ;32: ,, ;50: Roll J, Boyer JL, Barry D, Klatskin G. The prognostic importance of clinical and histologic features in asymptomatic and symptomatic primary biliary cirrhosis. N Engl J Med 1983;308: Heseltine L, Turner IB, Fussey SP et al. Primary biliary cirrhosis; Quantitation of antibodies to purified mitochondrial enzymes and correlation with disease progression. Gastroenterology 1990;99: Hoffmann R, Grewe M, Estler HC, Schulze- Specking A, Decker K. Regulation of tumor necrosis factor- alpha- mrna synthesis and distribution of tumor necrosis factor- alpha- mrna synthesizing cells in rat liver during experimental endotoxemia. J Hepatol 1994;20: Scheuer PJ. Liver biopsy interpretation. 2nd ed. Baltimore; Williams and Wilkins, Pares A, Rimola A, Bruguera M et al. Renal tubular acidosis in primary biliary cirrhosis. Gastroenterology 1981;80: Morreale M, T sirigotis M, Hughes MD, Brumfitt W, McIntyre N, Burroughs AK. Significant bacteriuria has prognostic significance in primary biliary cirrhosis. J Hepatol 1989;9: Burroughs AK, Rosenstein IJ, Ebstein O, Hamilton- Miller JM, Brumfitt W, Sherlock S. Bacteriuria and primary biliary cirrhosis. Gut 1984;25: Buttler P, Hamilton-Miller JM, McIntyre N, Burroughs AK. Natural history of bacteriuria in women with primary biliary cirrhosis and the effect of antimicrobial therapy in symptomatic and asymptomatic groups. Gut 1995;36: ,. IgA nephropathy.. :. 1999;

6 228 The Korean Journal of Hepatology : Vol. 6. No Batts KP, Jorgensen RA, Dickson ER, Lindor KD. Effect of ursodeoxycholic acid on hepatic inflammation and histological stage in patients with primary biliary cirrhosis. Am J Gastroenterol 1996;91:

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<30382EC0C7C7D0B0ADC1C22E687770> 대한내과학회지: 제 76 권 제 2 호 2009 의학강좌-개원의를 위한 모범처방(Current Clinical Practice) 간기능검사의 이해와 적용 인제대학교 의과대학 일산백병원 내과학교실 김 경 아 Understanding and application of liver function tests Kyung-Ah Kim, M.D. Department of

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歯간학회지6-2.PDF