6 2 2000 ; 223-228 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;6:223-228) Key Words : Primary biliary cirrhosis, IgA nephropathy, Renal abscess 2000 3 13 ; 2000 4 26 ; 2000 5 15 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, ( ) 330-715, Phone: 0417-550- 3924; Fax: 0417-554- 1438; 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,. - glutamyltranspeptidase ( - GTP) 311 IU/L, bilirubin (total/ direct) 5.47/ 4.16 mg/ dl,,,, CREST. 1. 5 38.5,, (. 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). 64 4.,. 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.
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 (85-450 mg/ dl), IgM. 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). ursodeoxycholic acid (UDCA) bilirubin alkaline phosphatase..,,, 50 60% 6. 24 12,, 1 g,, immunoglobulin. 16. 6 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. Immunofluorescent study revealed positive staining for IgA in the glomerular mesangial area (IgA stain, 200).. 11,12 Morreale 11 144 19% C4 factor B.. E. coli 1 35%. 95% pyruvate dehydrogenase complex E2 2 57%. 13. 7 CD8 T. A.. 8, Scheuer Ludwig 9. 1. 11, 2 (membranous glomerulonephritis), (focal glomeru-. 3, 4 lonephritis) IgA
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