CLINICAL GASTROENTEROLOGY

Autoirninune chronic active hepatitis (lupoid hepatitis) and prirnary sclerosing cholangitis in two young adult feinales GERALD Y. MINUK, MD, FRCP(C), LLOYD R. SUTHERLAND, MD, FRCP(C), S. CHRIS PAPPAS, MD, FRCP(C),)AMES K. KELLY, MD, FRCP(C), SUE E. MARTIN, MD

ABSTRACT: Autoimmune chron ic active hepatitis (CAH) and primary sderosing cholangitis (PSC) arc chronic diseases of the hepatobiliary system that have many cli nical, immunologic and genetic features in commo n. Despite these similarities, there are few reports of the two diseases coexisting. Two young women with clinical, biochemical, serologic, radiologic and histologic findings compatible with both autoimmune CAH and PSC are described. The observation that there may be a striking overlap in the featu res of these two diseases and recent improvements in diagnostic imaging of the biliary tract suggest that the association of these two diseases in the same individual may be more common than is presently appreciated. Can J Gastroentero l 1988; 2(1): 22-27 Key Words: Autoimmune chronic active hepatitis, lnf/.ammatory bowel disease, Primary sclerosing cholangitis. Department of Medicine, University of Ca/gar.•, Calgary, Alberta, Canada and Lit•er Disease Section, Digestive Diseases Branch NIADDK, National Institutes of Health, Bethesda, Maryland,

USA Correspondence: Dr Gerald Y. Minuk, Liver Diseases Unit - H604, University of Manitoba, Health Sciences Centre, 820 Sherbrook Street, Winnipeg, Manitoba R3A 1R9 Received for (Jublication October 1987. Accepted December 1987

22

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UPOID OR AUTOIMMUNE CHRON-

ic active hepatitis (CAH) and primary sclerosi ng chola ngitis (PSC) are chronic diseases of the hepatobiliary system chat h ave many features in common. Boch diseases affect young adults and frequently progress to cirrhosis and death from liver failure (16). Each is marked by h ypcrgammaglobulinemia (5, 7), elevated levels of immune complex-like activity (8,9) and the presence of autoancibodies (5, 10-13). Patients with both disorders have an increased incide nce of ocher 'autoimmune' diseases including inflammatory bowel disease (2, 13-16). Sixty to 80% of patients with autoimmune CAH or PSC are HLA B8 positive, whereas only 20% of th e general CAN ] GASTROENT EROL

Autorrnrnune CAH and PSC

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Figure 1) Endo1cop1c cholang1ogram from ulle one showing a long 1mccure of the common d11ct mrh slighr proximal dilacarion and numerous strictures at dw let'CI of common duct

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(5,17,18). The high incidence of HLA B8 raises the possibility char the diseases may share common pathogenic mechanisms. The coexistence of autoimmune CAH and PSC would be compatible with this possibility. Despite the overlapping features of these diseases, there is a paucity of reports documenting their coexistence (o,19). In the present report two paucnts with features of both autoimmune CAH and PSC arc described, emphasizing the problems encountered in establishing these diagnoses.

nodu lar with prominent lymphadenopathy at the porta hepatis and inflammatory debris in the common bile duce. An operanve cholang1ogram revealed a distorted common bile dun compatible with sclerosmg cholangim (Figure I). A liver biopsy demonscratetl nonsuppurativc obliterative cholang1tis. On further review of the patient\ history, she recalled occasional episodes of prolonged nonbloody diarrhea 111 the recent past. She took no medications other than oral con tracepnvcs and consumed little nlcohol. Hashimoco's thyroiditis hatl been diagnosed ac age six. The patient's family history reveakd that her morher had diabetes mel11tus, myxetlcma anJ perniuous anemi,1, and her father and paternal unde haJ cehac tlise.ise. Physkal examination showctl noperipheral signs of d1rot11l livn d1seasL'. The liver and ,plecn were not enlarged. L1hurnwry 111vest1gations at that time revealed hemoglobin of 12.1 g 100 ml, white blootl cell wunt of 13,200/mm with 60°0 polymorphonudear leukocytes. Biliruh111 was 3.5 mg/ JL (normal, less than 1.4); al kaline phosphatase 946 iu / L (normal, less than 115); and aspartate ammotransferase (AST) 35 iu/ L (normal, bs

th.in 40). Protein cleurophores1s anti 1mmunoglohulin quantunuon were within normnl limits. t\mismooth muscle nmibody, nnt1m1tochondnal anribody and \'1ral hepatitis serologv were negan\·e. The patient was renssessetl one year later when shl' Je\·elopetl bloodv tliarrhea. Physirnl e;..nmrnation was unchangetl but sigmoidoscopv revealed a granular mums a wnh fr1ahiliry. Rectal biopsy was cons1srenr wuh tnflammarorv bowel di~easc, most ltkelv ulcerative colitis, anJ this was further supported by an air contrnst barium enema. A repeat liver biop:-v showed evidence of cholnngitis with focal portal scarring compatihle with early L1rrhos1s. Endoscopic retrograde Lholang1opnmreawgraphy (ERCP) showed no l'v1denLe of extrahepatic obsrruLUOn hut the upper poruon of che common hile tluu was again JiscorteJ, suggesuve of sclerosing d10langitis. The p:itient was started on sulfosal.1zine for ukerativc coitus and Jid reasonably well for the next year. She was reassessed in late 1982 for rL'currenc ep1gastric pain. Labor:itory nssessment at that time revealed alkaline phosphatase of 762 iu/ L and AST of 366 1u L. Antinuc.lcar factor was pos1t1ve at a l: 160 mre and previously negauve antismooth muscle

CASE ONE A 30-year-old female presented in 1978 with a five week history of abdominal pain and recent onset of jau nd1ce and pruricus. A clinical diagnosis of choledocholithiasis was made and laparotomy was performed. Ar surgery no stones were found in t he biliary tract but the liver was noted to be Vol. 2 No. I. March 1988

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Figure 2) The porwl triad.1 display a modcraceh hem"! mfiltracr of h-mphc,c.te\ ancl monc,s-.t