Does antimitochondrial antibody status affect response to treatment in patients with primary biliary cirrhosis? Outcomes of ursodeoxycholic acid therapy and liver transplantation

W. Ray Kim, John J. Poterucha, Roberta A. Jorgensen, Kenneth P. Batts, Henry A. Homburger, E. Rolland Dickson, Ruud A F Krom, Russell H. Wiesner, Keith Lindor

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Abstract

Approximately 5% to 10% of patients with features otherwise consistent with primary biliary cirrhosis (PBC) lack antimitochondrial antibodies (AMA). Most of these patients have other autoantibodies, a syndrome recently named 'autoimmune cholangitis.' We report our experience in patients with AMA- negative PBC treated with ursodeoxycholic acid (UDCA) and/or liver transplantation (OLT). The study of response to UDCA was performed as follows. While recruiting patients for a previously reported multicenter trial, we identified 8 patients with AMA-negative PBC. The patients were given UDCA and followed up at regular intervals. The characteristics of AMA- negative patients at presentation were similar to those of AMA-positive patients with PBC. The clinical outcomes and sequential liver biochemistries of UDCA treatment were also comparable with those of AMA-positive patients. The study of outcome of OLT was performed as follows. We identified OLT recipients at the Mayo Clinic who had clinical, radiological, and histological features compatible with PBC. Their pretransplant AMA status was determined, and each AMA-negative patient was paired with 2 AMA-positive patients. Of 85 OLT recipients with a diagnosis of PBC, 6 (7.1%) were AMA negative, including 1 who had undergone UDCA therapy. After a median of 36 months of follow-up, graft and patient survival rates and subsequent histological changes (disease recurrence and steroid-resistant or late rejections) were comparable in AMA-negative and -positive PBC patients. In summary, in our experience of 13 AMA-negative PBC patients (including 9 who met the criteria for a diagnosis of autoimmune cholangitis), treatment with UDCA or OLT resulted in similar outcomes to those found in AMA-positive patients. We conclude that AMA status does not affect the response in PBC patients to treatment with UDCA or OLT.

Original languageEnglish (US)
Pages (from-to)22-26
Number of pages5
JournalHepatology
Volume26
Issue number1
StatePublished - Jul 1997
Externally publishedYes

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Ursodeoxycholic Acid
Biliary Liver Cirrhosis
Liver Transplantation
Antibodies
Therapeutics
Cholangitis
Graft Survival

ASJC Scopus subject areas

  • Hepatology

Cite this

Does antimitochondrial antibody status affect response to treatment in patients with primary biliary cirrhosis? Outcomes of ursodeoxycholic acid therapy and liver transplantation. / Kim, W. Ray; Poterucha, John J.; Jorgensen, Roberta A.; Batts, Kenneth P.; Homburger, Henry A.; Dickson, E. Rolland; Krom, Ruud A F; Wiesner, Russell H.; Lindor, Keith.

In: Hepatology, Vol. 26, No. 1, 07.1997, p. 22-26.

Research output: Contribution to journalArticle

Kim, WR, Poterucha, JJ, Jorgensen, RA, Batts, KP, Homburger, HA, Dickson, ER, Krom, RAF, Wiesner, RH & Lindor, K 1997, 'Does antimitochondrial antibody status affect response to treatment in patients with primary biliary cirrhosis? Outcomes of ursodeoxycholic acid therapy and liver transplantation', Hepatology, vol. 26, no. 1, pp. 22-26.
Kim, W. Ray ; Poterucha, John J. ; Jorgensen, Roberta A. ; Batts, Kenneth P. ; Homburger, Henry A. ; Dickson, E. Rolland ; Krom, Ruud A F ; Wiesner, Russell H. ; Lindor, Keith. / Does antimitochondrial antibody status affect response to treatment in patients with primary biliary cirrhosis? Outcomes of ursodeoxycholic acid therapy and liver transplantation. In: Hepatology. 1997 ; Vol. 26, No. 1. pp. 22-26.
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abstract = "Approximately 5{\%} to 10{\%} of patients with features otherwise consistent with primary biliary cirrhosis (PBC) lack antimitochondrial antibodies (AMA). Most of these patients have other autoantibodies, a syndrome recently named 'autoimmune cholangitis.' We report our experience in patients with AMA- negative PBC treated with ursodeoxycholic acid (UDCA) and/or liver transplantation (OLT). The study of response to UDCA was performed as follows. While recruiting patients for a previously reported multicenter trial, we identified 8 patients with AMA-negative PBC. The patients were given UDCA and followed up at regular intervals. The characteristics of AMA- negative patients at presentation were similar to those of AMA-positive patients with PBC. The clinical outcomes and sequential liver biochemistries of UDCA treatment were also comparable with those of AMA-positive patients. The study of outcome of OLT was performed as follows. We identified OLT recipients at the Mayo Clinic who had clinical, radiological, and histological features compatible with PBC. Their pretransplant AMA status was determined, and each AMA-negative patient was paired with 2 AMA-positive patients. Of 85 OLT recipients with a diagnosis of PBC, 6 (7.1{\%}) were AMA negative, including 1 who had undergone UDCA therapy. After a median of 36 months of follow-up, graft and patient survival rates and subsequent histological changes (disease recurrence and steroid-resistant or late rejections) were comparable in AMA-negative and -positive PBC patients. In summary, in our experience of 13 AMA-negative PBC patients (including 9 who met the criteria for a diagnosis of autoimmune cholangitis), treatment with UDCA or OLT resulted in similar outcomes to those found in AMA-positive patients. We conclude that AMA status does not affect the response in PBC patients to treatment with UDCA or OLT.",
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AU - Poterucha, John J.

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AU - Batts, Kenneth P.

AU - Homburger, Henry A.

AU - Dickson, E. Rolland

AU - Krom, Ruud A F

AU - Wiesner, Russell H.

AU - Lindor, Keith

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