Case Report
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World J Hepatology. Oct 27, 2011; 3(10): 271-274
Published online Oct 27, 2011. doi: 10.4254/wjh.v3.i10.271
A histologically proven case of progressive liver sarcoidosis with variceal rupture
Hitoshi Yoshiji, Kou Kitagawa, Ryuichi Noguchi, Masahito Uemura, Yasuhide Ikenaka, Yosuke Aihara, Keisuke Nakanishi, Yusaku Shirai, Chie Morioka, Hiroshi Fukui
Hitoshi Yoshiji, Kou Kitagawa, Ryuichi Noguchi, Masahito Uemura, Yasuhide Ikenaka, Yosuke Aihara, Keisuke Nakanishi, Yusaku Shirai, Chie Morioka, Hiroshi Fukui, Third Department of Internal Medicine, Nara Medical University, Kashihara, Nara 634-8522, Japan
Author contributions: Yoshiji H and Kitagawa K described the clinical case, obtained informed consent from the patient, conceived the study, participated in its design, assisted in data collection, coordinated and helped draft the manuscript; Yoshiji H undertook the literature research and contributed to the writing; Uemura M, Ikenaka Y, Noguchi R, Shirai Y, Aihara Y, Nakanishi K, Morioka C and Fukui H were responsible for the diagnosis, patient management and review.
Correspondence to: Hitoshi Yoshiji, MD, PhD, Third Department of Internal Medicine, Nara Medical University, Shijo-cho 840, Kashihara, Nara 634-8522, Japan. yoshijih@naramed-u.ac.jp
Telephone: +81-744-223051 Fax: +81-744-247122
Received: May 3, 2011
Revised: August 18, 2011
Accepted: October 20, 2011
Published online: October 27, 2011
Abstract

Sarcoidosis is a chronic multi-systemic granulomatous disease, and liver involvement frequently occurs. in most cases, no evidence of liver dysfunction is observed, and portal hypertension due to sarcoid liver diseases is a rareoccurrence. Moreover, no case of liver sarcoidosis has ever been reported with confirmation of the disease progression. Herein we describe a patient having hepatic sarcoidosis with severe portal hypertension and liver dysfunction. The diagnosis was histologically confirmed from granulomatous status to established liver cirrhosis over 10 years. A 46-year-old woman developed massive hematemesis due to the rupture of gastric cardial varices. She underwent emergency endoscopic injection sclerotherapy, and clear evidence of chronic hepatic failure. Twelve years ago, she was diagnosed as having sarcoidosis with respiratory clinicalsymptoms. Liver biopsy revealed asymptomatic incidental granulomas without fibrosis development. After a couple of years, features of liver dysfunction were manifest and progressed. Ten years after the first biopsy, a second liver biopsy was performed, and well established dense fibrosis was revealed. Although significant liver dysfunction with portal hypertension is rarely seen in sarcoidosis, this case indicates that we have to consider the possibility that sarcoidosis may cause end-stage liver cirrhosis.

Keywords: Liver sarcoidosis; Portal hypertension; Hepatic failure; Liver cirrhosis