Hepatic sarcoidosis with vanishing bile duct syndrome, cirrhosis, and portal phlebosclerosis. Report of an autopsy case.
ABSTRACT A few cases of sarcoidosis are associated with progressive liver disease, with a wide variety of clinicopathologic features. Herein, we report an autopsy case (65-year-old man). During an examination for liver dysfunction, cirrhosis with cholestatic dysfunction and splenomegaly were found. Needle liver biopsy revealed cirrhosis with lymphocytic piecemeal necrosis, dense septal fibrosis, and ductopenia. In addition, noncaseating epithelioid granuloma was also seen in the periportal region. Ductal enzymes and immunoglobulin M (IgM) levels were elevated, although antimitochondrial antibodies were negative. Instead, angiotensin-converting enzyme was elevated. He died of pulmonary failure and lung cancer. The autopsy liver (1,220 g) showed multinodular cirrhosis with broad and dense septa that divided the parenchyma. Mild lymphoid cell infiltration was seen in the periportal region. About a half of the interlobular bile ducts were lost, and the remaining bile ducts showed prominent periductal fibrosis, resembling sclerosing cholangitis. Interestingly, a few interlobular bile ducts showed chronic nonsuppurative cholangitis with epithelioid granulomas. Intrahepatic portal veins showed luminal narrowing with prominent phlebosclerosis. Hepatobiliary pathologies that resemble primary biliary cirrhosis and primary sclerosing cholangitis and that are followed by vanishing bile duct syndrome, chronic active hepatitis-related cirrhosis, and intrahepatic portal venous phlebosclerosis occur in a single case of sarcoidosis.
SourceAvailable from: Jean-François Cadranel[Show abstract] [Hide abstract]
ABSTRACT: Sarcoidosis is a systemic granulomatous disease which may involve many organs. In approximately 95% of patients there is liver involvement, with noncaseating hepatic granulomas occurring in 21 to 99% of patients with sarcoidosis. Liver involvement is usually asymptomatic and limited to mild to moderate abnormalities in liver biochemistry. The occurrence of jaundice in sarcoidosis is rare; extensive imaging procedures and the examination of liver biopsies permit a precise diagnostic. Ductopenia associated with sarcoidosis has been reported in less than 20 cases and can lead to biliary cirrhosis and liver- related death. We report here on a case of ductopenia-related sarcoidosis in which primary biliary cirrhosis and extrahepatic cholestasis have been carefully excluded. The patient follow up was 8 years. Although ursodesoxycholic acid appears to improve liver biochemistry it does not preclude the rapid occurrence of extensive fibrosis. A review of the literature of reported cases of ductopenia related to sarcoidosis is provided.06/2011; 3(6):170-4. DOI:10.4254/wjh.v3.i6.170
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ABSTRACT: Here we report a case of a 25-year-old woman with mesenteric and hepatic sarcoidosis without lung involvement complicated by severe noncirrhotic portal hypertension. In 1992, at the clinical presentation, she had abdominal pain, asthenia, and weight loss. Splenomegaly, signs of flogosis, sideropenic anemia, and cholestasis were observed. Laparoscopic abdominal exploration and histological analysis demonstrated noncaseating granulomas of the liver, abdominal lymph nodes, and mesenteric connective tissue. The clinical course was severe with episodic remissions and recrudescences characterized by ascites (mild or moderate), elevation of bilirubin levels (mean: 1.1 mg/dl; range: 0.9-3.5 mg/dl), reduction of albumin levels (mean: 4 g/dl; range: 3.4-4.2 g/dl), and prolongation of elevated international normalized ratio (mean: 1; range: 0.9-1.4). In 1997, the patient had variceal bleeding. Complete hemostasis was obtained with band ligation. Liver function was preserved, and until 2000 the disease remained stable. In 2001, the patient became pregnant. At the 36th week of gestation, the patient delivered a healthy female infant and afterwards remained in clinical remission. This report stresses that sarcoidosis can have a hepatic and mesenteric involvement in absence of thoracic lymphadenopathy. Portal hypertension may be severe, and in absence of cirrhosis it may be associated with portal thrombosis. Finally, portal hypertension in patients with hepatic sarcoidosis and preserved liver function should not be considered as an absolute contraindication to pregnancy.European journal of gastroenterology & hepatology 11/2008; 20(10):1032-5. DOI:10.1097/MEG.0b013e3282f4488d · 2.15 Impact Factor
Article: Systemic causes of cholestasis.[Show abstract] [Hide abstract]
ABSTRACT: Systemic causes of cholestasis constitute a diverse group of diseases across organ systems. The pathophysiology of cholestasis in systemic disease can be a consequence of direct involvement of a disease process within the liver or extrahepatic biliary system or secondary to immune-mediated changes in bile flow. Evaluating a patient with cholestasis for a systemic cause requires an understanding of the patient's risk factors, clinical setting (eg, hospitalized or immunosuppressed patient), clinical features, and pattern of laboratory abnormalities.Clinics in liver disease 05/2013; 17(2):301-17. DOI:10.1016/j.cld.2012.11.001 · 2.70 Impact Factor