Sarcoidosis primary biliary cirrhosis

Hepatocytes are rich in mitochondria, which play an important role in hepatic metabolism. In certain pathologic conditions (most often alcoholic liver disease) mitochondria became enlarged; nevertheless, even in these conditions they are hardly detectable on light microscopy. Recently an antimitochondrial antibody (mAM), which recognizes a 60-kDa protein, has been characterized. The purpose of the present study was to study immunoreactivity of this antibody in a series of liver biopsies. We studied 146 liver biopsies using an mAM...

We report a patient in whom sarcoidosis coexisted with sclerosing cholangitis and chronic atrophic autoimmune gastritis. There are some autoimmune diseases associated with primary sclerosing cholangitis; the difference between sarcoidosis and all other autoimmune diseases associated with primary sclerosing cholangitis is the ability of the former to damage the biliary tree. Moreover, when sarcoidosis behaves like cholestasis it can damage the biliary tree, mimicking primary sclerosing cholangitis, with high immunoglobulin M but without inflammatory bowel disease and p-ANCAs negative...

Cluster of differentiation 1 (CD1) is a family of four distinct nonpolymorphic major histocompatibility complex class I-like molecules that can present microbial nonpeptide lipid antigens to T cells. Among the CD1 gene family, CD1d is found in a wide range of tissues including the intestine and liver, and has been proposed to play an important role in mucosal immunity. Primary biliary cirrhosis (PBC) is an immune-mediated liver disease involving the intrahepatic small bile ducts, which also belong to the mucosal immune system...

In sarcoidosis, granulomas are frequently present in multiple organs, including the liver. Typically, epithelioid granulomas (noncaseating) are scattered throughout the liver, but confluent granulomas can be present in cases with severe hepatic involvement. The characteristic inclusions in giant cells (for example, Schaumann bodies and asteroid bodies) are not seen in all cases and are not pathognomonic. The granulomas of sarcoidosis may heal without a trace, but confluent granulomas can result in extensive, irregular scarring...

Giant cell granulomas in liver biopsies is a relative common finding. Among the many causes of granulomatous lesions of the liver primary biliary cirrhosis and sarcoidosis are the most frequently diagnosed. On the other hand sarcoid-like granulomatous reaction can be encountered associated to malignant tumours. Purpose of the present paper is to describe a case of a sarcoid-like reaction of the liver associated to gastric adenocarcinoma. The patient was a 66 yr old man who underwent gastrectomy for a signet-ring cell adenocarcinoma...

In a small number of cases the co-existence of primary biliary cirrhosis and sarcoidosis is assumed from clinical serological and histological findings. A case of sarcoidosis is reported in which the M2 antibody, a highly specific marker for primary biliary cirrhosis, was detected. The patient also developed a severe myositis and a possible overlap syndrome is discussed.

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NCPH is a result of obliteration of portal veins. Many inflammatory conditions may initiate the process by causing endothelial injury. As obliteration progresses, there is local stasis and low-grade portal hypertension. In many cases, superimposed PVT occurs before portal hypertension becomes clinically evident. Hypercoagulability is an important cofactor. Diagnosis requires the exclusion of cirrhosis. Focal atrophy and nodular hyperplasia on biopsy may be a clue to the presence of small vessel obliteration...

Granulomas in liver biopsy specimens from adult patients with chronic ductopenic cholestatic liver disease are a characteristic feature of primary biliary cirrhosis. However, we found a similar combination of abnormalities in 7 out of 100 native livers (7%) from patients who had orthotopic liver transplantation for primary sclerosing cholangitis. In a control group of native livers from 100 patients with primary biliary cirrhosis, the prevalence of granulomas was exactly the same, 7%. In the primary sclerosing cholangitis group, 13 addition livers showed a granulomatous epithelioid cell response, with or without foreign body type giant cells, to extravasated bile...

We report a case of granulomatous hepatitis in a patient with hyperthyroidism resulting from Graves' disease. A 30-year-old man presented with massive weight loss, jaundice, tachyarrhythmia and goitre. Liver function tests showed mild cytolysis and cholestasis and massive hyperbilirubinaemia. The echogram of liver and bile ducts was normal and no infection was found. A liver biopsy revealed a mixed cytolytic and cholestatic hepatitis with intralobular epithelioid granulomas. No specific cause was identified, and sarcoidosis and primary biliary cirrhosis were ruled out...

The patterns of hepatic injury were studied in 100 patients with a diagnosis of sarcoidosis and clinical evidence of liver disease that led to diagnostic liver biopsy. Granulomas were present in all patients; they occupied from < 1% to > 90% of the total volume of tissue examined and were most often located in the portal/periportal region. In none of the 100 cases were infectious organisms identified by special stains, culture, or serology. In 99% of cases, these granulomas were noncaseating; in one of the 100 cases central caseation was noted...

Primary biliary cirrhosis (PBC) and sarcoidosis are both granulomatous disorders of unknown etiology. Although they are distinct clinical entities, a small number of patients presenting with manifestations of both has been reported. We describe a female patient with PBC and sarcoidosis who developed a malignant cutaneous tumor with histologic and immunohistochemical characteristics of a sweat gland carcinoma (SGC). An SGC, developing in a patient with concurrent PBC and sarcoidosis has not been reported previously...

Although liver and/or spleen sarcoidosis usually shows no symptoms and subtle elevated levels of liver enzymes, diagnostic image scanning methods (abdominal ultrasonic scan, computed tomographic scan, magnetic resonance image, and Ga and Tc scintiscan) can easily detect the lesion. Laparoscopy unveils isolated scattered sarcoid nodules on the surface of the liver and/or spleen and biopsied specimen reveal a non-caseating epithelioid granuloma containing multinucleated giant cells especially in the portal area...

The finding of epithelioid cell granulomas within liver biopsies is a not uncommon occurrence. We undertook this study to investigate the underlying conditions responsible for a diagnosis of granulomatous hepatitis in Northern Ireland during the thirteen year period 1980-1992. One hundred and sixty-three patients with hepatic granulomas were identified, accounting for 4% of all liver biopsies undertaken during the period of the study. In 145 cases (89%) a definite clinical diagnosis was established. The most common clinical diagnoses were primary biliary cirrhosis which accounted for 90 cases (55%) and sarcoidosis which accounted for 30 cases (18%)...

In a southern Italy hospital, in five years 1523 liver biopsy specimens have been performed and histologically examined. Granulomas are found in specimens from 15 patient (1%). They are seven females and eight males with an average age of 57 years (range 43-71). Seven of the 15 specimens are Menghini-type percutaneous needle, five are surgical and three are laparoscopic bioptic specimens. Four patients are correlated with infectious diseases: 2 with hepatitis C virus (HCV), 1 with hepatitis B virus (HBV) and 1 with Mycobacterium Tuberculosis...

Nine cases of portal hypertension of unusual etiology are presented: 1 with retroperitoneal fibrosis, 2 with alveolar hydatid disease, 3 with arterio-portal fistula, 2 with primary biliary cirrhosis and 1 with sarcoidosis.

A survey of all patients in whom liver biopsy showed epithelioid granulomas was undertaken at two major teaching hospitals in Glasgow for the period 1970-1979. Seventy-seven patients with hepatic granulomas were studied retrospectively. In 53 cases (69 per cent) a clear-cut clinical diagnosis was established, which included sarcoidosis (eight cases), tuberculosis (eight), extrahepatic biliary obstruction (seven), primary liver diseases (11), neoplasm (six), bacterial infection (five) and miscellaneous (eight)...

Two West Indian patients with Kveim-biopsy proven sarcoidosis developed chronic cholestatic liver disease, clinically and biochemically similar to primary biliary cirrhosis. Liver histology revealed multiple granulomas with reduction in bile ducts and, in one patient, progression to biliary cirrhosis. Portal hypertension was present in both patients leading to severe variceal haemorrhage in one. Mitochondrial antibody was negative in both patients and when used in conjunction with the Kveim-Siltzbach skin test serves to differentiate chronic intrahepatic cholestasis secondary to sarcoidosis from primary biliary cirrhosis...

Alcoholics with abnormal liver function tests are generally assumed to have one of the recognised patterns of alcoholic liver injury. This report described a group of nine patients who were initially thought to have alcoholic liver disease but were found on liver biopsy to have a variety of liver disorders unrelated to alcohol. Liver biopsy showed granulomatous hepatitis in three, primary biliary cirrhosis in two, and cholestasis of unknown cause, large duct biliary obstruction, haemochromatosis with secondary carcinoma and Budd-Chiari syndrome in one each...

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