Hepatic sarcoidosis primary biliary cirrhosis

Sarcoidosis is a granulomatous disease of unknown origin, with pulmonary findings in more than 90% of patients. Extrapulmonary involvement is common and all organs can be involved (especially lymph nodes, eyes, joints, central nervous system) but it is rare to find an isolated extrapulmonary disease (less than 10% of patients). Granulomatous inflammation of the spleen and the liver is common in patients with systemic sarcoidosis, while hepatosplenic enlargement is unusual and splenic involvement rare. We report two cases of systemic sarcoidosis, that onset with splenic and hepatosplenic disease, and one case with splenic sarcoidosis without pulmonary involvement...

BACKGROUND: Sarcoidosis is a multi-system disorder characterised by non-caseating granulomas. Coexistence of sarcoidosis with immune-mediated and chronic inflammatory diseases has been described in case series. However, the coexistence of two different diseases in individuals can occur by chance, even if each of the diseases is rare. AIM: To determine whether sarcoidosis necessitating hospital admission or day-case care coexists with a range of immune-mediated and chronic inflammatory diseases more commonly than expected by chance...

BACKGROUND: Hepatic granuloma (HG) is a well defined histopathological finding with an heterogenous clinical presentation. Diagnosis of a specific clinical entity is not possible every time. Descriptive studies may shed light on the various etiologies also common and distinctive findings among these patients. METHODS: We reviewed the results of the liver biopsies of 592 patients. Characteristics of the patients with HG were extracted from the hospital charts. Laboratory studies included biochemical tests, hepatitis C virus (HCV) antibody, Brucella agglutination tests, tuberculin skin test...

The utility of protocol liver allograft biopsies remains controversial, particularly in patients with normal liver function tests (LFTs). However, histologic evaluation of these biopsies provides an opportunity to examine the types and severity of liver diseases that can occur in livers with normal clinical and biochemical function. We studied 165 protocol allograft biopsies taken from 100 liver transplant patients at the time of normal LFTs and normal clinical function at 3 to 8 months (n=36), 1 year (n=52), 2 to 3 years (n=54), and 4 to 5 years (n=23)...

BACKGROUND/AIMS: The incidence of hepatic granulomas is reported in 2-15% of liver biopsies. This study was carried out to evaluate the incidence and aetiology of hepatic granulomas in a German Institute of Pathology with specialization in liver diseases. METHODS: A retrospective case review was performed on 12,161 liver biopsies of the Institute of Pathology (University of Cologne) between 1996 and 2004. Aetiology was determined according to histomorphological changes, clinicopathological data and liver tissue polymerase chain reaction (PCR) for detection of diverse putative pathogens in the liver tissue...

Sarcoidosis and primary biliary cirrhosis (PBC) are thought to be two distinct disorders of unknown origin. However, both are characterized by hepatic granuloma formation and may also manifest cutaneous granulomatous inflammation. In this report, we describe two cases of cutaneous sarcoidosis occurring in the setting of PBC and review 7 additional cases from the literature of granulomatous skin disease associated with PBC. Although the pathogenesis of both sarcoidosis and PBC remains elusive, the simultaneous occurrence of these uncommon diseases suggests a common pathway may contribute to granuloma formation in both disorders...

Primary biliary cirrhosis (PBC) and systemic sarcoidosis are granulomatous diseases of unknown etiology whose hepatic manifestations may infrequently be imitative of one another. Described herein is the first reported case in the medical literature of systemic sarcoidosis developing after liver transplantation for PBC. The presented patient, who suffered from typical clinical, laboratory, and pathologic manifestations of PBC, developed decompensated liver cirrhosis within a course of 8 years, necessitating orthotopic liver transplantation...

BACKGROUND: Hepatic granulomas have been reported in 2-15% of unselected liver biopsies, with a wide clinical profile responsible for their presence. To date, no series concerning the prevalence and the etiology of granulomas from Greece has been reported. OBJECTIVES: To evaluate the prevalence and the etiology of hepatic granulomas and to investigate whether there has been an alteration in distribution of diagnoses in our series compared with those published so far in the literature...

Hepatic granulomas were present in 3.7% of liver biopsies from a 6-year Greek study. The majority of cases were due to autoimmune disease (primary biliary cirrhosis), followed by sarcoidosis and idiopathic causes. Infections were infrequent. This profile is similar to series from the USA, Ireland and Scotland. It contrasts dramatically with series from Turkey and Saudi Arabia where infectious aetiologies form the majority of cases, and autoimmune cases are not reported. Tuberculosis and schistosomiasis are the most prevalent infections...

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The choice of immunosuppressive regime used after liver transplantation depends on many factors, which should include the effect of disease recurrence; recurrence of disease after liver transplantation may be affected by the degree and type of immunosuppression used and recurrent disease may affect patient and graft survival. For autoimmune diseases, recurrence of primary biliary cirrhosis develops sooner and more rapidly in those on tacrolimus compared with cyclosporine, but graft loss from recurrent disease is uncommon; recurrence rates of primary sclerosing cholangitis is unaffected by immunosuppressive regimes and recurrence of autoimmune hepatitis may be reduced by prescription of corticosteroids...

The cholangiopathies represent diseases and syndromes affecting the biliary system at any site between the canals of Hering and the ampulla of Vater. Hepato-canalicular cholestasis reflects biliary secretory failure of the hepatocyte caused by disturbances of intracellular organelles or damage to the bile canalicular excretory functions. Drug reactions are related especially to antibiotics, phenothiazine derivates and carbamazepine. Immune-mediated cholangiopathies cause destruction and reduction of interlobular bile ducts, and are sometimes called vanishing bile duct diseases...

BACKGROUND: Epithelioid granulomas have been reported in 2-15% of unselected liver biopsies, with numerous underlying aetiologies described. However, all UK series were reported before identification of hepatitis C virus (HCV). AIM: To evaluate the current aetiologies of hepatic granulomas and to assess the prognosis for the "idiopathic" group, in which all investigations for a recognised cause were negative or normal. METHODS: A retrospective review of patient case notes between 1991 and 2001; all patients who had a liver biopsy at Glasgow Royal Infirmary revealing epithelioid granulomas had their case notes and liver biopsies reviewed and a standard proforma completed...

PURPOSE: A retrospective study was set up to investigate active pathologic processes associated with sarcoidosis diagnosed in 32 patients. METHODS: Eighteen patients had two identified granulomatous localizations (56%) and 14 patients had three localizations or more (27%). Comorbidity was noticed in nine patients (28% of cases). Sarcoidosis was associated with an infectious disease five times (hepatitis C virus [HCV] infection three times, including one case after recombinant interferon alpha therapy, and HIV and HCV co-infection two times)...

Secondary Sjögren's syndrome is due to another disease. When it develops in connective tissue diseases, their causative role is unchallenged. In AIDS or hepatitis C, exocrine involvement is virus related. Whether or not it qualifies for Sjögren's syndrome is debated. Amyloidosis and sarcoidosis do not produce direct, autoimmune lesions of the glands, hence their exocrine involvements are considered as differential diagnoses. The most common Sjögren's syndrome is found in rheumatoid arthritis. When it appears, the arthritis has been evolving for years, and has reached its typical, seropositive and erosive stage...

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...

The monocyte chemotactic proteins (MCPs) form a distinct structurally related subclass of C-C chemokines. MCPs select specific target cells due to binding to a distinct set of chemokine receptors and because of their effects on monocytes, and may participate in the process of granuloma formation during bacterial and/or mycobacterial infections. The aetiology of primary biliary cirrhosis (PBC) is still unclear, although bacterial infection and autoimmune processes have been implicated. In this study, the expression of three of the most potent monocyte chemoattractants, MCP-1, -2, and -3, was examined in patients with PBC and the data were compared with results for other liver diseases including primary sclerosing cholangitis (PSC), chronic viral hepatitis C, hepatic sarcoidosis, and normal liver...

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...