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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sarcoidosis is a multisystem disease characterized by noncaseating granulomas in the affected organs, including skin, heart, nervous system, and joints. Diagnosis of sarcoidosis is generally based upon a compatible history, demonstration of granulomas in at least two different organs, negative staining and culture for acid fast bacilli, absence of occupational or domestic exposure to toxins, and lack of drug-induced disease. Involvement of the hollow organs is rare. Rather than being due to sarcoidosis, some reported mucosal lesions may simply have incidental granulomas. Extrinsic compression from lymphadenopathy can occur throughout the gastrointestinal tract. The stomach, particularly the antrum, is the most common extrahepatic organ to be involved, while the small bowel is the least common. Liver involvement frequently occurs and ranges from asymptomatic incidental granulomas to portal hypertension from granulomas in the portal triad, usually with relatively preserved liver function. CT scans show hepatosplenomegaly and adenopathy, followed in frequency by focal low-attenuation lesions of the liver and spleen. Ascites is usually a transudate from right heart failure (because of pulmonary hypertension) or portal hypertension (because of biliary cirrhosis). Rarely, an exudative ascites may occur from studding of the peritoneum with nodules. Pancreatic involvement presents as a mass, usually in the head or a diffusely firm, nodular organ. Corticosteroids should be instituted when organ function is threatened, usually lungs, eyes, and central nervous system. Their role in the treatment of hepatic sarcoidosis is unclear. The overall prognosis is good although most patients will have some permanent organ impairment. Cardiac and pulmonary diseases are the main causes of death.
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PMID:Gastrointestinal and hepatic manifestations of sarcoidosis. 1936 62

A 57-year-old white woman had serum ferritin 793 ng/mL, HFE C282Y homozygosity, elevated serum angiotensin-converting enzyme (ACE) levels, 3+ hepatocyte iron, cirrhosis, hepatic granulomas, and portal hypertension. Her 37-year-old son had ferritin 869 ng/mL, C282Y/wt, elevated ACE levels, 2+ hepatocyte iron, bridging fibrosis, and hepatic granulomas. Her daughters had HFE C282Y/H63D and C282Y/wt, respectively; neither had a hemochromatosis phenotype, sarcoid, or severe liver disease. All 4 subjects had nonalcoholic hepatic steatosis. Sarcoid did not segregate with the human leukocyte antigen-A and -B haplotype shared by the proband, her son, and 1 daughter. Phlebotomy to achieve iron depletion in the proband and her son yielded 1.6 and 1.5 g iron, respectively; their ACE levels remained elevated. We reviewed previous reports of 4 patients with hemochromatosis and sarcoid. We conclude that a combination of sarcoid, steatosis, and excessive hepatocyte iron caused cirrhosis or hepatic fibrosis in the proband and her son.
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PMID:HFE hemochromatosis and hepatic sarcoid. 1944 63

Patients with chronic liver disease exhibit various cardiovascular and pulmonary complications. Hepatopulmonary syndrome results in dyspnea due to intrapulmonary arteriovenous shunting and ventilation-perfusion mismatch. Portopulmonary hypertension occurs in patients with portal hypertension. Intrathoracic portosystemic collateral vascular pathways develop in patients with portal hypertension to allow decompression of the portal vein into the systemic circulation. Hepatic hydrothorax may develop in patients with cirrhosis and ascites. Massive necrosis of the liver from any cause may be associated with acute hypoxic respiratory failure, necessitating ventilatory support. Bacterial infection is common in cirrhotic patients because of a compromised host defense system. Hepatocellular carcinoma may produce hematogenous lung metastases, intrathoracic lymph node metastases, direct intracardiac extension, and pulmonary embolism. Interferon therapy for treatment of chronic active hepatitis C may disturb cellular immune activation in some patients and contribute to the onset and progression of sarcoidosis. Awareness of the various thoracic manifestations in chronic liver disease can be helpful for making a differential diagnosis and planning proper management.
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PMID:Thoracic complications of liver cirrhosis: radiologic findings. 1944 18

Hepatopulmonary syndrome (HSP) is characterized by the triad of advanced liver disease, arterial hypoxemia, and intrapulmonary vascular dilatation. Most cases of HSP are associated with cirrhotic portal hypertension; however, it has also been reported in acute liver failure patients. An estimated prevalence of HPS in patients with chronic liver disease is around 5-32%. Sarcoidosis is a granulomatous disorder with noncaseating granulomas involving the lungs in 90% of cases. Sarcoidosis can involve the liver but in only about 1% of cases does it lead to cirrhosis and portal hypertension. In this review, we report a rare case of liver cirrhosis due to sarcoidosis with associated hepatopulmonary syndrome and discuss this in detail.
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PMID:Hepatopulmonary syndrome and liver sarcoidosis: a tale of two diseases. 2001 47

Portopulmonary hypertension is a rare and severe complication of patients with cirrhosis. Sarcoidosis, a disease of unknown etiology, is also a cause of pulonary hypertension and right heart dysfunction. We report the case of a 51-year-old male patient, suffering from cirrhosis due to Wilson's disease, portal hypertension and pulmonary hypertension (PH), who developed severe pericarditis. Wilson's disease was diagnosed 8 years before his last admission to our hospital and was being successfully treated with D-penicillamine. PH was recognized 2 years before admission and being treated with bosentan. The patient complained for dyspnea at rest and the 2D echocardiogram revealed a significant amount of pericardial fluid. All other causes of acute pericarditis were excluded and his laboratory, imaging and histopathological investigation showed evidence of sarcoidosis. He underwent a therapy with corticosteroids (methylprednisolone) and his follow-up examination showed remarkable decrease of the levels of mean pulmonary artery pressure and pericardial fluid.
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PMID:Sarcoidosis-induced pericarditis in a patient with portopulmonary hypertension: a case report. 1991 93

Granulomatous liver disease constitutes a category of hepatic disorders and is at present diagnosed in approximately 4% of liver biopsies. Hepatic granulomas develop through the interactions of T lymphocytes and macrophages, with the integral involvement of T-helper (T(H)) 1 or T(H)2 pathways or both, depending on the specific granulomatous disease. Hepatic granulomas may be manifested clinically by elevated levels of serum alkaline phosphatase and g-glutamyltransferase enzymes, damage to specific structures (eg, intrahepatic bile ducts in primary biliary cirrhosis), or infrequently, progressive liver disease with portal hypertension and cirrhosis (eg, sarcoidosis). Systemic immunologic disorders, infectious diseases, drug hepatotoxicity, and reaction to neoplastic disease are the major causative factors responsible for granulomas in the liver. These causes and recent epidemiologic trends are covered in this discussion.
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PMID:Hepatic granulomas: pathogenesis and differential diagnosis. 2105 85

Despite aggressive treatment, sarcoidosis may be debilitating and progressive. The role of tumor necrosis factor (TNF)-a in the genesis of granulomas is ambiguous. It has proven to be critical in the formation and maintenance of granulomatous inflammation and its antagonist, Infliximab, has therefore been used with success in the treatment of patients with sarcoidosis. There are, however, reports of onset of sarcoidosis in patients in treatment for other conditions and that had no outbursts before submission to this therapy. We used Infliximab in the treatment of patients with sarcoidosis who either didn't respond to corticosteroids and other conventional drugs or developed unacceptable side effects to these drugs. The initial dose was 5mg/Kg body weight and subsequent doses were given at weeks 2, 4 and then every other 8 weeks for a total period of one year. We treated ten patients with biopsy proven sarcoidosis, five men and five women, with a mean age of 47.1 years ranging from 28 to 63 years of age. Three patients had severe neurological symptoms, two had hepatic cirrhosis, one had granulomatous inflammation of the lachrymal gland and had already been submitted to many surgeries, one had extensive pulmonary involvement (stage III), one had disfiguring lupus pernio and two presented disabling cutaneous nodules. In four patients the dosage of corticosteroids or other immunosuppressive drugs was suspended, in three the dosage was reduced and in one, corticosteroids were added to the Infliximab therapy. In five of the patients there was a significant improvement. One of the patients with neurological symptoms displayed a complete recovery, while another had significant improvement of vision deficit enabling her to read again. Two patients withdrew from therapy, one due to lack of improvement of neurological symptoms and the other due to the onset of organizing pneumonia spawned by Infliximab. Two patients developed anti-histone antibodies during treatment. Infliximab seems effective in treating patients who are either refractory or develop side effects to a standard regimen of corticosteroids and immunosuppressive agents. These patients, treated with Infliximab, should be under tight surveillance in order to quickly identify possible secondary effects.
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PMID:Infliximab for treating sarcoidosis patients, Portuguese experience. 2147 72

In addition to focal liver lesions, diffuse and vascular disorders of the liver represent a wide spectrum of liver diseases which are from the radiological point of view often difficult or nearly impossible to diagnose. Classical diagnostic methods are computed tomography and magnetic resonance imaging in addition to ultrasound. Diffuse parenchymal damage caused by diseases of various etiologies is therefore difficult to evaluate because it often lacks characteristic morphological features. For hepatic steatosis, hemochromatosis/siderosis as an example of a diffuse storage disease and sarcoidosis and candidiasis as infectious/inflammatory diseases, an image-based diagnosis is appropriate in some cases. For most diffuse liver diseases, however only nonspecific changes are visualized. Vascular pathologies of the liver, such as the Budd-Chiari syndrome and portal vein thrombosis, however, can usually be diagnosed very clearly using radiology and there is also a very effective interventional radiological treatment. Chronic diseases very often culminate in liver cirrhosis which is highly associated with an increased risk of liver cancer.
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PMID:[Diffuse and vascular hepatic diseases]. 2180 55

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.
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PMID:A histologically proven case of progressive liver sarcoidosis with variceal rupture. 2205 11

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.
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PMID:Ductopenia related liver sarcoidosis. 2250 31


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