Diagnosis and treatment of hepatic sarcoidosis.
ABSTRACT The presence of granulomas in the liver raises consideration of a wide differential diagnosis, but in most Western series, sarcoidosis accounts for a majority of cases. This review will focus specifically on the diagnosis of and therapy for hepatic sarcoidosis. Sarcoidosis is a systemic granulomatous disease of unknown etiology. Hepatic involvement of sarcoidosis was described in 11.5% of 736 patients enrolled in the ACCESS study. However, presence alone of granulomas in an organ in sarcoidosis does not dictate treatment. The decision to treat should be based on symptoms and severity of disease. Although hepatic involvement usually is asymptomatic, a minority of patients progress to chronic cholestatic disease, portal hypertension, and cirrhosis that may require liver transplantation. Treatment of hepatic sarcoidosis should be reserved for patients who manifest this spectrum of disease. Glucocorticoid treatment is first-line therapy for hepatic sarcoidosis, improving symptoms and abnormal laboratory values but generally having no effect on progression of disease. In addition to glucocorticoids, immunomodulators such as azathioprine, methotrexate, hydroxychloroquine, and infliximab have been used with some positive effects on symptoms, liver enzyme abnormalities, and hepatomegaly, but none has been shown to prevent progression of disease. Ultimately, in cases of overt liver failure, liver transplantation is the definitive treatment. Overall, treatment for hepatic sarcoidosis is targeted toward alleviation of symptoms but has no curative potential at this time. Focus should be on discovering the etiology of the disease to target therapy at prevention, not cure.
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- American Journal of Roentgenology 08/2004; 183(1):171-3. · 2.90 Impact Factor
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ABSTRACT: GCSs exert their anti-inflammatory effects through influencing multiple signal transduction pathways. Their most important action is switching off multiple activated inflammatory genes through inhibition of HAT and recruitment of HDAC2 activity to the inflammatory gene transcriptional complex. In addition, GCSs may activate several anti-inflammatory genes and increase the degradation of mRNA encoding certain inflammatory proteins. This broad array of actions may account for the striking effectiveness of GCSs in complex inflammatory diseases such as asthma and the difficulty in finding alternative anti-inflammatory drugs. There is now a better understanding of how the responsiveness to GCSs is reduced in patients who have severe asthma, who have asthma and smoke, and who have COPD. An important mechanism now emerging is a reduction in HDAC2 activity as a result of oxidative stress. These new insights into GCS action may lead to new approaches to treating inflammatory lung diseases and in particular to increasing effectiveness of steroids in situations where they are less effective.Immunology and Allergy Clinics of North America 09/2005; 25(3):451-68. · 2.38 Impact Factor