Ayyala US, Padilla ML. Diagnosis and treatment of hepatic sarcoidosis

Mount Sinai Medical Center, One Gustave L. Levy Place, Box 1232, New York, NY 10029-6574, USA. .
Current Treatment Options in Gastroenterology 01/2007; 9(6):475-83. DOI: 10.1007/s11938-006-0004-9
Source: PubMed


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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    • "Furthermore, UDCA seems promising, since there have been reports that it can improve cholestasis by decreasing cytokine and immunoglobulin production [21]. The use of other immunosuppressive and cytotoxic drugs has been reported in single cases or small series of patients with questionable results, supporting the need of more evidencebased therapies in the future [20] [23]. "

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    ABSTRACT: Sarcoidosis is a systemic inflammatory disorder of unknown etiology. Although any organ may be involved, the lungs are most frequently affected. The clinical course of the disease is highly variable, with up to two-thirds of untreated patients experiencing spontaneous remission within 12-24 months of onset of symptoms. When therapy is required, corticosteroids are considered standard, but studies demonstrating their ability to modify the long-term outcome in this disease are lacking. Often, the myriad of adverse side effects of corticosteroids necessitate the addition of immunosuppressants, cytotoxic agents or biologic therapies to maintain disease remission. Unfortunately, optimal therapeutic regimens have not been described. Patients who do not respond to therapy often experience progressive fibrotic changes and end-organ damage, which ultimately may result in significant morbidity or death. Agents commonly used to treat patients with sarcoidosis and emerging therapeutic options are discussed.
    No preview · Article · Jul 2007 · Expert Opinion on Pharmacotherapy
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    ABSTRACT: An unusual case of a patient with Löfgren syndrome peritoneal involvement by sarcoidosis. Patient and methods: A 36-year-old woman presented with Löfgren syndrome and an increase in liver enzyme levels. An abdominal CT scan showed multiple nodules on the peritoneum mimicking peritoneal carcinomatosis. Laparoscopy was conducted with biopsy of the peritoneal nodules. Biopsy specimens from the peritoneum, liver, and bronchi showed noncaseating granulomas, and the search for tuberculosis was negative. Clinical and biological features resolved within 6 months, without therapy with steroids, while a thoracic CT scan as well as an abdominal CT scan showed no change. To our knowledge, this is the first reported case of peritoneal sarcoidosis associated with Löfgren syndrome. A longer follow-up will, however, be required to assess the chronicity of the disease.
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