Hepatic sarcoidosis

Valla, D., Pessegueiro-Miranda, H., Degott, C., Lebrec, D., Rueff, B., Benhamon, J.R Hepatic sarcoidosis with portal hypertension. A report of seven cases with a review of the literature. Quart. J. Med. 1987 63 531-544... 

Renal adenocarcinoma has been reported in a 50-year-old man after 3 years of cyclophosphamide treatment for hepatic sarcoidosis (54). 

Das D, Smith A, Warnes TW. Hepatic sarcoidosis and renal carcinoma. J Clin Gastroenterol 1999 28(l) 61-3. 

Interstitial pulmonary fibrosis Cirrhosis of the liver Chronic active hepatitis Sarcoidosis... 

Cuchacovich R, Hagan J, Khan T, Richer A, Espinoza LR. Tumor necrosis factor-alpha (TNF-a) -blockade-induced hepatic sarcoidosis in psoriatic arthritis (PsA) case report and review of the literature. Clin Rheumatol 2011 30(1) 133-7. 

Kennedy PT, Zakaria N, Modawi SB, et al. Natural history of hepatic sarcoidosis and its response to treatment. Eur J Gastroenterol Hepatol 2006 18 721-726. 

Hepatic sarcoidosis is more than twice as common in African Americans compared to Caucasians (28,59,63). It is more likely that sibling pairs with sarcoidosis will both have liver involvement than two unrelated sarcoidosis patients (64), which suggests that there may be a genetic explanation for this phenotype. There is no increased prevalence based on gender or age (28,63). No geographic area of high prevalence has been identified. 

Most patients with hepatic sarcoidosis are asymptomatic (59,65). The disease is often diagnosed by liver biopsy as part of a workup for abnormal serum hver function tests or abnormalities on an abdominal chest CT scan. Pruritus and abdominal pain are two of the more common symptoms, with the latter present in 15% (15/100) of cases (63). Jaundice, fever, and weight loss are present in less... 

The most common liver function test abnormality in hepatic sarcoidosis is an elevated serum alkaline phosphatase, which is present in more than 90% of patients with signs or symptoms of hepatic sarcoidosis (59,67,68) but is found in as few as 15% (32/217) of patients with histologic evidence of disease (62). Occasionally, this elevation may exceed 5 to 10 times the upper limits of normal... 

Fifty to 70% of patients with clinical evidence of hepatic sarcoidosis have elevations in serum transaminases that are usually less elevated than the serum alkaline phosphatase (63,67). Hypoalbuminemia, hyperbilimbinemia, and hepatic encephalopathy may rarely occur with chronic progressive disease... 

The exact frequency of hepatic abnormalities on abdominal CT is unknown because aU series have involved a selection bias and/or have been retrospective. However, the radiographic features of hepatic sarcoidosis have been well described. Hepatomegaly is the most common liver abnormality detected on CT (70-73) and is frequently associated with splenomegaly (73). Hepatomegaly from sarcoidosis may occur in patients with normal chest radiographs (Scadding stage 0) (70). 

Cirrhosis has been reported in 6% (6/100) of patients with hepatic sarcoidosis (63). Some of these patients have concomitant cholestatic features with loss of bile ducts indicating a pattern of primary biliary cirrhosis as previously described (63). However, cirrhosis in the absence of a cholestatic pattern may also be seen (63,67,84,85). 

A patient with hepatic sarcoidosis virtually always demonstrates hepatic granulomas on liver biopsy (60). However, the presence of noncaseating hepatic granulomas does not establish the diagnosis of hepatic sarcoidosis, as there are... 

Diagnostic Approach for Hepatic Sarcoidosis When Biopsy of An Extrahepatic Organ Shows Granuiomatous infiammation... 

Most patients with hepatic sarcoidosis do not require treatment (81). Although treatment with corticosteroids can improve liver function tests in approximately half of asymptomatic patients, three-fourths of such patients who are not treated eventually undergo spontaneous improvement in liver function tests and the rest remain stable (59). Furthermore, evidence suggests that corticosteroid treatment of hepatic sarcoidosis promotes relapse (87). On the basis of these data, therapy for hepatic sarcoidosis is not indicated in asymptomatic patients with Uver function test elevations. Such patients should be followed with serial Uver function tests, although it is rare for them to develop hepatic failure (59). 

As mentioned previously, patients with hepatic sarcoidosis may develop a chronic cholestatic syndrome with jaundice, fever, malaise, anorexia, weight loss, pruritus, and a cholestatic pattern of abnormal Uver function tests (77-79). These symptoms are often severe and require treatment. Prednisone in doses of 30 to 60 mg/day may improve symptoms, lower serum alkaline phosphatase levels, and improve hepatomegaly (77,96). Ursodeoxycholic acid, which inhibits intestinal absorption and increases biliary secretion of cholic and chenodeoxycholic acids (97), is often effective for the cholestatic syndrome of hepatic sarcoidosis (98,99). A dose of 10 mg/kg/day has been recommended (98,99). 

Devaney K, Goodman ZD, Epstein MS, et al. Hepatic sarcoidosis clinicopatho-logic features in 100 patients. Am J Surg Pathol 1993 17 1272-1280. 

Periera-Lima J, Schaffher F. Chronic cholestasis in hepatic sarcoidosis with clinical features resembling primary biliary dtrhosis report of two cases. Am J Med 1987 83 144-148. 

Vilinskas J, Joyeuse R, Serlin O. Hepatic sarcoidosis with portal hypertension. Am J Surg 1970 120 393-396. 

Lu CL, Chen CY, Hou MC, et al. The experience of endoscopic tissue glue injection in the treatment of hepatic sarcoidosis related gastric variceal bleeding report of a case. Hepato-gastroenterol 1999 46 2293-2295. 

Sherlock S, Dooley J. Hepatic sarcoidosis. In Diseases of the liver and biliary system, 10th ed. Oxford Blackwell Science, 1997 163-173. 

Mullins PD, Youngs GR. Favorable prognosis following variceal haemorrhage complicating hepatic sarcoidosis. Eur J Gastroenterol Hepatol 1995 7 185-186. 

Fidler HM, Hadziyannis SJ, Dhillon AP, et al. Recurrent hepatic sarcoidosis following liver transplantation. Transplant Proc 1997 29 2509-2510. 

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