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Hepatic Sarcoidosis

  • Micheal Tadros1,
  • Faripour Forouhar2 and
  • George Y. Wu1
 Author information
Journal of Clinical and Translational Hepatology   2013;1(2):87-93

doi: 10.14218/JCTH.2013.00016


Sarcoidosis is a multisystem disease characterized by the presence of non-caseating granulomas in affected organs. Pulmonary involvement is the most common site of disease activity. However, hepatic involvement is also common in sarcoidosis, occurring in up to 70% of patients. Most patients with liver involvement are asymptomatic. Therefore, the majority of cases are discovered incidentally, frequently by the finding of elevated liver enzymes. Pain in the right upper quadrant of the abdomen, fatigue, pruritus, and jaundice may be associated with liver involvement. Portal hypertension and cirrhosis are complications linked to long-standing hepatic sarcoidosis. Liver biopsy is usually required to confirm the diagnosis. It is important to differentiate hepatic sarcoidosis from other autoimmune and granulomatous liver diseases. Not all cases of hepatic sarcoidosis require treatment. For symptomatic patients, the first line treatment includes corticosteroids or ursodeoxycholic acid. Various immunosuppressant agents can be used as second line agents. Rarely, severe cases require liver transplantation.


Sarcoid, Granuloma, Epitheloid


Sarcoidosis is an inflammatory disorder of unknown etiology categorized by the presence of non-caseating granulomas, which can involve multiple organs of the body,1 in the absence of infections, other autoimmune diseases, and exposure to foreign agents. It has been estimated that the worldwide prevalence of sarcoidosis is 2–60 per 100,000 people.2 Sarcoidosis affects all ethnicities with higher prevalence rates described in patients of Scandinavian descent.2 In contrast, sarcoidosis has been only rarely reported in patients of Chinese and Taiwanese origin.3,4 In the USA, African Americans have been reported to have a three times higher age-adjusted incidence of the disease.1,5 Sarcoidosis affects females more often than males across all ages and ethnicities,6 with an age peak of 20–40 years.6 A second age peak (>50 years) has also been described.6

The hallmark of sarcoidosis is the formation of epithelioid granulomas that are typically non-caseating, in the absence of tuberculosis, fungal infection, malignancy, or other causes of granulomatous reactions.7 The pulmonary system is the most common site of involvement, and is affected in about 90% of cases.6 Extra-thoracic sarcoidosis occurs in 40–50% of patients.8 Sites affected include peripheral lymph nodes (30%), the hepatic system, the spleen, stomach, small bowel, bone, and skin. Involvement of the solid organs can present as organ enlargement. Dermatological sarcoidosis may present as erythema nodosum or lupus pernio. Myopathy, uveitis, granulomatous meningitis, facial nerve palsy, cardiomyopathy, and parotid enlargement have been reported less commonly.

There are two classic syndromes described for sarcoidosis. The first is Löfgren syndrome, which is characterized by hilar lymphadenopathy, erythema nodosum, arthritis and fever.6 The second is Heerfordton syndrome, which is characterized by uveitis, swelling of the parotid gland, fever, and facial palsy.9

The pathogenesis of sarcoidosis is still not completely understood, but it is thought to be multifactorial, involving the interplay of immunological, genetic, and environmental factors.1,6 It has been theorized that an environmental trigger initiates a specific immune reaction in a genetically predisposed individual. The immunological response in sarcoidosis includes T helper 1 (Th1) cells triggered by an antigen, leading to cytokine production, mainly tumor necrosis factor alpha10 which in turn, leads to macrophage aggregation with subsequent granuloma formation.11 Certain histocompatibility antigens have been associated with sarcoidosis such as human leukocyte antigen-A1 (HLA-A1), B8, DRB1, DQB1, and DRB3.12 This suggests a genetic susceptibility and familial clustering of the disease. Some of the possible postulated triggers of environmental antigens include reactive oxygen species, and viruses (herpes simplex virus, cytomegalovirus, retroviruses) and bacteria (Borrelia burgdorferi, mycobacteria).2,6

Epidemiology of hepatic sarcoidosis

In biopsy and autopsy studies of patients with systemic sarcoidosis, liver involvement was found in about 50–80%,17 whereas only 10–30% of patients presented with abnormal liver enzymes during laboratory testing.2,18 The majority of patients with hepatic sarcoidosis are asymptomatic, despite the presence of granulomas on biopsy, abnormal liver enzymes, or radiological evidence of disease.14 It has been reported that the percentage of patients with clinically significant disease is less than 20%.19 Risk factors associated with hepatic sarcoidosis include African American ethnicity, prior exposure to pegylated interferon, and the presence of splenomegaly.13,14,20 Reports on the differences in prevalence based on gender have been contradictory. In studies containing hundreds of patients, one study reported liver disease to be more common in women,21 whereas the other showed the opposite.22

Spectrum of hepatic sarcoidosis

There is a spectrum of clinical presentations of liver involvement in sarcoidosis. Of the more than 50% of patients who have hepatic granulomas on liver biopsy, only 10–30% have elevated serum liver enzymes.2,18 About 20% of patients have palpable hepatomegaly or splenomegaly. Radiological examination (ultrasound or computed tomography) is accurate in the detection of organomegaly, demonstrating this feature in about 40% of cases.13,17 However, even in cases of elevated serum liver enzymes or radiological abnormalities, evidence of organ dysfunction is rare.

Some clinical symptoms and signs suggest liver involvement, and may warrant a liver biopsy. Systemic symptoms such as fatigue, fever, and arthralgias are non-specific, but are present in the majority of patients with active liver sarcoidosis.23,24 More specific symptoms include jaundice and pruritus, mostly from chronic cholestasis.25,26 Right upper quadrant abdominal pain, in theory, results from stretching of the Glisson's capsule by hepatomegaly as a consequence of the increasing intrahepatic volume occupied by the developing hepatic granulomas. Abdominal pain has been reported to occur in 15% of patients with sarcoidosis, and jaundice in less than 5%.19

Long-standing disease has been reported to result in portal hypertension in 3–18% of patients.16 This may be due initially to compression of portal venules by granulomas, and can result in variceal bleeding. A small proportion (6–8%) of patients can develop portal hypertension because of progression to cirrhosis. Indeed, some patients can develop end-stage liver disease and require liver transplantation.26–28 These account for about 0.012% of the total number of liver transplantation cases in the USA. Unfortunately, they often have a worse prognosis than patients with other cholestastic liver diseases such as primary biliary cirrhosis and primary sclerosingcholangitis.29 Some case reports have linked sarcoidosis with an increased risk of developing Budd-Chiari30 syndrome, hepatopulmonary syndrome,28 hepatocellular carcinoma,31,32 and biliary sarcoid mimicking cholangiocarcinoma. Table 1 lists postulated mechanisms of liver injury in hepatic sarcoidosis. Table 2 summarizes the clinical spectrum of the disease.

Table 1

Postulated mechanisms of liver injury in hepatic sarcoidosis

Liver injuryPossible mechanism
Jaundice13,14Chronic cholestasis from hepatic fibrosis, bile duct strictures (mimics cholangiocarcinoma)Obstruction of small bile ductulesExternal compression from a sarcoid mass at the pancreatic head, or porta-hepatis adenopathyHepatocellular carcinoma
Cirrhosis13,15Granuloma formation with subsequent hepatic fibrosis, with or without granulomatous phlebitis and thrombosisSecondary biliary cirrhosis
Portal hypertension16,17Intrahepatic pre-sinusoidal obstruction secondary to granulomas or sinusoidal obstruction from biliary fibrosis or cirrhosisPre-hepatic obstruction from portal vein thrombosisPost-hepatic (hepatic vein thrombosis); Budd-Chiari syndromePortal hypertension can occur without cirrhosis
Ascites17Cirrhosis and portal hypertensionCor pulmonaleHypoalbuminemiaPeritoneal sarcoidosisChylous ascites (sarcoid lymphadenopathy)
Table 2

The clinical spectrum of hepatic sarcoidosis17,19,33

FeaturePresentation (%)
Abnormal liver profile30%10–15% patients are symptomatic, and 85% are symptomatic.
Organomegaly (Liver or spleen)50% detected on radiological exam, < 15–20% detected clinically
Clinical hepatitisAbdominal pain 15%, pruritus andjaundice < 5%
Portal hypertension3–18%
Liver failure requiring liver transplantation0.012% of all liver transplants in the USA


Elevations of alkaline phosphatase and/or gamma glutamyltranspeptidase have been correlated with cholestasis and liver involvement.25,34 Alkaline phosphatase of hepatobiliary origin can be elevated 5–10 times the upper limit of normal. Aminotransferase elevations are usually mild and less frequent compared with elevations in alkaline phosphatase.19 The severity of liver test abnormalities was shown to be associated with the extent of granulomatous inflammation.33 Hyperglobulinema may be seen. Measurement of the serum angiotensin converting enzyme (ACE) levels can be useful; they have been reported to be elevated in about 60% of patients with active sarcoidosis.35–37 However, the test lacks sensitivity and specificity, with low positive and negative predictive values (84 and 74%, respectively).38 Normal levels can be seen in chronic forms and in patients who have been on corticosteroids. Therefore, a normal ACE level does not rule out sarcoidosis, but an elevated level may help distinguish sarcoidosis from other granulomatous disease. Elevated ACE levels can also be present in patients with inflammatory bowel disease.36

Radiological studies39 such as ultrasonography,40 computed tomography (CT)41 or magnetic resonance imaging (MRI)42,43 may show hepatomegaly or multiple hypointense or hypoattenuated liver nodules. The occurrence of such multiple nodules can be confused with liver metastasis or other granulomatous diseases. The presence of concomitant splenic granulomas may help point towards a diagnosis of sarcoidosis.

Liver biopsy should be considered if the diagnosis is uncertain and treatment is considered. Some authors recommend obtaining a liver biopsy if aminotransferase levels are elevated at least two-fold above the upper limit of normal. Histopathological examination is the definitive diagnostic tool. In sarcoidosis, granulomas are usually abundant and well-formed in the periportal and the portal regions. Typically, the granulomas are non-caseating and epithelioid (Fig. 1). There is scant lymphocytic inflammatory infiltration. Occasionally, fibrinoid necrosis, prominent fibrosis, and multinucleated giant cells can be seen. Staining for acid-fast mycobacteria, and for fungi should be negative. Progression of the disease can result in micronodular cirrhosis.27

A liver biopsy specimen showing a single portal epithelioid granuloma (arrow) surrounded by a thin cuff of lymphocytes
Fig. 1  A liver biopsy specimen showing a single portal epithelioid granuloma (arrow) surrounded by a thin cuff of lymphocytes

H&E, ×100.

A liver biopsy specimen showing: a, a conglomerate of granulomatous reaction (arrow) with significant fibrosis (arrow head); b, significant as well conglomerate of epithelioid granulomas with giant cells (arrow), but without necrosis
Fig. 2  A liver biopsy specimen showing: a, a conglomerate of granulomatous reaction (arrow) with significant fibrosis (arrow head); b, significant as well conglomerate of epithelioid granulomas with giant cells (arrow), but without necrosis

Trichrome, ×400.

A liver biopsy specimen showing a sarcoid granuloma in liver with central fibrinoid necrosis (arrow)
Fig. 3  A liver biopsy specimen showing a sarcoid granuloma in liver with central fibrinoid necrosis (arrow)

H&E, ×400.

Differential diagnosis

Hepatic sarcoidosis needs to be differentiated from other (autoimmune) liver diseases, especially primary biliary cirrhosis and primary sclerosing cholangitis44,45 (Table 3).

Table 3

List of features that distinguish hepatic sarcoid from primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC)

HistologyGranulomas - usually few, poorly defined and located in the portal tract along damaged bile ducts, with eosinophils.Florid ductal lesions present in early diseaseGranulomas: many, well-formed and usually periportal.Prominent fibrosis and multi-nucleated giant cells.Less prominent biliary injury.Concentric prominent periductal fibrosis: “onion skin” fibrosis. No granulomas.
Serum TestsAnti-mitochondrial antibody (AMA)Serum angiotensin converting enzyme (ACE)Peripheral anti-neutrophil cytoplasmic antibody (p-ANCA)
ImagingMagnetic resonance cholangiopancreatography (MRCP), endoscopic retrograde cholangiopancreatography (ERCP): generalized beading of the biliary tree
Extra-hepatic diseaseRarePulmonary nodules, hilar lymphadenopathyUlcerative colitis

The differential diagnosis of granulomatous liver disease can be separated into four main categories based on the type of granulomas present:7 epithelioid, foreign body, lipogranulomatous, and lymphohistiocytic. Sarcoid epithelioid granulomas are characterized by macrophages that aggregate to form giant cells surrounded by fibrin rings. Table 4 includes some of these etiologies, and their differentiating characteristics. In the setting of post-transplant granulomas, it is important to remember that the treatment itself can lead to granulomas from drug reaction, and predispose patients to develop opportunistic infections that can cause liver granulomas.7,46,47 In cases of sarcoidosis co-existing with hepatitis C, granulomas may be due to hepatitis C, or may have been induced by prior exposure to interferon therapy for hepatitis C.48

Table 4

List of features of various granulomatous liver diseases (adapted from Coash et al.7)

Mycobacterium tuberculosisAcid-fast bacteria (AFB) inside epithelioid granulomas and giant cells, often with a ring of lymphocytes and histiocytes
M aviumin tracellulareAggregates of foamy macrophages in parenchyma and portal triads with positive AFB staining
M lepraeFoamy histiocytes in portal tracts and lobules with multiple AFB found
BrucellaNon-caseating granulomas
RickettsiaFibrin rings surrounding vesicles of fat
FrancisellaSuppurative microabscesses with surrounding macrophages
ListeriaMicroabscesses with small granulomas
Bartonella henselaeStellate abscesses with three distinct zones
Tropheryma whippleiEpithelioid granulomas
HistoplasmaMacrophages and lymphocytes with histoplasma and epithelioid cells in the center
SchistosomaEosinophils with fibrosis and collagen deposition in peri-portal and peri-sinusoidal areas, often with eggs in the center
LeishmaniaFibrin ring or epithelioid granulomas
Hepatitis CEpithelioid granulomas
Drugs and chemicalsGranulomas with eosinophils
MalignancyNon-necrotic granulomas


When evaluating patients with hepatic sarcoidosis for treatment, consultation with other specialists involved in the patient's care is recommended to ensure that other organ systems that may be involved with sarcoidosis such as the pulmonary system, central nervous system and/or ocular systems are treated adequately.49

Observation without medical management

Observation alone is indicated for patients with asymptomatic liver disease or mild elevations of serum liver enzymes, and normal synthetic liver function without evidence of cholestasis.17,19,49 Hepatomegaly alone noted on physical examination and/or radiographic investigation in the absence of symptoms does not qualify as an indication for treatment. It has been noted that in some asymptomatic patients, abnormal serum liver tests can resolve spontaneously or remain stable for many years.25,34

Medical treatment

For patients who have symptoms of liver involvement, and have biochemical evidence of cholestasis or who are at high risk for developing hepatic complications, pharmacological therapy should be considered.17,19,49 The first line agents that have been studied include corticosteroids and ursodeoxycholic acid.


It has been shown that corticosteroids can decrease the number of hepatic granulomas by suppression of the inflammatory response,50 and reduce liver size25 which may be helpful for patients with persistent abdominal pain due to hepatomegaly. In addition, steroids are recommended for patients with constitutional symptoms such as fever, fatigue, pruritus, and weight loss. Low-dose prednisone (10–20 mg/daily) may be sufficient for those with mild symptoms, while a higher dose of prednisone (20–40 mg/daily) is warranted for those with severe symptoms.17,51 Prednisone can also be considered for patients with significant adenopathy in the porta hepatis.

Treatment duration should be determined by clinical and laboratory response. Some authors recommend 12 months of therapy before tapering the dose.52 Relapsing symptoms may require long-term therapy or steroid-sparing agents. Side effects of long-term steroid use (including ostopenia, bone fractures, avascular hip necrosis, hyperglycemia, cataracts, and hypertension) should be considered.52 No studies have reported on the use of oral budesonide (a systemic steroid eliminated on first pass through the liver) in the treatment of hepatic sarcoidosis.

The benefit of steroids in patients at the extremes of the clinical spectrum (that is, asymptomatic individuals or patients with cirrhosis and portal hypertension) is questionable. Steroids can help in the normalization of these laboratory abnormalities in asymptomatic patients with elevated aminotransferases.14,34 However, it is important to consider that serum liver tests can normalize spontaneously.25,34 Because there is a lack of long-term studies, it is unclear if steroids prevent liver damage or halt the progression of the disease. In patients with progressive disease (significant fibrosis, chronic cholestasis, and portal hypertension), steroid treatment has not been shown to have beneficial effects.16

Ursodeoxycholic acid

Ursodeoxycholic acid decreases biliary secretion of cholic and chenodeoxycholic acid, and inhibits the intestinal absorption of bile salt. It also helps to modulate the immune response by decreasing HLA class I and class II antigens on the cell membranes of the hepatic and bile duct epithelial cells, respectively.53 Recent studies have shown a beneficial effect of this agent in the treatment of hepatic sarcoidosis, especially when patients with cholestasis are symptomatic with pruritus.49 It has the potential effect of delaying the progression of the disease. Given its relative safety compared to steroids, some authors recommend starting with ursodeoxycholic acid before prednisone. In a recent retrospective study of 17 patients comparing placebo vs. prednisone vs. ursodeoxycholic acid,54 ursodeoxycholic acid was found to be superior in improving aminotransferase levels, pruritus, and fatigue.54 The usual dose is 13–15 mg/kg orally per day.55

Second-line and third-line agents

These agents may be useful for patients for whom prednisone fails or patients who are deemed to be steroid dependent. Azathioprine has been reported to normalize aminotransferases, but can also cause acute hepatitis.49 Methotrexate, glutathione, chlorambucil, cyclosporine, cyclophosphamide, thalidomide, pentoxifylline, and infliximab56,57 have been reported to be beneficial. However, there is inadequate evidence to support the use of these agents.49

Treatment of advanced liver disease

Portal hypertension and decompensated cirrhosis should be managed by standard measures. Steroids may not be beneficial, and liver transplantation may be required. Reported rates of graft and patient survival for hepatic sarcoidosis are 78% at 1 year, 66–67% at 3 years, and 60–61% at 5 years.29 Recurrence of disease in the graft has been reported.58


The mortality rate of sarcoidosis is about 1–5%.59,60 Death usually occurs from severe pulmonary, cardiac, and central nervous system disease rather than hepatic involvement. These survival rates are comparable to other causes of transplantation, except for PBC and PSC.29,61,62


Hepatic sarcoidosis is a granulomatous disease of unknown etiology. Around 70% of patients have epithelioid non-caseating granulomas on liver biopsy; 20–40% of patients have hepatomegaly or elevated liver enzymes. Most of the cases with liver involvement are asymptomatic and do not require treatment. Pain in the right upper quadrant of the abdomen, fatigue, pruritus, and jaundice are the most commonly associated symptoms. For symptomatic patients, prednisone and/or ursodeoxcholic acid may be considered. Those patients who do not respond to steroids and/or ursodeoxcholic acid may require other immunosuppressive agents. Portal hypertension and cirrhosis are possible complications from long-standing hepatic sarcoidosis. Patients may require liver transplantation, but disease recurrence in the graft has been reported, and survival rates tend to be lower than those for patients transplanted for other diseases.



serum angiotensin converting enzyme


acid-fast bacteria


anti-mitochondrial antibody


endoscopic retrograde cholangiopancreatography


magnetic resonance cholangiopancreatography


magnetic resonance imaging


peripheral anti-neutrophil cytoplasmic antibody


primary biliary cirrhosis


primary sclerosing cholangitis



The support of the Herman Lopata Chair in Hepatitis Research (to GYW) is gratefully acknowledged.

Conflict of interest


Authors’ contributions

Writing the medical text (MT), writing the pathology section (FF), organizing and editing the manuscript (GYW).


  1. Culver DA. Sarcoidosis. Immunol Allergy Clin North Am 2012;32:487-511
  2. Baughman RP, Lower EE. Sarcoidosis. Harrison's Principles of Internal Medicine ;2008:2135-2142
  3. Perng RP, Chen JH, Tsai TT, Hsieh WC. Sarcoidosis among Chinese in Taiwan. J Formos Med Assoc 1997;96:697-699
  4. Perng RP, Chou KT, Chu H, Chung YM. Familial sarcoidosis in Taiwan. J Formos Med Assoc 2007;106:499-503
  5. Rybicki BA, Major M, Popovich J, Maliarik MJ, Iannuzzi MC. Racial differences in sarcoidosis incidence: A 5-year study in a health maintenance organization. Am J Epidemiol 1997;145:234-241
  6. Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med 2007;357:2153-2165+2108
  7. Coash M, Forouhar F, Wu CH, Wu GY. Granulomatous liver diseases: A review. J Formos Med Assoc 2012;111:3-13
  8. Belfer MH, Stevens RW. Sarcoidosis: A primary care review. Am Fam Physician 1998;58:2041-2050
  9. Takahashi N, Horie T. Heerfordt syndrome. Nihon Rinsho Jpn J Clin Med 2002;60:1822-1826
  10. Zissel G, Prasse A, Muller-Quernheim J. Immunologic response of sarcoidosis. Semin Respir Crit Care Med 2010;31:390-403
  11. Chesnutt MS, Gifford AH, Prendergast TJ. Pulmonary disorders. Current medical diagnosis & treatment 2010
  12. Lazarus A. Sarcoidosis: Epidemiology, Etiology, Pathogenesis, and Genetics. Dis Mon 2009;55:649-660
  13. Judson MA. Hepatic, splenic, and gastrointestinal involvement with sarcoidosis. Semin Respir Crit Care Med 2002;23:529-541
  14. Kennedy PTF, Zakaria N, Modawi SB, Papadopoulou AM, Murray-Lyon I, Du Bois RM. Natural history of hepatic sarcoidosis and its response to treatment. Eur J Gastroenterol Hepatol 2006;18:721-726
  15. Moreno-Merlo F, Wanless IR, Shimamatsu K, Sherman M, Greig P, Chiasson D. The role of granulomatous phlebitis and thrombosis in the pathogenesis of cirrhosis and portal hypertension in sarcoidosis. Hepatology 1997;26:554-560
  16. Valla D, Pessegueiro-Miranda H, Degott C, Lebrec D, Rueff B, Benhamou JP. Hepatic sarcoidosis with portal hypertension. A report of seven cases with a review of the literature. Q J Med 1987;63:531-544
  17. Ebert EC, Kierson M, Hagspiel KD. Gastrointestinal and hepatic manifestations of sarcoidosis. Am J Gastroenterol 2008;103:3184-3192
  18. Chen ES D, Moller DR. Sarcoidosis. Current Rheumatology Diagnosis and Treatment 2004
  19. Dulai PS, Rothstein RI. Disseminated sarcoidosis presenting as granulomatous gastritis: A clinical review of the gastrointestinal and hepatic manifestations of sarcoidosis. J Clin Gastroenterol 2012;46:367-374
  20. Adla M, Downey KK, Ahmad J. Hepatic sarcoidosis associated with pegylated interferon alfa therapy for chronic hepatitis C: Case report and review of literature. Dig Dis Sci 2008;53:2810-2812
  21. Newman LS, Rose CS, Bresnitz EA, Rossman MD, Barnard J, Frederick M. A case control etiologic study of sarcoidosis: environmental and occupational risk factors. Am J Respir Crit Care Med 2004;170:1324-1330
  22. Valla DC, Benhamou JP. Hepatic granulomas and hepatic sarcoidosis. Clin Liver Dis 2000;4:269-85, ix–x
  23. Harder H, Büchler MW, Fröhlich B, Ströbel P, Bergmann F, Neff W. Extrapulmonary sarcoidosis of liver and pancreas: A case report and review of literature. World J Gastroenterol 2007;13:2504-2509
  24. Israel HL, Margolis ML, Rose LJ. Hepatic granulomatosis and sarcoidosis - Further observations. Dig Dis Sci 1984;29:353-356
  25. Israel HL, Sones M. Sarcoidosis: Clinical observation on one hundred sixty cases. Arch Intern Med 1958;102:766-776
  26. Blich M, Edoute Y. Clinical manifestations of sarcoid liver disease. J Gastroenterol Hepatol 2004;19:732-737
  27. Malhotra A, Naniwadekar A, Sood G. Hepatobiliary and pancreatic: Cirrhosis secondary to hepatic sarcoidosis. J Gastroenterol Hepatol 2008;23:1942
  28. Gupta S, Faughnan ME, Prud'homme GJ, Hwang DM, Munoz DG, Kopplin P. Sarcoidosis complicated by cirrhosis and hepatopulmonary syndrome. Can Respir J 2008;15:124-126
  29. Vanatta JM, Modanlou KA, Dean AG, Nezakatgoo N, Campos L, Nair S. Outcomes of orthotopic liver transplantation for hepatic sarcoidosis: An analysis of the United Network for organ sharing/organ Procurement and Transplantation Network data files for a comparative study with cholestatic liver diseases. Liver Transpl 2011;17:1027-1034
  30. Deniz K, Ward SC, Rosen A, Grewal P, Xu R. Budd-Chiari syndrome in sarcoidosis involving liver. Liver Int 2008;28:580-581
  31. Chalasani P, Vohra M, Sheagren JN. An association of sarcoidosis with hepatocellular carcinoma. Ann Oncol 2005;16:1714-1715
  32. Wong VS, Adab N, Youngs GR, Sturgess R. Hepatic sarcoidosis complicated by hepatocellular carcinoma. Eur J Gastroenterol Hepatol 1999;11:353-355
  33. Cremers J, Drent M, Driessen A, Nieman F, Wijnen P, Baughman R. Liver-test abnormalities in sarcoidosis. Eur J Gastroenterol Hepatol 2012;24:17-24
  34. Vatti R, Sharma OP. Course of asymptomatic liver involvement in sarcoidosis: Role of therapy in selected cases. Sarcoidosis Vasc Diffuse Lung Dis 1997;14:73-76
  35. Silverstein E, Friedland J, Kitt M, Lyons HA. Increased serum angiotensin converting enzyme activity in sarcoidosis. Isr J Med Sci 1977;13:995-1000
  36. Silverstein E, Fierst SM, Simon MR, Weinstock JV, Friedland J. Angiotensin-converting enzyme in Crohn's disease and ulcerative colitis. Am J Clin Pathol 1981;75:175-178
  37. Lieberman J. Elevation of serum angiotension converting enzyme (ACE) level in sarcoidosis. Am J Med 1975;59:365-372
  38. Studdy PR, Bird R. Serum angiotensin converting enzyme in sarcoidosis - Its value in present clinical practice. Ann Clin Biochem 1989;26:13-18
  39. Koyama T, Ueda H, Togashi K, Umeoka S, Kataoka M, Nagai S. Radiologic Manifestations of Sarcoidosis in Various Organs. Radiographics 2004;24:87-104
  40. Kessler A, Mitchell DG, Israel HL, Goldberg BB. Hepatic and splenic sarcoidosis: Ultrasound and MR imaging. Abdom Imaging 1993;18:159-163
  41. Nakata K, Iwata K, Kojima K, Kanai K. Computed tomography of liver sarcoidosis. J Comput Assist Tomogr 1989;13:707-708
  42. Warshauer DM, Molina PL, Hamman SM, Koehler RE, Paulson EK, Bechtold RE. Nodular sarcoidosis of the liver and spleen: Analysis of 32 cases. Radiology 1995;195:757-762
  43. Warshauer DM, Semelka RC, Ascher SM. Nodular sarcoidosis of the liver and spleen: appearance on MR images. J Magn Reson Imaging 1994;4:553-557
  44. Alam I, Levenson SD, Ferrell LD, Bass NM. Diffuse intrahepatic biliary strictures in sarcoidosis resembling sclerosing cholangitis: Case report and review of the literature. Dig Dis Sci 1997;42:1295-1301
  45. Rudzki C, Ishak KG, Zimmerman HJ. Chronic intrahepatic cholestasis of sarcoidosis. Am J Med 1975;59:373-387
  46. Lagana SM, Moreira RK, Lefkowitch JH. Hepatic Granulomas: Pathogenesis and Differential Diagnosis. Clin Liver Dis 2010;14:605-617
  47. Gur C, Lalazar G, Doviner V, Fridlender ZG, Molcho V, Abu-Much S. Late-onset sarcoidosis after liver transplantation for primary biliary cirrhosis. J Clin Gastroenterol 2007;41:329-332
  48. Fiel MI, Shukla D, Saraf N, Xu R, Schiano TD. Development of hepatic granulomas in patients receiving pegylated interferon therapy for recurrent hepatitis C virus post liver transplantation. Transpl Infect Dis 2008;10:184-189
  49. Cremers JP, Drent M, Baughman RP, Wijnen PA, Koek GH. Therapeutic approach of hepatic sarcoidosis. Curr Opin Pulm Med 2012;18:472-482
  50. Moller DR. Treatment of sarcoidosis – from a basic science point of view. J Intern Med 2003;253:31-40
  51. Rose AS, Tielker MA, Knox KS. Hepatic, Ocular, and Cutaneous Sarcoidosis. Clin Chest Med 2008;29:509-524
  52. MacArthur KL, Forouhar F, Wu GY. Intra-abdominal complications of sarcoidosis. J Formos Med Assoc 2010;109:484-492
  53. Bécheur H, Dall'osto H, Chatellier G, Charton-Bain M, Aubertin J, Attar A. Effect of ursodeoxycholic acid on chronic intrahepatic cholestasis due to sarcoidosis. Dig Dis Sci 1997;42:789-791
  54. Bakker GJ, Haan YC, Maillette de Buy Wenniger LJ, Beuers U. Sarcoidosis of the liver: to treat or not to treat?. Neth J Med 2012;70:349-356
  55. Ayyala US, Padilla ML. Diagnosis and treatment of hepatic sarcoidosis. Curr Treat Options Gastroenterol 2006;9:475-483
  56. Baughman RP. Methotrexate for sarcoidosis. Sarcoidosis Vasc Dif 1998;15:147-149
  57. Doty JD, Mazur JE, Judson MA. Treatment of sarcoidosis with infliximab. Chest 2005;127:1064-1071
  58. Fidler HM, Hadziyannis SJ, Dhillon AP, Sherlock S, Burroughs AK. Recurrent hepatic sarcoidosis following liver transplantation. Transplant Proc 1997;29:2509-2510
  59. Costabel U, Hunninghake GW. ATS/ERS/WASOG statement on sarcoidosis. Eur Respir J 1999;14:735-737
  60. Iwai K, Tachibana T, Hosoda Y, Matsui Y. Sarcoidosis autopsies in Japan. Frequency and trend in the last 28 years. Sarcoidosis 1988;5:60-65
  61. Casavilla FA, Gordon R, Wright HI, Gavaler JS, Starzl TE, Van Thiel DH. Clinical course after liver transplantation in patients with sarcoidosis. Ann Intern Med 1993;118:865-866
  62. Barbers RG. Role of transplantation (lung, liver, and heart) in sarcoidosis. Clin Chest Med 1997;18:865-874
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Hepatic Sarcoidosis

Micheal Tadros, Faripour Forouhar, George Y. Wu
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