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Hepatic Sarcoidosis

Sarcoidosis of the Liver

Sarcoidosis is a multisystem inflammatory disorder characterized by the presence of granulomas in lymph nodes, lungs, and other organs. The liver is among the most common extrapulmonary sites compromised by the disease, and affected individuals are diagnosed with hepatic sarcoidosis.


Presentation

Up to 70% of patients suffering from HS are asymptomatic [6], while a majority of those who develop symptoms claim abdominal pain. In this context, an abdominal examination may reveal hepatomegaly and possibly splenomegaly [1]. Constitutive symptoms like fatigue, fever, and arthralgia have occasionally been reported. Few patients present with cholestasis and consequent pruritus, jaundice, pale stools and dark urine. Others suffer from portal hypertension. The latter commonly results from biliary cirrhosis and may lead to ascites, peripheral edema, and right heart failure [3]. Similarly, portal hypertension open link may be caused by the formation of thrombi in hepatic veins, a condition known as Budd-Chiari syndrome .

Of note, sarcoidosis is a systemic disease rarely limited to the liver. Thus, patients may present with lymphadenopathy, pulmonary sarcoidosis, skin lesions, uveitis and neurological deficits, among others. In fact, the presence of symptoms not consistent with hepatic disease is of major importance to diagnose HS.

Massive Splenomegaly
  • Hepatic rhabdomyosarcoma in an adult : a rare primary malignant liver tumor. case report and literature review Hepatic schistosomiasis with massive splenomegaly : a case report and literature review Price: 10,00 Chhagan Bihari, Archana Rastogi, Naveen[acta-gastroenterologica.be]
Weight Loss
  • Clinical presentation was with weight loss, hepatomegaly and abnormal liver function tests. In addition there was fever, itching, splenomegaly and abdominal lymphadenopathy in some. CT scan revealed mediastinal lymphadenopathy in all.[ncbi.nlm.nih.gov]
  • Abdominal pain and pruritus are noted 15% while fever, weight loss and jaundice less than 5%. There is elevation in serum transferases in 50-70% however, the degree is less than the alkaline phosphatase.[imrespdx.com]
  • He started noticing weight loss in 1994; over a period of a few months his weight decreased from 55-60 kg to 40 kg.[jpma.org.pk]
  • Hepatic sarcoidosis may present as fatigue, RUQ abdominal pain, pruritus, jaundice, weight loss, and fever. 3 Features of esophageal sarcoidosis include dysphagia from impaired motility, esophagitis, stricture, or extrinsic compression from paraesophageal[acgcasereports.gi.org]
  • Extra-hepatic manifestations Systemic signs of fatigue and weight loss were found in four patients. Pulmonary involvement was present in all patients and was symptomatic in the form of a dry cough or dyspnea.[panafrican-med-journal.com]
Malaise
  • Fever, malaise, and other systemic symptoms are the usual presenting manifestations of an infective etiology; prolonged FUO is especially common in TB and fungal infections. The history is critical in establishing a drug etiology.[hepatitiscentral.com]
Hepatomegaly
  • Clinical presentation was with weight loss, hepatomegaly and abnormal liver function tests. In addition there was fever, itching, splenomegaly and abdominal lymphadenopathy in some. CT scan revealed mediastinal lymphadenopathy in all.[ncbi.nlm.nih.gov]
  • Hepatomegaly was observed in all patients as well as abnormal serum liver function test reflected by anicteric cholestasis. Liver biopsy, showed in all granulomatous lesions consistent with sarcoidosis and severe fibrosis in 2 cases.[ncbi.nlm.nih.gov]
  • About half of patients had abnormal imaging study with hypodense nodular lesions being the most common abnormality (six patients) followed by hepatomegaly (three patients). Liver biopsy revealed non-caseating granuloma in 88% (14 of 16 patients).[ncbi.nlm.nih.gov]
  • […] lab test results and imaging studies and did not trigger any symptoms. 88% of patients with liver involvement had elevated alkaline phosphatase levels. 90% of those with liver involvement had high levels of gamma-glutamyl transferase. 3 patients had hepatomegaly[mdedge.com]
  • In this context, an abdominal examination may reveal hepatomegaly and possibly splenomegaly. Constitutive symptoms like fatigue, fever, and arthralgia have occasionally been reported.[symptoma.com]
Jaundice
  • Abstract Two cases of sarcoidosis with predominantly hepatic involvement are reported, both with the gradual development of progressive jaundice and other clinical features of primary biliary cirrhosis without immunologic or morphologic evidence of that[ncbi.nlm.nih.gov]
  • Microscopic hepatic involvement is common in these patients but is mostly clinically silent. specific symptoms include jaundice and pruritus, mostly from chronic cholestasis.[ncbi.nlm.nih.gov]
  • There are small trials suggesting the use of urosodeoxycholic acid in those with cholestatic jaundice with pruritus.[imrespdx.com]
  • KEYWORDS: Corticosteroids; jaundice; liver; sarcoidosis[ncbi.nlm.nih.gov]
  • Few patients present with cholestasis and consequent pruritus, jaundice, pale stools and dark urine. Others suffer from portal hypertension.[symptoma.com]
Scleral Icterus
  • Physical exam revealed scleral icterus, generalized lymphadenopathy, and tender hepatosplenomegaly. To evaluate his symptoms, an esophagogastroduodenoscopy (EGD) was done and showed esophageal varices and a gastric ulcer.[acgcasereports.gi.org]
Arthralgia
  • Constitutive symptoms like fatigue, fever, and arthralgia have occasionally been reported. Few patients present with cholestasis and consequent pruritus, jaundice, pale stools and dark urine. Others suffer from portal hypertension.[symptoma.com]
  • Abstract A 35-year-old man presented with a 3-year history of arthralgia and purple coloration of the skin of his fingers and feet. Hand and foot radiography showed cystic bone lesions on phalanges suggestive of sarcoidosis.[reumatologiaclinica.org]
Night Sweats
  • How to Stop Night Sweats in Men and Women Night sweats generally are a symptom of an underlying problem that may require medical treatment.[emedicinehealth.com]
  • He denied persistent fever, night sweats, cough, shortness of breath, change in appetite, diarrhea, lack of energy, joint pains or skin changes.[jpma.org.pk]
Dark Urine
  • Few patients present with cholestasis and consequent pruritus, jaundice, pale stools and dark urine. Others suffer from portal hypertension.[symptoma.com]

Workup

Elevated serum concentrations of alkaline phosphatase and γ-glutamyltransferase are characteristic of but not specific for HS. Indeed, the incidental detection of such anomalies is often the first hint at the disease. They may or may not be accompanied by increases in alanine aminotransferase and aspartate aminotransferase concentrations. Furthermore, enhanced levels of serum angiotensin-converting enzyme have repeatedly been measured in samples obtained from HS patients.

Foci of granulomatous inflammation may be visualized applying sonography, computed tomography, or magnetic resonance imaging [7] [8]. Granulomas typically depict as hypointense or hypodense areas and often concentrate in periportal regions. Granulomas are usually observed in lymph nodes and other end-organs, too [9]. Because isolated HS is very rare [10], the presence of granulomas in non-hepatic tissues is sometimes considered a prerequisite for the diagnosis of HS.

Based on blood tests and diagnostic imaging alone, it may be difficult to distinguish HS from tuberculosis. In order to do so, biopsy specimens have to be obtained and examined for the presence of acid-fast bacilli. Additionally, histopathological analyses of such samples allow to rule out malignancies. HS-related granulomas are non-caseating, epithelioid granulomas.

Liver Biopsy
  • The diagnosis of the hepatic sarcoidosis was confirmed by histopathological examination of liver biopsy. The patient was treated with corticosteroids.[ncbi.nlm.nih.gov]
  • Liver biopsy revealed non-caseating granuloma in almost all cases. Cirrhosis was seen in a significant number of patients.[ncbi.nlm.nih.gov]
  • Liver biopsy showed non caseating granulomas. As she had developed steroid-induced diabetes she was started on chloroquine and responded well with regression of the liver and spleen during one year of treatment.[ncbi.nlm.nih.gov]
  • Liver biopsy showed noncaseating epithelioid granulomas. Serum angiotensin converting enzyme was elevated in four cases. All patients had received anti-tuberculosis treatment with clinical diagnosis of hepatic tuberculosis.[ncbi.nlm.nih.gov]
  • The authors' protocol for management of patients with suspected sarcoidosis of the liver without focal lesions includes a transjugular liver biopsy with portal pressure measurements to confirm the diagnosis, rule out coexisting liver diseases and to identify[ncbi.nlm.nih.gov]

Treatment

  • The current body of research in regard to treatment suggests steroids to be the mainstay of therapy. However, there is a role for additional immunosuppressive therapy in cases where the initial treatment is refractory to steroids.[ncbi.nlm.nih.gov]
  • Not all cases of hepatic sarcoidosis require treatment. For symptomatic patients, the first line treatment includes corticosteroids or ursodeoxycholic acid.[ncbi.nlm.nih.gov]
  • We postulate that this could be related to cyclophosphamide treatment.[ncbi.nlm.nih.gov]
  • Evidence based guidelines for this location treatment is lacking and the effect of corticosteroids may be inadequate.[ncbi.nlm.nih.gov]
  • As she had developed steroid-induced diabetes she was started on chloroquine and responded well with regression of the liver and spleen during one year of treatment.[ncbi.nlm.nih.gov]

Prognosis

  • CONCLUSION: Portal hypertension due to sarcoid liver disease can have a favourable prognosis and early referral for port-systemic shunting may be inadvisable.[ncbi.nlm.nih.gov]
  • While HS is generally associated with a favorable prognosis, liver transplantation may become necessary in severe cases.[symptoma.com]
  • Course and prognosis of sarcoidosis around the world. Am. J. Mcd, 1974:57:847-52. 18.Edmondstone WM, Wilson AG, Sarcoidosis in Caucasians, Blacks, and Asians in London, Br. J. Dis. Chest, 1985:79:27-36. 19.Mana 3, Salazar A. Manresa F.[jpma.org.pk]
  • Seite 117 - Pulmonary sarcoidosis: role of CT in the evaluation of disease activity and functional impairment and in prognosis assessment. Radiology 191 (3): 675-680 141. ‎[books.google.de]
  • In some studies, the starting dose of 30 mg/day was proven sufficient to improve prognosis.[casesjournal.biomedcentral.com]

Etiology

  • INTRODUCTION: Sarcoidosis is a multisystem granulomatous disease of unknown etiology. Although hepatic granulomas occur in 50-65% of patients with systemic sarcoidosis, isolated liver sarcoidosis is rare.[ncbi.nlm.nih.gov]
  • Focus should be on discovering the etiology of the disease to target therapy at prevention, not cure.[ncbi.nlm.nih.gov]
  • Necroinflammatory changes included spotty necrosis suggesting hepatitis of diverse etiologies (including viral infection and drug reaction) and chronic portal inflammation suggestive of chronic active hepatitis.[ncbi.nlm.nih.gov]
  • Sarcoidosis is an inflammatory disease of unknown etiology that may comprise distinct organ systems. While pulmonary sarcoidosis is most frequently observed, up to 70% of sarcoidosis patients present with non-caseating granulomas in hepatic tissues.[symptoma.com]
  • We were then left with a list of infiltrative etiologies, malignancy and drug/toxins. Liver biopsy was the key to the diagnosis. According to UpToDate, 50-65% of patients with sarcoidosis have granulomas on liver biopsy.[imrespdx.com]

Epidemiology

  • .); and the Department of Biostatistics and Research Epidemiology, Henry Ford Health System, Detroit (B.A.R.). Address reprint requests to Dr.[nejm.org]
  • Hospital, age, racial, occupational, geographical, clinical and survivorship characteristics in the epidemiology of sarcoidosis. Am. J. Epidemiol., 1971:94:222-30. 17.Siltzbach LE, James DG. Neville F. et al.[jpma.org.pk]
  • Epidemiology of sarcoidosis: recent advances and future prospects. Semin Respir Crit Care Med 2007;28:22–35. 4. Kang EH. Sarcoidosis in Korea: revisited. J Korean Med Assoc 2008;51:925–932. 5.[synapse.koreamed.org]
  • Pulmonary hypertension in advanced sarcoidosis: epidemiology and clinical characteristics. CT findings in severe thoracic sarcoidosis.[chronicillnessrecovery.org]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology A granuloma is a localized collection of chronic inflammatory cells with epithelioid cells and giant multinucleated cells. Caseation necrosis or foreign body tissue (eg, schistosome eggs) may be present.[merckmanuals.com]
  • Pathophysiology Granuloma formation is incompletely understood. The lesions are regarded as a host attempt to protect against poorly soluble exogenous or endogenous irritants.[hepatitiscentral.com]

Prevention

  • The results suggest that HRT may help to prevent the progression of sarcoidosis in postmenopausal patients.[ncbi.nlm.nih.gov]
  • Corticosteroids did not prevent the development of portal hypertension.[ncbi.nlm.nih.gov]
  • Focus should be on discovering the etiology of the disease to target therapy at prevention, not cure.[ncbi.nlm.nih.gov]
  • Focus should be on discovering the etiology of the disease to target therapy at prevention, not cure. Keywords Infliximab Sarcoidosis Primary Biliary Cirrhosis Brucellosis Main Side Effect These keywords were added by machine and not by the authors.[link.springer.com]
  • Prevention Can sarcoidosis be prevented or avoided? There is no way to avoid getting sarcoidosis. If you are at higher risk for the disease, it may be better for you to avoid environmental substances that are known to trigger it.[familydoctor.org]

Summary

Sarcoidosis is an inflammatory disease of unknown etiology that may comprise distinct organ systems. While pulmonary sarcoidosis is most frequently observed, up to 70% of sarcoidosis patients present with non-caseating granulomas in hepatic tissues [1]. This condition is referred to as hepatic sarcoidosis (HS). Affected individuals are mostly asymptomatic, but may develop cholestasis and portal hypertension. To date, there is no cure for HS and patients are most commonly prescribed anti-inflammatory drugs to limit disease progression [2] [3]. While HS is generally associated with a favorable prognosis, liver transplantation may become necessary in severe cases [4]. Otherwise, sarcoidosis-related morbidity and mortality mainly result from pulmonary, cardiac and central nervous system involvement [5].

References

Article

  1. Tadros M, Forouhar F, Wu GY. Hepatic Sarcoidosis. J Clin Transl Hepatol. 2013; 1(2):87-93.
  2. Modaresi Esfeh J, Culver D, Plesec T, John B. Clinical presentation and protocol for management of hepatic sarcoidosis. Expert Rev Gastroenterol Hepatol. 2015; 9(3):349-358.
  3. Syed U, Alkhawam H, Bakhit M, Companioni RA, Walfish A. Hepatic sarcoidosis: pathogenesis, clinical context, and treatment options. Scand J Gastroenterol. 2016; 51(9):1025-1030.
  4. Bilal M, Satapathy SK, Ismail MK, Vanatta JM. Long-Term Outcomes of Liver Transplantation for Hepatic Sarcoidosis: A Single Center Experience. J Clin Exp Hepatol. 2016; 6(2):94-99.
  5. Hu X, Carmona EM, Yi ES, Pellikka PA, Ryu J. Causes of death in patients with chronic sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2016; 33(3):275-280.
  6. Dulai PS, Rothstein RI. Disseminated sarcoidosis open link presenting as granulomatous gastritis: a clinical review of the gastrointestinal and hepatic manifestations of sarcoidosis. J Clin Gastroenterol. 2012; 46(5):367-374.
  7. Ufuk F, Herek D. CT of Hepatic Sarcoidosis: Small Nodular Lesions Simulating Metastatic Disease. Pol J Radiol. 2015; 80:945-954.
  8. Ferreira A, Ramalho M, de Campos RO, et al. Hepatic sarcoidosis: MR appearances in patients with chronic liver disease. Magn Reson Imaging. 2013; 31(3):432-438.
  9. Ebert EC, Kierson M, Hagspiel KD. Gastrointestinal and hepatic manifestations of sarcoidosis. Am J Gastroenterol. 2008; 103(12):3184-3192; quiz 3193.
  10. Jovicić I, Popović DDj, Toncev L, et al. Isolated hepatic sarcoidosis. Vojnosanit Pregl. 2014; 71(4):399-403.

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Last updated: 2018-06-22 04:37