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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.

Abdominal Lymphadenopathy
  • In addition there was fever, itching, splenomegaly and abdominal lymphadenopathy in some. CT scan revealed mediastinal lymphadenopathy in all. Liver biopsy showed noncaseating epithelioid granulomas.[ncbi.nlm.nih.gov]
  • Extensive abdominal lymphadenopathy in sarcoidosis. Am J Gastroenterol 1981; 75 :367–9. 26. Noel, JM, Katona, IM, Pineiro-Carrero, VM. Sarcoidosis resulting in duodenal obstruction in an adolescent. J Ped Gastroenterol Nutr 1997; 24 :594–598. 27.[nature.com]
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]
  • More generalized symptoms of sarcoidosis include: weight loss, fatigue, night sweats, fever, or just an overall feeling of ill health. It is important to note that sarcoidosis is usually not crippling.[emedicinehealth.com]
Malaise
  • We reported a 69-year-old female patient with abdominal pain, malaise, vertigo, headaches, hands tremor and partial loss of hearing.[ncbi.nlm.nih.gov]
  • 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]
Pathologist
  • Liver Pathology will be essential reading for every pathologist who evaluates liver pathology specimens. In addition it will be a valuable resource for pathology residents and fellows.[books.google.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]
  • There are small trials suggesting the use of urosodeoxycholic acid in those with cholestatic jaundice with pruritus.[imrespdx.com]
  • Few patients present with cholestasis and consequent pruritus, jaundice, pale stools and dark urine. Others suffer from portal hypertension.[symptoma.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]
  • 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]
  • […] 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]
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]
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]
  • She endorsed a 13-pound weight loss over the prior few months but denied additional symptoms, including fevers, chills, night sweats, and other gastrointestinal symptoms. Physical examination found scleral icterus but was otherwise unremarkable.[acgcasereports.gi.org]
  • General symptoms of Sarcoidosis include fever, sleep problems, night sweats, fatigue, weakness, and a sense of feeling ill.[disabled-world.com]
  • Weight loss, fatigue, night sweats, fever, or just an overall feeling of ill health can also be clues to the disease.[medicinenet.com]
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]
Dark Urine
  • Few patients present with cholestasis and consequent pruritus, jaundice, pale stools and dark urine. Others suffer from portal hypertension.[symptoma.com]
Hand Tremor
  • We reported a 69-year-old female patient with abdominal pain, malaise, vertigo, headaches, hands tremor and partial loss of hearing.[ncbi.nlm.nih.gov]

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 Enzymes Abnormal
  • 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[ncbi.nlm.nih.gov]
Treponema Pallidum
  • Tests for Toxoplasma gondii, Bartonella, Brucella, Treponema pallidum, and Histoplasma were negative. Purified protein derivative (PPD) skin test was non-reactive. Chest computed tomography (CT) showed hilar and mediastinal lymphadenopathy.[acgcasereports.gi.org]
Toxoplasma Gondii
  • Tests for Toxoplasma gondii, Bartonella, Brucella, Treponema pallidum, and Histoplasma were negative. Purified protein derivative (PPD) skin test was non-reactive. Chest computed tomography (CT) showed hilar and mediastinal lymphadenopathy.[acgcasereports.gi.org]
Bloody Ascites
  • Bloody ascites in sarcoidosis. Chest 1985; 88 :917–918. 99. Trimble, EL, Saigo, PE, Freeberg, GW, et al. Peritoneal sarcoidosis and elevated CA 125. Obstetrics Gynecol 1991; 78 :976–977. 100. Uthman, IW, Bizri, AR, Shabb, NS, et al.[nature.com]
Granulomatous Tissue
  • The infiltrative mass was likely an outgrowth of the granulomatous tissue infiltration or pseudotumor, giving the appearance of an obstructing tumor. The mass resolved with steroid therapy. A unifying diagnosis was initially difficult.[acgcasereports.gi.org]
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]

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]

Prognosis

  • 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

  • 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]
  • 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]

Epidemiology

  • 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]
  • .); and the Department of Biostatistics and Research Epidemiology, Henry Ford Health System, Detroit (B.A.R.). Address reprint requests to Dr.[nejm.org]
  • "The epidemiology of sarcoidosis in Rochester, Minnesota: a population-based study of incidence and survival" (PDF). American Journal of Epidemiology. 123 (5): 840–5. PMID 3962966.[en.wikipedia.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]
  • The exact mechanism of this relation is not known, but some evidence supports the hypothesis that this is a consequence of Th1 lymphokine prevalence. [55] [82] Tests of delayed cutaneous hypersensitivity have been used to measure progression. [83] Pathophysiology[en.wikipedia.org]

Prevention

  • The results suggest that HRT may help to prevent the progression of sarcoidosis in postmenopausal patients.[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: 2019-07-11 21:19