Hepatic Sarcoidosis-an Unusual Cause of Jaundice: a Case Report

Introduction: Sarcoidosis is a chronic disorder. Its hallmark is the presence of noncaseating granuloma. Most cases are asymptomatic. However, few of them progress to liver cirrhosis and liver failure. Treatment of hepatic sarcoidosis is challenging as there is no large randomized controlled till date has been done to evaluate the e�cacy of drugs. The Unique characteristic of this case report is that Sarcoidosis may present with jaundice and need to be borne in mind when a patient presenting with jaundice and no usual cause failed to be identify any usual cause. Despite having sarcoidosis, the patient did not have any respiratory symptom. Case presentation: We are presenting a case of hepatic sarcoidosis who presented with abdominal pain, fever and jaundice. After excluding of all common causes of jaundice, hepatic sarcoidosis was diagnosed with liver biopsy. Conclusion: High levels of suspicion is needed to diagnose hepatic sarcoidosis as this is an unusual cause of jaundice. Although rare, hepatic sarcoidosis can be treated with steroids. The prime take-away message from this case report is to consider Sarcoidosis as an unusual but treatable cause of jaundice when the conventional causes are failed to be the cause of jaundice.


Introduction
Sarcoidosis is a chronic systemic in ammatory disorder which can affect multiple organs (1).The hallmark of this disorder is the formation of noncaseating granuloma [2].The worldwide prevalence of Sarcoidosis is 2-60 per 100000 people [3].It is more prevalent in Scandinavian countries as well as African Americans [3].Sarcoidosis affects all racial as well as ethnic groups.Females are mostly affected than the males.It usually develops before 50 years of age with the peak age of incidence is between 20-40 years [4].Sarcoidosis can affect virtually any organ, However, Pulmonary system is affected in 90% of cases [5].Liver is being affected in upto 70% cases [3].However, the clinical spectrum of hepatic sarcoidosis range from being asymptomatic to development of liver cirrhosis requiring liver transplantation.Here, we present a case report of hepatic sarcoidosis who was diagnosed by liver biopsy.

CASE REPORT
Mrs. X ; 40 years married female got admitted on 12/3/23 with high grade irregular fever without chill & rigor; relieving on taking anti pyretic that started about 1 year back.She had no history of contact with active TB patient.She also had upper abdominal pain with anorexia and unintentional signi cant weight loss.Pain was dull aching mild to moderate in intensity, without any aggravating or relieving factors and get subsided on analgesic or antispasmodic.She also had gradual upper abdominal distension with a feeling of heaviness in the right upper quadrant for the last 6 months.She gave no history of jaundice, hematemesis, melaena, dysuria, hematuria, cough, breathlessness, chest pain, oedema, red painful eyes, visual disturbance.She was mildly anemic.
Her liver was enlarged about 10cm, nontender, smooth surfaced, rm in consistency with rounded inferior margin; no hepatic bruit or rub.Spleen was enlarged 5 cm from the left costal margin along its axis.Her investigation ndings revealed low hemoglobin of 8.5gm/dl with High ESR of 25m in 1st hour.Her serum bilirubin was 2.5 mg/dl ,SGPT/ALT was 48IU/L with ALP 1401 IU/L.Her chest x ray revealed bilateral hilar lymphadenopathy.CT scan of abdomen revealed hepatomegaly.Hepatic Fibroscan revealed 10.3kPa.She also had raised ACE level of 218u/L.Liver biopsy revealed multiple small noncaseating granulomas in the portal area made of epithelioid cell, giant cells and small Number of lymphocytes (Fig. 1).Portal areas also reveals moderate in ltration of chronic in ammatory cells and show periportal Fibrosis.She was put on Prednisolone 40mg once daily.Following the steroid induction, she was followed up six weeks later when her Liver function test normalized.Hence, she was maintained on 10mg once daily as a maintenance dose.

DISCUSSION
Sarcoidosis is a multisystem disorder of unknown etiology characterized by non-caseating granulomas.
Its pathogenesis is unclear [4].Liver is the third organ being mostly affected after lungs and lymph nodes.
Extrapulmonary sarcoidosis may occur with or without lungs being involved [4].Isolated extrapulmonary sarcoidosis occur in less than 10% cases [7].The clinical spectrum of hepatic sarcoidosis range from being asymptomatic to liver cirrhosis causing liver failure [3].Only 5-30% patients show clinical signs symptoms [7].About 10-30% cases have elevated liver enzymes whereas 20% patients show palpable hepatomegaly [3].Few patients develop liver cirrhosis or end stage liver disease and require liver transplantation [8].Abdominal pain has been reported in 15% cases while jaundice has been reported in 5% on speci c symptoms include fever, malaise, arthralgia [2].Liver function tests usually reveals raised ALP levels 5-10 times the upper limit of the normal range whereas ALT levels are generally mildly raised [2].Serum ACE level is raised in 60% cases [2].Imaging studies like MRI or CT scan or Ultrasonography may reveal hepatomegaly or hypointense or hypo attenuated liver nodules.Presence of such nodules may confuse with metastatic or other granulomatous diseases [9].Liver biopsy followed by histopathology is the gold standard to diagnose sarcoidosis.Noncaseating and Epithelioid Granulomas are abundant and are mainly in the portal and periportal regions [10].Granulomas are reported in 60%-80% cases of liver sarcoidosis [11].Management of Hepatic Sarcoidosis include observation and medical management.Observation is indicated when the patient is asymptomatic [3].Medical therapy is indicated in case of severe hepatic dysfunction as well as marked increased in levels of transaminases [11].Steroids in the form of prednisolone is the drug of choice.Marked abdominal pain with hepatomegaly as well as constitutional symptoms like fever, night sweats and arthralgias are other indications to commence steroids [3].Ursodeoxycholic acid is used in case of cholestasis.Other second line drugs include Azathriopine, Methotrexate, In iximab, Cyclosporine etc [3].However, no large randomized controlled trials have so far been undertaken to assess the e cacy of any of these drugs [7].In advanced sarcoidosis, Liver transplantation may be the only options to be considered for cure [7].The limitation of our case report is that we could not do the follow up biopsy which was needed to con rm the histologic resolution.
The authors would like to acknowledge the department of Histopathology of Sheikh Russel National Gastroliver Institute and Hospital for their valuable inputs.

Supplementary Files
This is a list of supplementary les associated with this preprint.Click to download.

Figure 1 Liver
Figures