Hepatic Sarcoidosis Research Articles

The Varying Histology of Hepatic Sarcoidosis and the Relation of Bile Duct Damage and Loss to the Presence of Portal Hypertension and Cirrhosis

The Varying Histology of Hepatic Sarcoidosis and the Relation of Bile Duct Damage and Loss to the Presence of Portal Hypertension and Cirrhosis

A rare case of Isolated hepatic sarcoidosis with symptomatic hypercalcemia

A rare case of Isolated hepatic sarcoidosis with symptomatic hypercalcemia

A rare case of hepatic sarcoidosis, masquerading as lymphoma

A rare case of hepatic sarcoidosis, masquerading as lymphoma

Imaging findings in a rare case of alveolar, hepatic and splenic sarcoidosis presenting with thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) is an acute haematological disorder characterized by severe ADAMTS13 enzyme deficiency, leading to consumptive thrombocytopenia, mechanical haemolysis, and organ damage. Its association with multisystemic sarcoidosis is extremely rare and, to the best of our knowledge, described in only two cases so far. We present the case of a 52-year-old woman with TTP and antibodies anti-ADAMTS13, showing computed tomography (CT) and magnetic resonance imaging (MRI) evidence of pulmonary, hepatic, and splenic lesions initially reported as ischemic/inflammatory changes. A follow-up MRI of the abdomen revealed increased evidence of the liver lesions, focal intrahepatic bile duct dilatation, splenic lesions, and enlarged hepatic hilar nodes. The follow-up chest CT showed increased evidence of the parenchymal lung consolidations. Given the radiological persistence of those alterations and the history of TTP, the hypothesis of an IgG4-related disease was then made. The IgG4 levels were found to be normal, while the histological examination of the liver revealed non-necrotizing granulomatous chronic inflammation. Elevated levels of angiotensin-converting enzyme were found, and the QuantiFERON-TB Gold test was negative for tuberculosis infection. Thus, the overall clinical picture was consistent with multisystemic sarcoidosis (alveolar, hepatic, and splenic). The diagnosis of sarcoidosis, already challenging due to the variability of its clinical presentation, can become even more complicated when it manifests with uncommon haematological manifestations such as TTP, along with non-specific extra-pulmonary involvement. While imaging aids in documenting organ damage, the definitive diagnosis of sarcoidosis necessitates histologic confirmation of noncaseating granulomas and the exclusion of other possible granulomatous diseases.

The clinical management of hepatic sarcoidosis: A systematic review.

Hepatic sarcoidosis is an uncommon clinical condition in which clear recommendations are lacking in its treatment. We aimed to review systematically the literature on hepatic sarcoidosis treatment to guide clinicians. Using MEDLINE, PubMed, CINAHL, Cochrane Library, and Google Scholar databases, we searched original articles on clinical studies reporting the outcome of adult hepatic sarcoidosis patients following treatment with various pharmacological agents. The primary end point was focused on assessing symptomatic relief and biochemical improvement posttreatment. Out of 614 retrieved references, 34 published studies were eligible, providing data for a total of 268 patients with hepatic sarcoidosis. First-line therapy with corticosteroids alone was reported in 187 patients, whilst ursodeoxycholic acid (UDCA) was used in 40 patients. Symptomatic and biochemical responses were reported among 113(60.4%) and 80(42.8%) cases of corticosteroids respectively, whereas UDCA showed a complete response in 23(57.5%) patients. Second-line therapy was used in steroid-refractory cases, with most cases being reported for azathioprine (n = 32) and methotrexate (n = 28). Notably, 15(46.9%) and 11(39.2%) patients showed both clinical and biochemical responses respectively. Biological therapy including anti-tumor necrosis factor (anti-TNF) was used as third line therapy in twelve cases with a 72.7% symptomatic and biochemical response rate each. The quality of evidence for the treatment of hepatic sarcoidosis was poor. Nevertheless, it appears that corticosteroid or UDCA may be utilized as first-line therapy. For cases that are refractory to corticosteroids, steroid-sparing immunosuppressive agents and anti-TNF have shown some promising results, but further high-quality studies are required.

Cirrhosis due to hepatic sarcoidosis: A rare presentation

Key Clinical MessageSarcoidosis, although predominantly affecting the lungs, can present with cirrhosis, posing diagnostic challenges. Elevated ACE levels and atypical liver enzyme patterns should prompt consideration of sarcoidosis in cryptogenic cirrhosis cases, necessitating comprehensive evaluation including liver biopsy and imaging for accurate diagnosis and timely management.AbstractSarcoidosis is a systemic disease that can affect various organs, leading to a diverse range of clinical manifestations that make diagnosis challenging. Here, we present a case of sarcoidosis in a middle‐aged male who presented with cirrhosis. The cause of cirrhosis remained unknown for 4 years until the development of lymphadenopathy and ground‐glass opacities on lung imaging. A liver biopsy was performed, which revealed noncaseating granulomatous inflammation, thereby identifying sarcoidosis as the cause of cirrhosis. The patient was treated with oral steroids, which slightly improved his liver function over a short period. Given the diverse presentations of sarcoidosis, it should be considered as a possible differential diagnosis in cases of cryptogenic cirrhosis.

Liver biopsy as a useful diagnostic tool for hepatic sarcoidosis: A case report

Liver biopsy as a useful diagnostic tool for hepatic sarcoidosis: A case report

Clinical challenges in the management of hepatic sarcoidosis.

Clinical challenges in the management of hepatic sarcoidosis.

Isolated hepatic sarcoidosis: A case series.

Sarcoidosis is a multi-organ inflammatory disease that can have hepatic involvement in up to 80% of cases. Rarely, sarcoidosis can manifest with only confined disease to the liver. While most patients with hepatic sarcoidosis are clinically silent, certain cases can have insidious onset leading to cirrhosis and secondary complications. Here, we describe three cases of isolated hepatic sarcoidosis to illustrate the range of presentations that may be associated with this condition. Clinicians should be vigilant in consideration of hepatic sarcoidosis as a culprit when investigating patients with undifferentiated liver disease.

S1468 Factors Associated With Transplant Waitlist Outcomes Among Patients With Hepatic Sarcoidosis

S1468 Factors Associated With Transplant Waitlist Outcomes Among Patients With Hepatic Sarcoidosis

S3841 Calling the Uncommon Common? Hepatic Sarcoidosis Presenting as Cirrhosis and Portal Hypertension

S3841 Calling the Uncommon Common? Hepatic Sarcoidosis Presenting as Cirrhosis and Portal Hypertension

Diagnostic value of elevated serum angiotensin-converting enzyme and lymphopenia in patients with granulomatous hepatitis.

Granulomatous hepatitis (GH) is associated with various aetiologies, especially inflammatory and infectious disorders. Sarcoidosis is a granulomatous disease in which the liver is the fourth most affected organ. Since epithelioid cell granulomas are not specific to sarcoidosis and since most patients with hepatic sarcoidosis are asymptomatic, valuable diagnostic biomarkers are needed to support the diagnosis of sarcoidosis. This study proposes to assess the diagnostic value of serum angiotensin converting enzyme (sACE) and lymphopenia in GH for sarcoidosis. We retrospectively analyzed the records of 90 patients referred to the internal medicine or hepatogastroenterology departments of the Lyon University Hospital (Lyon, France) between March 2002 and January 2020 in a context of GH. In our tertiary center, 38 patients with sarcoidosis were identified among 73 patients with GH. Lymphopenia had a high specificity (85.7%), which increased when combined with elevated (97.0%). Interestingly, specificity increased in patients under 50 years old (100%). Those results suggests that lymphopenia and sACE may be valuable biomarkers for sarcoidosis diagnosis in GH when combined, especially in younger patients.

Isolated Hepatosplenic Sarcoidosis- An Unusual Presentation

Sarcoidosis is a rare autoimmune condition with appearance of non-caseating granulomas in multiple sites most commonly involving pulmonary sites and hilar lymph nodes. Females are more commonly affected than males with peak age of diagnosis between 20 to 40 years. Hepatic Sarcoidosis occurs in 11% to 80% of cases and is mostly asymptomatic. Symptoms include jaundice, nausea, vomiting, abdominal pain, fatigue, pruritus, fever, arthralgias and hepatosplenomegaly. Herein, we report an unusual case of isolated hepatosplenic sarcoidosis encounterd in our routine practice.

Hepatic Sarcoidosis Presenting as Cholestatic Liver Injury Exacerbated by Nitrofurantoin Use

Please view the PDF to see the formatted meeting abstract.

Identification of isolated hepatic sarcoidosis with [18F]FDG and [68Ga]Ga-FAPI PET/CT.

Identification of isolated hepatic sarcoidosis with [18F]FDG and [68Ga]Ga-FAPI PET/CT.

Survey on hepatic sarcoidosis in Italy

Survey on hepatic sarcoidosis in Italy

An interesting presentation of hepatic sarcoidosis

An interesting presentation of hepatic sarcoidosis

Hepatic sarcoidosis with symptomatic portal hypertension: A report of 12 cases with review of the literature.

Sarcoidosis is a systemic granulomatosis of unknown etiology, characterized by the presence of immune granulomas. Liver damage is a relatively common extra-pulmonary manifestation, occurring in 3.6-30% of cases. Some patients can develop symptomatic portal hypertension (PH). Few series have evaluated the prognosis of symptomatic PH as well as the efficacy and safety of specific treatment on this complication. This is a multicenter retrospective study of cases of histologically proven hepatic sarcoidosis with symptomatic PH (ascites, digestive hemorrhage) and/or hepatic encephalopathy. Demographic characteristics, comorbidities, clinical manifestations of sarcoidosis, biological data, imaging study of the liver, treatment, and clinical outcomes were collected. Twelve patients were identified, with a mean follow-up of 140 months. The M/F ratio was 1 and Caucasian origin was the most represented (75%). Seven patients presented with hepatic comorbidities: metabolic syndrome, chronic alcoholism or chronic viral hepatitis. Apart from hepatic involvement, mediastino-pulmonary involvement was the most common followed by osteoarticular and skin. Liver damage was inaugural in two thirds of cases. Nine patients developed ascites, six presented esophageal varices complicated by gastrointestinal bleeding. Three patients presented with both ascites and variceal bleeding. One case of hepatic encephalopathy was observed. Five patients presented signs of hepatocellular insufficiency during follow-up, of whom three had hepatic comorbidities. Eight out of 12 patients required second-line treatment after failure of corticosteroids, three patients underwent ligation of esophageal varices but with recurrent digestive bleeding in all cases. Two patients benefited from a transjugular intrahepatic portosystemic shunt (TIPS), also with poor result. At the end of follow-up, five patients were alive and seven patients died. Two patients received a liver transplant, with good result and without recurrence of sarcoidosis on the transplant thereafter. Two patients had quiet sarcoidosis on low dose of corticosteroids and one patient was lost to follow-up. Symptomatic PH related to hepatic sarcoidosis is a severe complication, with high morbidity and mortality, and frequent failure of specific treatments of PH. Early management of these patients, with detection of hepatic comorbidities seems important. In case of therapeutic failure, liver transplantation is an option to consider.

A case of hepatic and bone marrow sarcoidosis with progressive renal failure: diagnostic and therapeutic challenges.

Sarcoidosis is a systemic granulomatous disease, sometimes characterized by an extrapulmonary localization in 30 - 50% of cases. We describe a 60-year-old Italian man with an unexplained history of fatigue, ascitis and progressive renal function impairment. Diagnosis of hepatic and bone marrow sarcoidosis was established by histology, and fast improvement of renal function was obtained after starting corticosteroid therapy. Atypical presentation and simultaneous involvement of liver, bone marrow and kidneys make diagnosis of extrapulmonary sarcoidosis still a diagnostic challenge. Delayed diagnosis could lead to serious organ damage like a progressive severe kidney failure.

STAGE 0 HEPATIC SARCOIDOSIS WITH SUPERIMPOSED PRIMARY BILIARY CIRRHOSIS

STAGE 0 HEPATIC SARCOIDOSIS WITH SUPERIMPOSED PRIMARY BILIARY CIRRHOSIS