Hepatic sarcoidosis resembling primary sclerosing cholangitis

Chen, Ying; Ciobanu, C; Mohrmann, Laurel

doi:10.1136/bcr-2021-243492

Cited by 3 publications

(3 citation statements)

References 25 publications

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“…In one study, budesonide with a dosing of 3 mg twice daily was used. However, data on the duration of maintenance doses were unavailable 19 . With regard to UDCA, the dosage that was prescribed ranged from 10 to 15 mg/kg or 300 mg twice daily.…”

Section: Resultsmentioning

confidence: 99%

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The clinical management of hepatic sarcoidosis: A systematic review

Sinnanaidu,

Kumar,

Shunmugam

et al. 2024

JGH Open

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BackgroundHepatic 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.MethodsUsing 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.ResultsOut 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.ConclusionThe 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.

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Section: Resultsmentioning

confidence: 99%

“…However, data on the duration of maintenance doses were unavailable. 19 With regard to UDCA, the dosage that was prescribed ranged from 10 to 15 mg/kg or 300 mg twice daily. For symptom improvement, 113 (60.2%) showed symptom resolution post-treatment with corticosteroids in 23 studies.…”

Section: Treatment Regimensmentioning

confidence: 99%

The clinical management of hepatic sarcoidosis: A systematic review

Sinnanaidu,

Kumar,

Shunmugam

et al. 2024

JGH Open

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“…In some reported cases, patients were initially misdiagnosed with PSC. 11,12 Our patient was initially suspected of having PSC owing to his clinical presentation, but a negative history of inflammatory bowel disease and negative p-ANCA test results prompted the physician to perform a liver biopsy.…”

Section: Investigations and Treatmentmentioning

confidence: 99%

Cirrhosis due to hepatic sarcoidosis: A rare presentation

Islam,

Kamal,

Murshed

et al. 2024

Clinical Case Reports

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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.

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Secondary sclerosing cholangitis and IgG4-sclerosing cholangitis – A review of cholangiographic and ultrasound imaging

Möller

Braden

Culver

et al. 2022

Endoscopic Ultrasound

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Sclerosing cholangitis (SC) represents a spectrum of chronic progressive cholestatic diseases of the intrahepatic and/or extrahepatic biliary system characterized by patchy inflammation, fibrosis, and stricturing. Primary and secondary SC must be distinguished given the different treatment modalities, risks of malignancy, and progression to portal hypertension, cirrhosis, and hepatic failure. This review focuses on secondary SC and the pathogenic mechanisms, risk factors, clinical presentation, and novel imaging modalities that help to distinguish between these conditions. We explore the detailed use of cholangiography and ultrasound imaging techniques.

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Hepatic sarcoidosis resembling primary sclerosing cholangitis

Cited by 3 publications

References 25 publications

The clinical management of hepatic sarcoidosis: A systematic review

The clinical management of hepatic sarcoidosis: A systematic review

Cirrhosis due to hepatic sarcoidosis: A rare presentation

Secondary sclerosing cholangitis and IgG4-sclerosing cholangitis – A review of cholangiographic and ultrasound imaging

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