Primary biliary cirrhosis and hepatic sarcoidosis: A case report

Alempijević, Tamara; Sokic-Milutinovic, Aleksandra; Toncev, Ljubisa; Pavlovic-Markovic, Aleksandra; Djuranović, Srdjan; Tomanović, Nada; Drulović, Jelena

doi:10.2298/vsp1401083a

Cited by 4 publications

(5 citation statements)

References 20 publications

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“…В основе патогенеза заболевания лежит развитие воспалительного процесса, который преимущественно опосредуется Т-хелперами 1-го типа и макрофагами и ведет к формированию неказеозных эпителиоидно-клеточных гранулем [11]. Саркоидоз чаще диагностируется у пациентов в возрасте от 25 до 40 лет, несколько чаще болеют женщины [13]. Поражение печени при саркоидозе отмечается достаточно часто: 60-65% по данным морфологических исследований [12].…”

Section: Discussionunclassified

“…Гепатомегалия присутствует более чем у 1/2 больных. Отклонения печеночных функциональных тестов выявляются приблизительно у 35% пациентов [13]. В 90% случаев отмечается повышение активности ЩФ, в 50-70% -аминотрансфераз, γ-глутамилтранс феразы, уровней общего и прямого билирубина [14].…”

Section: Discussionunclassified

“…В первом случае чаще выявляются повреждение эпителия желчных протоков и исчезновение мелких внутрипеченочных желчных протоков. В портальных трактах обнаруживается инфильтрация из CD4+ и CD8+ Т-лимфоцитов, В-лимфоцитов, а также макрофагов, эозинофилов и NK-клеток [13]. Дуктопения (уменьшение количества внутрипеченочных желчных протоков) -типичное проявление ПБХ, однако в редких случаях также может наблюдаться и при саркоидозе печени [17].…”

Section: Discussionunclassified

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Sarcoidosis and primary biliary cholangitis in a patient with cholestasis

Brovko

Akulkina

Kalashnikov

et al. 2021

Terapevticheskii arkhiv

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Primary biliary cholangitis and sarcoidosis are both cholestatic liver diseases. Currently, there are no established specific criteria for distinguishing the diseases from each other; diagnosis is based on the anamnesis, as well as the results of physical, laboratory and instrumental examination. The case report presents a female patient with a rare combination of histologically verified liver sarcoidosis and primary biliary cholangitis. Despite the similar clinical manifestations, the approaches to the treatment of these diseases are completely different, that underlines the importance of the differential diagnosis to exclude combined liver damage.

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Section: Discussionunclassified

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Sarcoidosis and primary biliary cholangitis in a patient with cholestasis

Brovko

Akulkina

Kalashnikov

et al. 2021

Terapevticheskii arkhiv

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“…Generally, about one in three patients with an autoimmune liver disease is diagnosed with a concomitant extrahepatic autoimmune disease, which may include rheumatological, endocrine, gastrointestinal, pulmonary, or dermatological conditions. Rare cases of autoimmune liver diseases, such as cholangiocarcinoma, 5 hepatic sarcoidosis, 6 pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome, 7 and primary cutaneous amyloidosis, 8 have also been reported. The pathogenesis of these conditions includes the activation of both innate and adaptive immune responses targeting cholangiocytes as well as different extrahepatic tissues.…”

Section: Discussionmentioning

confidence: 99%

Primary biliary cirrhosis–autoimmune hepatitis overlap syndrome in a patient with paroxysmal nocturnal hemoglobinuria: a case report

et al. 2021

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Primary biliary cirrhosis (PBC)–autoimmune hepatitis (AIH) overlap syndrome is frequently associated with extrahepatic autoimmune disorders. Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disease that is characterized by complement-mediated hemolysis due to erythrocyte membrane defects. However, autoimmune liver disease was not previously reported to be associated with PNH. A 37-year-old female patient was referred to our hospital with elevated liver enzymes and hematuria. On the basis of the symptoms and results of laboratory tests, radiographic studies, and pathologic results, she was diagnosed with PBC–AIH overlap syndrome and PNH. She was treated with a combination of ursodeoxycholic acid and prednisolone. The patient was symptom-free, with laboratory findings within near-normal range. The patient had recovered well at the 24-month follow-up evaluation. While we acknowledge that this was a single case, these findings expand our knowledge of immunological diseases that are associated with PNH and suggest an immune-mediated pathogenic pathway between PNH and PBC–AIH overlap syndrome. The combination of ursodeoxycholic acid and prednisolone can achieve therapeutic success. Routine follow-up of these patients is necessary to document disease progression.

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“…Antimitochondrial antibody serology is helpful; however, sarcoidosis and PBC are not mutually exclusive, and coexisting sarcoid and PBC have been reported. 96,97 With chronic cholestasis, ductopenia, and fibrous scarring, primary sclerosing cholangitis (PSC) also becomes a diagnostic consideration in this setting. Other noninfectious causes of epithelioid granulomas in the liver include drug reactions, foreign body reactions (i.e., talc, suture, etc.…”

Section: Histopathologymentioning

confidence: 99%

A Comprehensive Review of Hepatic Sarcoid

2018

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Sarcoidosis is a multisystem inflammatory disease, which is characterized by the development of noncaseating granulomas amid healthy tissue. While most commonly affecting the mediastinum and/or lungs, sarcoid can also affect the liver, resulting in "hepatic sarcoid." The spectrum of disease in hepatic sarcoid is variable, ranging from asymptomatic or mild liver enzyme abnormalities to end-stage liver disease requiring liver transplantation. Because clinically-significant hepatic sarcoid is rare, research on epidemiology, clinical manifestations, and treatment is limited. The mainstays of hepatic sarcoid treatment have involved steroids and more recently, ursodeoxycholic acid; however, novel immunomodulatory agents provide new possibilities for management. Questions remain regarding screening, diagnostic modalities, and what to treat with and when. The purpose of this review is to summarize the available research on the epidemiology, clinical course, and management of hepatic sarcoid, and identify gaps in our knowledge to motivate future research.

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Primary biliary cirrhosis and hepatic sarcoidosis: A case report

Abstract: In PBC patients it is important to consider coexisting granulomatous liver diseases if elevated liver function tests persist despite UDCA therapy.

Cited by 4 publications

References 20 publications

Sarcoidosis and primary biliary cholangitis in a patient with cholestasis

Sarcoidosis and primary biliary cholangitis in a patient with cholestasis

Primary biliary cirrhosis–autoimmune hepatitis overlap syndrome in a patient with paroxysmal nocturnal hemoglobinuria: a case report

A Comprehensive Review of Hepatic Sarcoid

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