Hepatic Sarcoidosis: Current Concepts and Treatments

Rossi, G.; Ziol, Marianne; Roulot, Dominique; Valeyre, Dominique; Mahévas, Matthieu

doi:10.1055/s-0040-1713799

Cited by 12 publications

(12 citation statements)

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“…Circumstances of Discovery and Diagnosis Liver sarcoidosis is more frequent in African-Americans than in Europeans, particularly in childhood, where it is seen in nearly half of cases at diagnosis [4,61]. Despite the fact that granulomas can be found in the liver in up to 50-70% of sarcoidosis patients, clinically significant hepatic sarcoidosis is observed in only 11.5-30% of them [24]. There are three main circumstances of discovery of liver sarcoidosis with different diagnostic questions.…”

Section: Liver and Spleen Sarcoidosismentioning

confidence: 99%

“…Another piece of evidence is a conspicuous response to corticosteroids after 4 weeks of treatment at the lung level [ 18 ], while a no-response within this period implies the re-consideration of the supposed link between the localization and sarcoidosis, justifying further investigation. For some localizations, an extra-biopsy may be required, most often for suspicion of renal [ 19 ], sinonasal [ 20 ], laryngeal [ 21 ], muscular [ 22 ], bone marrow [ 23 ], and liver (according to context) [ 24 ] sites. However, for some organs, like the central nervous system or the heart, a biopsy is rarely carried out for three main reasons.…”

Section: Diagnosis Of Extrapulmonary Organ Sarcoidosismentioning

confidence: 99%

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How to Tackle the Diagnosis and Treatment in the Diverse Scenarios of Extrapulmonary Sarcoidosis

et al. 2021

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Extrapulmonary sarcoidosis occurs in 30-50% of cases of sarcoidosis, most often in association with pulmonary involvement, and virtually any organ can be involved. Its incidence depends according to the organs considered, clinical phenotype, and history of sarcoidosis, but also on epidemiological factors like age, sex, geographic ancestry, and socio-professional factors. The presentation, symptomatology, organ dysfunction, severity, and lethal risk vary from and to patient even at the level of the same organ. The presentation may be specific or not, and its occurrence is at variable times in the history of sarcoidosis from initial to delayed. There are

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Section: Liver and Spleen Sarcoidosismentioning

confidence: 99%

Section: Diagnosis Of Extrapulmonary Organ Sarcoidosismentioning

confidence: 99%

How to Tackle the Diagnosis and Treatment in the Diverse Scenarios of Extrapulmonary Sarcoidosis

et al. 2021

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“…präsinusoidale und sinusoidale NCPH. Neben angeborenen Defekten, wie der polyzystischen Lebererkrankung [11] oder der kongenitalen hepatischen Fibrose [12], können intrahepatische Infiltrationen der Pfortader durch Lymphome oder chronische lymphatische Leukämie [13] sowie granulomatöse Erkrankungen, wie die Sarkoidose [14] und die Schistosomiasis, zur NCPH führen [15]. Zusätzlich können auch Speichererkrankungen, wie Amyloidose oder Morbus Gaucher, eine sinusoidale NCPH auslösen [16,17].…”

Section: Intrahepatische Ncphunclassified

Nichtzirrhotische portale Hypertension – Ursachen und praktisches Management

Queck

Trebicka

2021

Gastroenterologe

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ZusammenfassungUnter dem Krankheitsbild der nichtzirrhotischen portalen Hypertension (NCPH) wird eine heterogene Gruppe nichtzirrhotischer Pathogenesen portaler Hypertension zusammengefasst. Es wird anhand der korrespondierenden anatomischen Veränderungen zwischen einer prä-, intra- und posthepatischen Genese unterschieden. Gefäßveränderung proximal der Leber (z. B. Pfortaderthrombose) führen zu einer prähepatischen, distal der Leber gelegene (z. B. Budd-Chiari-Syndrom) zu einer posthepatischen NCPH. Intrahepatisch kann die NCPH durch angeborene Defekte (z. B. kongenitale hepatische Fibrose), granulomatöse Erkrankungen (Schistosomiasis, Sarkoidose), Malignome, Medikamente, das sinusoidale Obstruktionssyndrom, Speichererkrankungen (Amylodiose), idiopathisch und durch die portosinusoidale vaskuläre Erkrankung ausgelöst werden. Klinisch manifestiert sich die Erkrankung durch Komplikationen portaler Hypertension, jedoch häufig auch als Zufallsbefund (Varizen; leichtgradige Blutbildveränderungen; Transaminasen/γ-Glutamyltransferase-Erhöhung). Die Basisdiagnostik umfasst den laborchemischen und histologischen Ausschluss einer Lebergrunderkrankung und Zirrhose, die abdominelle Sonographie und die Gastroskopie. Aufgrund einer Assoziation zu hämato- und immunologischen Erkrankungen ist eine interdisziplinäre Abklärung indiziert. Neben der Therapie der Grunderkrankung ist beim Vorliegen einer Thrombose oder einer prothrombotischen Veranlagung eine Antikoagulation empfohlen. Komplikationen der portalen Hypertension werden ähnlich wie bei der zirrhotischen portalen Hypertension behandelt, jedoch findet die Shunttherapie als endgültige Therapie bei erhaltener Leberfunktion mehr Verwendung.

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“…It may be inaugural or complicate hepatic sarcoidosis history ( 12 ). Given the chronic nature of the disease, it is estimated that 3–28.6% of patients will progress to cirrhosis, as a result of parenchymal fibrosis secondary to granulomatous inflammation or vascular injury ( 10 , 13 ). Eventually, progression of the disease can lead toward liver failure requiring liver transplantation or death ( 14 ).…”

Section: Introductionmentioning

confidence: 99%

“…In view of the rarity of serious hepatic sarcoidosis cases, mainly due to complications of PH, there are only few descriptions of the therapeutic approach and prognosis of these patients, mostly in the form of case reports ( 13 ).…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2022

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IntroductionSarcoidosis 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.MethodsThis 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.ResultsTwelve 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.ConclusionSymptomatic 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.

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Hepatic Sarcoidosis: Current Concepts and Treatments

Cited by 12 publications

References 49 publications

How to Tackle the Diagnosis and Treatment in the Diverse Scenarios of Extrapulmonary Sarcoidosis

How to Tackle the Diagnosis and Treatment in the Diverse Scenarios of Extrapulmonary Sarcoidosis

Nichtzirrhotische portale Hypertension – Ursachen und praktisches Management

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

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