Sarcoidosis is a systemic non caseous granulomas disease. Liver is a common location but usually asymptomatic. Evidence based guidelines for this location treatment is lacking and the effect of corticosteroids may be inadequate. The aim of our study was to describe the clinical, biochemical, radiological and therapeutic features of seven patients with systemic sarcoidosis and liver involvement. A retrospective and descriptive monocentric study, over 3 years, including seven patients with systemic sarcoidosis and liver involvement. We included 5 women and 2 men with an average age of 43 years. Hepatic localization revealed sarcoidosis in 5 cases. Hepatomegaly was observed in all patients as well as abnormal serum liver function test reflected by anicteric cholestasis. Liver biopsy, showed in all granulomatous lesions consistent with sarcoidosis and severe fibrosis in 2 cases. Extra-hepatic manifestations were present in all patients represented mainly by pulmonary location. All patients were treated, five by corticosteroid and two with ursodeoxycholic acid (UDCA). Complete response was observed in one case, partial response in another case and corticosteroid refractoriness in one case. In two cases, corticosteroid therapy was introduced for less than 1 month, not allowing assessment of response. Antimalarials in combination with UDCA were used successfully in a patient with steroid-resistant liver disease. Liver involvement can reveal systemic sarcoidois. Given the risk of progression to severe liver disease, it must be screened in all patients with systemic sarcoidosis. Treatment is not systematic, and still based on corticosteroid therapy. In the absence of prospective randomized controlled trials, the efficacy of UDCA need to be proven.
HighlightsHepatic lesions have been infrequently reported in Alagille syndrome.Most of them have been described as hepatocellular carcinoma.Focal liver hyperplasia can also be a cause of focal lesion.Magnetic resonance imaging features can reliably differentiate them.
Sarcoidosis is a multisystem granulomatous disease of unknown origin. All organs may be affected. Liver involvement is common but it is rarely symptomatic. Only a few cases of Budd-Chiari syndrome (BCS) secondary to a hepatic sarcoidosis have been described so far. We describe a case of multisystemic sarcoidosis presenting with BCS. A 42-year old female was referred to our department for chronic and anicteric cholestasis. Laboratory and imaging investigations disclosed features of chronic BCS associated with multisystemic sarcoidosis. The positive diagnosis was based on microscopic features, which showed hepatic, gastric and cutaneous non-caseating granulomas. Screening for an underlying thrombophilic disorder was negative. The diagnosis of BCS complicating hepatic sarcoidosis was the most likely. She was put on corticosteroids and anticoagulation therapy. To our knowledge, few cases of sarcoidosis-related BCS have been reported in the literature. In addition to being an uncommon presentation of sarcoidosis, this case illustrates the importance of recognizing an unusual cause of BCS and its therapeutic difficulties.
Schwannomas are generally benign, slow growing tumors. They are rarely observed in the gastrointestinal tract with the most common site being the stomach. These tumors are usually asymptomatic. The preoperative diagnosis via endoscopy is a challenging issue due to the difficulty of differentiation from other submucosal tumors. A 54-year-old woman presented with epigastric pain persisting for the last 10 months. Upper endoscopy revealed an elevated submucosal mass of the gastric antrum. The overlying mucosa was normal. Biopsy specimens yielded only unspecific signs of mild inactive chronic inflammation. Endoscopic ultrasound examination noted a hypoechoic homogeneous mass lesion located in the gastric antrum. The mass appeared to arise from the muscularis propria, and there was no perigastric lymphadenopathy. A contrast-enhanced computed tomography scan identified a homogeneous round mass and arising from the antrum of the stomach. Submucosal tumor was suspected and surgical intervention was recommended. The patient underwent an elective laparoscopic partial gastrectomy. The histopathologic features and immunohistochemical-staining pattern were consistent with a benign gastric schwannoma. Our patient shows no recurrence with a follow-up of one year. The definitive diagnosis of gastric schwannomas requires immunohistochemical studies. Complete margin negative surgical resection, as in this case, is the curative treatment of choice. The clinical course is generally benign.
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