Rym Ennaifer

Autoimmune Diseases in Coeliac Disease: Effect of Gluten Exposure

Introduction: the prevalence of autoimmune diseases is increased in patients with coeliac disease. Duration of gluten exposure seems to predispose adolescents with coeliac disease to autoimmune diseases. Aim: In a retrospective cohort study, we assessed the relationship between autoimmune disorders and actual gluten exposure in patients with coeliac disease. Patients and methods: the frequency of autoimmune disorders was evaluated in 64 patients (53 females, 11 males, mean age 29 years, range 16—63) with coeliac disease. The effect of age at the end of follow up, age at diagnosis of coeliac disease, actual gluten-exposure time, gender and diagnostic delay was assessed. Results: the prevalence of autoimmune diseases was 17%. Mean duration of gluten exposure was 26 and 25 years for patients with and without autoimmunity, respectively. Logistic regression showed that a longer mean follow up (P = 0.044) was related to the prevalence of autoimmune disorders while actual gluten exposure was not predictive. Conclusion: in this study, the prevalence of autoimmune diseases in patients with late coeliac disease diagnosis does not correlate with duration of gluten intake. Confirmatory prospective, multicentre studies of the effect of gluten-free diet are needed in adults.

Encéphalopathie hépatique minime : un diagnostic précoce pour améliorer le pronostic

BACKGROUND AND AIMS Minimal hepatic encephalopathy (MHE) is the mildest form of the spectrum of hepatic encephalopathy that impairs health-related quality of life. The aim of this study is to evaluate the prevalence of MHE in patients with liver cirrhosis and analyze risk factors. METHODS Between September 2011 and December 2012, consecutive cirrhotic patients seen in our department were evaluated. Patients included were screened by the psychometric hepatic encephalopathy score (PHES) battery comprising 5 tests: number connection test A and B, line tracing test, serial dotting test and digit symbol test. Patients included were regularly followed-up for the development of overt hepatic encephalopathy, driving accidents, falls and death. RESULTS We included 45 cirrhotic patients. Etiology of cirrhosis was viral in half of cases. Child-Pugh score was A in 55.6 %, B in 26.7 % and C in 17.8 %. Median Meld score was 14. According to the PHES score, MHE was detected in 44.4 % of patients. Univariate analysis identified 4 variables significantly associated with the presence of MHE: age ≥ 55 years (P=0.031), poor educational status with years of study< 9 years (P=0.007), MELD score ≥ 15 (P=0.002) and Child-Pugh ≥ 7 (P=0.001). At multivariate analysis, the only independent risk factor of MHE was a MELD score≥15 (OR=15.4; P<0.001). During the follow-up, patients with MHE developed more often overt encephalopathy, falls and driving accidents, and had a lower survival, although the difference was not statically significant. CONCLUSION In this preliminary small series, prevalence of MHE in Tunisian cirrhotic patient was 44.4 %. A MELD score ≥ 15 was the only independent risk factor. MHE had a negative impact on the outcome, justifying an early diagnostic. Adequate therapy may improve cognitive function.

Hepatic sarcoidosis: a case series

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.

Budd-Chiari Syndrome: An Unusual Presentation of Multisystemic Sarcoidosis

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.

Focal liver hyperplasia in a patient with Alagille syndrome: Diagnostic difficulties. A case report

Highlights • Hepatic 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.

Gastric schwannoma: a case report

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.

De novo autoimmune hepatitis following liver transplantation for primary biliary cirrhosis: an unusual cause of late grafts dysfunction.

De novo autoimmune hepatitis (AIH) is a rare disorder first described in 1998. It occurs in patients who underwent liver transplantation for a different etiology. We present the case of a 56-year-old woman who was diagnosed with primary biliary cirrhosis and had liver transplantation for refractory pruritis. Seven years after transplantation, she presented alterations in the hepatic profile with hypertransaminasemia, elevated alkaline phosphatase and gamma-glutamyl-transferase. Her liver functions test also showed elevated IgG levels. Serum autoantibodies were negative except for antimitochondrial antibodies. Histological findings indicated features of AIH without bile duct damage or loss. She had a pretreatment AIH score of 13 points and a post treatment score of 15 points according to the International AIH Group. The patient was treated effectively with prednisolone and her liver function and globulin levels rapidly returned to normal.

Downhill oesophageal variceal bleeding: A rare complication in Behçet’s disease-related superior vena cava syndrome

Behçets disease (BD) is a multisystemic disorder that involves vessels of all sizes. Superior vena cava (SVC) thrombosis is a rare complication that can lead to the development of various collateral pathways. A 31-year-old man presented with SVC syndrome. He had a history of recurrent genital aphthosis. Computed tomography revealed extensive thrombosis of the right internal jugular, axillary, and subclavian veins with collateral circulation. The patient was diagnosed with BD, and he was started on anticoagulation and immunosuppressive therapy. One week later, he presented with haematemesis. Upper gastrointestinal endoscopy disclosed varices in the upper third of the oesophagus with stigmata of recent bleeding. Portal hypertension was ruled out. Anticoagulation therapy was discontinued. He was discharged on immunosuppressive therapy. Bleeding from downhill oesophageal varices should be suspected in any patient presenting with upper gastrointestinal bleeding and a history of SVC syndrome due to BD.

Toxic megacolon complicating a first course of Crohn’s disease: about two cases

Toxic megacolon is a rare and serious complication of Crohn’s disease. Because of the associated high morbidity and mortality, early recognition and management of toxic megacolon is important. Through two cases of toxic megacolon complicating Crohn’s disease, we assessed the clinical, radiologic and therapeutic characteristics of this complication. A 35-year-old man presented a first course of Crohn’s disease treated with corticosteroid. He exhibited sudden severe abdominal pain and distension with shock. A plain abdominal radiography revealed toxic megacolon. He underwent medical therapy, but symptoms not relieved. The patient underwent subtotal colectomy with ileostomy. The resected specimen confirmed the diagnosis. Recovery of digestive continuity was performed. Endoscopic evaluation six months later did not shown recurrence. A 57-year-old man presented with severe acute colitis inaugurating Crohn’s disease, was treated with corticosteroid and antibiotics. He exhibited signs of general peritonitis. Computed tomographic examination revealed toxic megacolon with free perforation, showing prominent dilation of the transverse colon and linear pneumatosis. The patient underwent emergent subtotal colectomy and ileostomy. The final histological patterns were consisting with diagnosis of Crohn’s disease associated with cytomegalovirus infection. The patient underwent antiviral therapy during 15 days. Because of the high risk of postoperative recurrence, he underwent immunosuppressive therapy. Recovery of digestive continuity was performed successfully. Toxic megacolon in Crohn’s disease is a serious turning of this disease. We underscore the importance of early diagnosis of toxic megacolon and rapid surgical intervention if improvement is not observed on medical therapy.

Multiples ileal adenomas in Crohn's disease: Sporadic or ileitis associated dysplasia?

La Presse Medicale - In Press.Proof corrected by the author Available online since jeudi 29 decembre 2016