Presentation of Idiopathic Portal Hypertension in a Patient with Limited Systemic Sclerosis
نویسندگان
چکیده
......................................... Received: 06-12-13 Accepted: 21-07-14 Abstract Systemic sclerosis is a multisystem rheumatic autoimmune disorder characterized by fibrosis of the skin and many other organs. The gastrointestinal tract is the second cause of systemic compromise of this disease, but idiopathic portal hypertension is an extremely rare condition in these patients. We describe the case of a female patient with atypical hepatic changes of idiopathic portal hypertension associated with limited systemic sclerosis. Since these entities rarely coexist in the same individual, and since this is the first clinical association in our environment, we present this literature review and case report.
منابع مشابه
Idiopathic portal hypertension complicating systemic sclerosis: a case report
BACKGROUND Patients with systemic sclerosis may develop mild abnormalities of liver function tests. More serious hepatic involvement has been well documented but is rare. Idiopathic portal hypertension had been reported only in a few female patients with systemic sclerosis. CASE PRESENTATION An 82-year-old man with known systemic sclerosis presented with melaena. Urgent gastroscopy revealed o...
متن کاملSURGICAL EXPERIENCE WITH PORTAL HYPERTENSION DUE TO HEPATOPORTAL SCLEROSIS (NONCIRRHOTIC PORTAL FIBROSIS)
Hepatoportal sclerosis or idiopathic portal hypertension has a worldwide distribution with prevalence in developing and temperate countries. Of 64 patients with this disease seen during a twelve year period, 36 underwent splenectomy or a shunt procedure. The indications for surgical intervention were severe hypersplenism and persistent left upper abdominal pain andlorhistory of frequent ep...
متن کاملIsolated pulmonary veno-occlusive disease and pulmonary arterial thrombosis in systemic sclerosis – a lethal combination
BACKGROUND Isolated pulmonary hypertension secondary to systemic sclerosis is not uncommon. Our patient with systemic sclerosis presented with a very aggressive form of pulmonary hypertension due to a lethal combination of pulmonary veno-occlusive disease (PVOD) and pulmonary arterial thrombosis. This combined presentation has never before been reported in medical literature. CASE REPORT A 75...
متن کاملPOEMS syndrome and idiopathic portal hypertension: a possible association.
A 48-year old female patient was admitted to the emergency department with upper gastrointestinal bleeding. Endoscopy showed large esophageal varices that were treated with band ligation. She had been treated with cyclophosphamide, melphalan, lenalidomide and corticosteroids for POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy or edema, M protein, skin changes). She had no other ris...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره شماره
صفحات -
تاریخ انتشار 2015