Biswas Sugata N, Elhence Anshuman, Agrawal Vinita, Ghoshal Uday C
Department of Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, IND.
Department of Pathology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, IND.
Cureus. 2022 Jan 27;14(1):e21676. doi: 10.7759/cureus.21676. eCollection 2022 Jan.
Primary biliary cholangitis (PBC), a chronic, autoimmune, cholestatic disease, typically occurs in elderly women and commonly presents with pruritus, fatigue, and cholestasis and its complications. Gastric antral vascular ectasia (GAVE), an uncommon cause of upper gastrointestinal bleeding, leading to transfusion-dependent chronic iron deficiency anemia, as the first presentation of PBC is unusual. We present the case of an elderly female with recurrent melena and transfusion-dependent anemia for a year without any history of jaundice, ascites, or hepatic encephalopathy. Investigations revealed iron-deficiency anemia, elevated transaminases, alkaline phosphatase (ALP), coarse liver, splenomegaly, and portal vein dilatation on ultrasound. An endoscopic evaluation revealed erythematous linear stripes in the antrum suggestive of GAVE, without esophageal or gastric varices. FibroScan (Echosens, Paris, France) revealed advanced F3 fibrosis. Further etiological workup showed positive antinuclear and antimitochondrial antibodies, elevated IgM levels, and negative viral markers (hepatitis B, C, A, and E). Clinically significant portal hypertension was revealed by the hepatic venous pressure gradient (HVPG), while transjugular liver biopsy (TJLB) revealed lymphocytic infiltration of bile duct epithelium with the destruction of small and medium-sized bile ductules. Iron supplementation, low-dose ursodeoxycholic acid, and argon plasma coagulation were used to treat the patient. At the three-month follow-up, no melena was reported and her hemoglobin and liver function tests remained normal. Patients with PBC presenting with GAVE and recurrent melena as a presenting symptom are rarely reported. An awareness of this presentation is important for its early diagnosis and effective treatment.
原发性胆汁性胆管炎（PBC）是一种慢性自身免疫性胆汁淤积性疾病，通常发生于老年女性，常见症状为瘙痒、乏力、胆汁淤积及其并发症。胃窦血管扩张症（GAVE）是上消化道出血的少见原因，可导致依赖输血的慢性缺铁性贫血，而作为PBC的首发表现则较为罕见。我们报告一例老年女性病例，该患者反复出现黑便并依赖输血治疗贫血达一年，无黄疸、腹水或肝性脑病病史。检查发现缺铁性贫血、转氨酶升高、碱性磷酸酶（ALP）升高、肝脏粗糙、脾肿大以及超声显示门静脉扩张。内镜评估发现胃窦有红斑样线性条纹，提示GAVE，无食管或胃静脉曲张。FibroScan（法国巴黎Echosens公司）检查显示为F3期晚期纤维化。进一步的病因学检查显示抗核抗体和抗线粒体抗体阳性、IgM水平升高以及病毒标志物（乙肝、丙肝、甲肝和戊肝）阴性。肝静脉压力梯度（HVPG）显示存在临床显著的门静脉高压，而经颈静脉肝活检（TJLB）显示胆管上皮淋巴细胞浸润，中小胆管被破坏。给予患者补铁、低剂量熊去氧胆酸及氩离子凝固术治疗。在三个月的随访中，未再报告黑便情况，其血红蛋白和肝功能检查均保持正常。以GAVE和反复黑便为首发症状的PBC患者鲜有报道。认识到这种表现对于早期诊断和有效治疗很重要。
