Fernandes Alexandra, Ferreira Ana Margarida, Ferreira Rosa, Mendes Sofia, Agostinho Cláudia, Almeida Nuno, Figueiredo Pedro, Ferreira Manuela, Amaro Pedro, Castro Lígia Prado E, Sofia Carlos
Gastroenterology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
Pathology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
GE Port J Gastroenterol. 2015 Sep 26;23(2):106-112. doi: 10.1016/j.jpge.2015.08.001. eCollection 2016 Mar-Apr.
Refractory celiac disease is an uncommon but serious complication of celiac disease. We describe a case of a severe refractory celiac disease type II, complicated with ulcerative jejunoileitis, in a 68 years old female, unresponsive to consecutive treatments with budesonide, prednisolone, cladribine and autologous stem cell transplantation. The patient maintained severe malnutrition, advanced osteoporosis, anaemia, vitamin deficiencies and hydro-electrolytic imbalances, necessitating consecutive hospitalizations for total parenteral nutrition. The patient also developed life-threatening complications, namely respiratory and urinary septic shock and also episodes of haemorrhagic shock secondary to ulcerative jejunoileitis. The progression to enteropathy associated T-cell lymphoma was never demonstrated, but the patient died 7 years after the diagnosis due to a septic shock secondary to a nosocomial pneumonia and osteomyelitis related to a spontaneous hip fracture. This case highlights the difficulties in the diagnostic process, therapeutic management and surveillance of this rare condition associated with very poor prognosis.
难治性乳糜泻是乳糜泻一种罕见但严重的并发症。我们描述了一例68岁女性的严重II型难治性乳糜泻病例，该病例并发溃疡性空肠回肠炎，对布地奈德、泼尼松龙、克拉屈滨和自体干细胞移植的连续治疗均无反应。患者持续存在严重营养不良、重度骨质疏松、贫血、维生素缺乏和水电解质失衡，需要连续住院接受全胃肠外营养。患者还出现了危及生命的并发症，即呼吸和泌尿感染性休克以及溃疡性空肠回肠炎继发的出血性休克发作。虽然从未证实进展为肠病相关T细胞淋巴瘤，但患者在诊断后7年因医院获得性肺炎继发的感染性休克以及与自发性髋部骨折相关的骨髓炎而死亡。该病例凸显了这种罕见疾病在诊断过程、治疗管理和监测方面的困难，其预后极差。
