克罗恩病的多重并发症及早期持续多学科治疗的必要性

Journal of medical cases Pub Date : 2023-11-01 Epub Date: 2023-11-23 DOI:10.14740/jmc4154
David Dogahe, Maxime Taghavi, Edouard Cubilier, Said Sanoussi, Ruth Duttman, Joelle Nortier, Maria do Carmo Filomena Mesquita
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引用次数: 0

摘要

克罗恩病是一种炎症性疾病,通常影响肠道,但也可能有许多不同的肠外表现。其中一种并发症是免疫球蛋白A肾病(IgAN),这是克罗恩病最常见的肾脏病变之一。克罗恩病患者的另一个关注焦点是癌症的风险,结肠直肠癌的风险较高,但也有肠外肿瘤,如肝胆、血液和泌尿道肿瘤。我们提出的情况下,一个年轻的病人患有长期克罗恩病和随后的IgAN导致终末期肾脏疾病和血液透析。该患者被诊断为年轻,在不同的国家接受了多次手术和不同的治疗。然后他在我们中心出现了IgAN引起的晚期慢性肾功能衰竭,由于多学科随访不佳而未知。在开始血液透析后不久,他腹部出现了一个大肿块,最初被认为是由克罗恩病相关瘘管引起的。这个肿块原来是尿管腺癌,一种罕见的膀胱癌,预后特别差。目前尚不清楚这种类型的癌症是否与克罗恩病或IgAN有关,而且以前也没有描述过这种联系。尿管癌的治疗通常依赖于手术,在某些情况下会加上化疗。不幸的是,我们的病人,他的病例在诊断时已经很严重,他被排除在治愈治疗之外,此后很快去世了。这个病例说明了克罗恩病患者需要严格随访的许多重要方面,包括定期检查、筛查调查和多学科评估的需要。此外,它描述了克罗恩病和IgAN背景下一种罕见癌症的发展,这些不同的病理之间没有先前确定的联系。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Multiple Complications of Crohn's Disease and the Need for Early and Continuous Multidisciplinary Undertaking.

Crohn's disease is an inflammatory disease that typically affects the bowels but can also have many different extraintestinal manifestations. One of those complications is immunoglobulin A nephropathy (IgAN), which is one of the most encountered renal lesions in the setting of Crohn's disease. Another point of focus for Crohn's patients is the risk of cancer, with a higher risk of colorectal cancer but also extraintestinal neoplasia such as hepatobiliary, hematological, and urinary tract neoplasia. We present the case of a young patient suffering from long-term Crohn's disease and subsequent IgAN leading to end-stage kidney disease and hemodialysis. The patient was diagnosed young and had undergone multiple surgeries and different treatments in various countries. He then presented in our center already with advanced chronic renal failure from IgAN that was unknown due to poor multidisciplinary follow-up. Shortly after starting hemodialysis, he developed a large abdominal mass, first thought to result from Crohn's-related fistula. This mass turned out to be a urachal adenocarcinoma, a rare type of bladder cancer with an especially poor prognosis. It is not known whether this type of cancer is associated with either Crohn's disease or IgAN, and no such association has been previously described. The treatment of urachal cancer usually relies on surgery, with the addition of chemotherapy in some cases. Unfortunately for our patient, his case was already so advanced at the moment of diagnosis that he was excluded from curative treatment and quickly passed away thereafter. This case illustrates many important aspects of the rigorous follow-up that is needed for Crohn's patients, with regular check-ups, screening investigations, and the need for multidisciplinary evaluation. Furthermore, it describes the development of a rare type of cancer in the setting of Crohn's disease and IgAN, with no prior established link between these different pathologies.

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