Del Rio Teresa, Burman Blaire, Siddique Asma, Forsythe Erin
Gastroenterology Department, Virginia Mason Medical Center, Seattle, Washington.
Gastro Hep Adv. 2025 Jun 24;4(10):100738. doi: 10.1016/j.gastha.2025.100738. eCollection 2025.
Refractory celiac disease is a rare complex autoimmune-mediated disorder, which has multiple complications, among the best known is enteropathy-associated T-cell lymphoma, other rare complications are central nervous system demyelinating disorder and cavitary mesenteric lymph node syndrome. In our case report, we present a 43-year-old female with a known history of refractory celiac disease who developed a progressive demyelinating disorder of unknown origin and elevated liver enzymes that warranted further investigation. This led to the diagnosis of the cavitary mesenteric lymph node syndrome and celiac disease associated with demyelinating disorder that are both very rare presentations of refractory celiac disease. Treatment options for this case presented a challenge, firstly due to the lack of literature but also to continued complications.
难治性乳糜泻是一种罕见的复杂自身免疫介导性疾病,有多种并发症,其中最广为人知的是肠病相关T细胞淋巴瘤,其他罕见并发症包括中枢神经系统脱髓鞘疾病和空泡性肠系膜淋巴结综合征。在我们的病例报告中,我们介绍了一名43岁女性,她有难治性乳糜泻病史,出现了病因不明的进行性脱髓鞘疾病以及肝酶升高,这需要进一步检查。这导致诊断为空泡性肠系膜淋巴结综合征以及与脱髓鞘疾病相关的乳糜泻,这两种都是难治性乳糜泻非常罕见的表现形式。该病例的治疗选择带来了挑战,首先是由于缺乏文献资料,也由于持续出现的并发症。