Selig Tyler M, Siddique Ayesha, Reagan John L, Feller Edward R, Shah Samir A
Department of Internal Medicine, Warren Alpert Medical School of Brown University, Providence, RI, USA.
Eastern Connecticut Pathology Consultants, Manchester, CT, USA.
Case Rep Gastroenterol. 2025 Apr 2;19(1):246-252. doi: 10.1159/000543939. eCollection 2025 Jan-Dec.
Collagenous sprue (CS) is an extremely rare enteropathy of unknown etiology and if untreated, can lead to substantial morbidity and mortality. CS often copresents with celiac disease. The presence of a subepithelial collagen band on histology differentiates CS from celiac disease, as both have villous blunting. The management of the two diseases is different, and thus it is critical that the proper diagnosis of CS is made promptly to prevent complications.
We present a case report of CS in an elderly male who was initially diagnosed with celiac disease alone before returning to care years later with unresolved gastrointestinal symptoms.
Clinicians must verify that CS has been ruled out following a celiac disease diagnosis. In regard to CS's mechanism, CS's high frequency of comorbid autoimmune conditions and its robust response to corticosteroids support an immune-mediated process. Future research should continue to aim to elucidate the mechanism as it would allow for a more targeted approach to treatment, such as anti-fibrotic or specific immunomodulator therapy.
胶原性口炎性腹泻(CS)是一种病因不明的极为罕见的肠病,若不治疗,可导致严重的发病率和死亡率。CS常与乳糜泻同时出现。组织学上上皮下胶原带的存在将CS与乳糜泻区分开来,因为两者均有绒毛变钝。这两种疾病的治疗方法不同,因此及时准确诊断CS以预防并发症至关重要。
我们报告一例老年男性CS病例,该患者最初仅被诊断为乳糜泻,数年后因未解决的胃肠道症状再次就诊。
临床医生在诊断乳糜泻后必须确认已排除CS。关于CS的发病机制,CS合并自身免疫性疾病的高频率以及对皮质类固醇的强烈反应支持免疫介导过程。未来的研究应继续致力于阐明其机制,因为这将有助于采取更具针对性的治疗方法,如抗纤维化或特异性免疫调节剂治疗。