Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA-Sanitas, Barcelona, Spain.
Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX, Barcelona, Spain.
Am J Gastroenterol. 2019 Aug;114(8):1238-1247. doi: 10.14309/ajg.0000000000000171.
Involvement of the gastrointestinal (GI) tract is an infrequent extrathoracic presentation of sarcoidosis. We reviewed 305 cases of GI involvement reported in 238 patients, in whom GI sarcoidosis was the first sign of the disease in half the cases. The disease does not affect the GI tract uniformly, with a clear oral-anal gradient (80% of reported cases involved the esophagus, stomach, and duodenum). Clinicopathological mechanisms of damage may include diffuse mucosal infiltration, endoluminal exophytic lesions, involvement of the myenteric plexus, and extrinsic compressions. Ten percent of patients presented with asymptomatic or subclinical disease found on endoscopy. The diagnosis is relevant clinically because 22% of cases reviewed presented as life threatening. In addition, initial clinical/endoscopic findings may be highly suggestive of GI cancer. The therapeutic approach is heterogeneous and included wait-and-see or symptomatic approaches, glucocorticoid/immunosuppressive therapy, and surgery. Sarcoidosis of the gut is a heterogeneous, potentially life-threatening condition that requires a multidisciplinary approach and early clinical suspicion to institute personalized therapeutic management and follow-up.
胃肠道(GI)受累是结节病少见的胸外表现。我们回顾了 238 例患者中的 305 例 GI 受累病例,其中半数患者的 GI 结节病是疾病的首发表现。该疾病不会均匀地影响胃肠道,存在明显的口腔-肛门梯度(报告的病例中有 80%累及食管、胃和十二指肠)。损害的临床病理机制可能包括弥漫性黏膜浸润、腔内外生性病变、肌间神经丛受累和外在压迫。10%的患者无症状或内镜下发现亚临床疾病。诊断在临床上很重要,因为我们回顾的病例中有 22%是有生命危险的。此外,初始临床/内镜表现可能高度提示 GI 癌。治疗方法具有异质性,包括观察等待或对症治疗、糖皮质激素/免疫抑制治疗和手术。肠道结节病是一种异质性的、潜在危及生命的疾病,需要多学科方法和早期临床怀疑来制定个性化的治疗管理和随访。