Matas-Garcia Ana, Milisenda José C, Espinosa Gerard, Cuatrecasas Míriam, Selva-O'Callaghan Albert, Grau Josep María, Prieto-González Sergio
Muscle Research Unit, Department of Internal Medicine, Hospital Clínic de Barcelona, Universidad de Barcelona, Center for Biomedical Research on Rare Diseases (CIBERER), 08036 Barcelona, Spain.
Department of Autoimmune Diseases, Hospital Clínic de Barcelona, Universitat de Barcelona, 08036 Barcelona, Spain.
Diagnostics (Basel). 2022 May 11;12(5):1200. doi: 10.3390/diagnostics12051200.
Dermatomyositis is a systemic vasculopathy mainly affecting skin, muscle and lung, but may affect the gastrointestinal tract. We aim to describe clinical characteristics of patients with severe gastrointestinal involvement related to dermatomyositis in our center and medical literature. We retrospectively analysed these patients in our center, including cases of erosions/ulcers, perforation or digestive bleeding. Reported cases from April 1990 to April 2021 were reviewed through PubMed and Cochrane. From our cohort ( = 188), only 3 presented gastrointestinal compromise. All were women (10, 46 and 68 years). The initial symptom was abdominal pain and all had ≥2 episodes of digestive bleeding. All died due to complications of gastrointestinal involvement. Available pathological samples showed vascular ectasia. From the literature review ( = 50), 77% were women with a mean age of 49 years and the main symptom was abdominal pain (65%). All presented active muscular and cutaneous involvement at complication diagnosis. Mortality was 41.7%. The underlying lesion was perforation or ulcer ( = 22), intestinal wall thickening ( = 2), macroscopic inflammation ( = 2) or intestinal pneumatosis ( = 15). In 13 cases, vasculitis was described. Gastrointestinal involvement in dermatomyositis denotes severity, so an early intensive treatment is recommended. Pathological findings suggest that the underlying pathophysiological mechanism is a vasculopathy and not a true vasculitis.
皮肌炎是一种主要累及皮肤、肌肉和肺部的系统性血管病，但也可能累及胃肠道。我们旨在描述本中心及医学文献中与皮肌炎相关的严重胃肠道受累患者的临床特征。我们对本中心的这些患者进行了回顾性分析，包括糜烂/溃疡、穿孔或消化道出血的病例。通过PubMed和Cochrane对1990年4月至2021年4月报道的病例进行了回顾。在我们的队列（n = 188）中，只有3例出现胃肠道损害。所有患者均为女性（年龄分别为10岁、46岁和68岁）。初始症状为腹痛，且均有≥2次消化道出血发作。所有患者均因胃肠道受累并发症死亡。可用的病理样本显示血管扩张。从文献回顾（n = 50）来看，77%为女性，平均年龄49岁，主要症状为腹痛（65%）。所有患者在并发症诊断时均有活动性肌肉和皮肤受累。死亡率为41.7%。潜在病变为穿孔或溃疡（n = 22）、肠壁增厚（n = 2）、宏观炎症（n = 2）或肠壁积气（n = 15）。13例病例描述有血管炎。皮肌炎患者的胃肠道受累表明病情严重，因此建议早期强化治疗。病理结果提示潜在的病理生理机制是血管病而非真正的血管炎。
