Department of Pathology, Veterans Affairs Palo Alto Health Care System, Palo Alto.
Department of Pathology, Stanford University School of Medicine, Stanford, CA.
Am J Surg Pathol. 2018 Jul;42(7):866-876. doi: 10.1097/PAS.0000000000001060.
Noninfectious gastrointestinal (GI) vasculopathic disorders are rare and are often overlooked in histopathologic examination or when forming differential diagnoses due to their rarity. However, involvement of the GI tract may lead to serious complications, including ischemia and perforation. Since awareness of the types of vasculopathy that may involve the GI tract is central to arriving at a correct diagnosis, we reviewed our institutional experience with GI tract vasculopathy in order to enhance diagnostic accuracy of these rare lesions. We report the clinical and histologic features of 16 cases (excluding 16 cases of immunoglobulin A vasculitis) diagnosed over a 20-year period. Of the 16 patients, 14 presented with symptoms related to the GI vasculopathy (including 2 presenting with a mass on endoscopic examination). The remaining 2 patients presented with incarcerated hernia and invasive adenocarcinoma. The vasculopathy was not associated with systemic disease and appeared limited to the GI tract in 8 patients. Eight had associated systemic disease, but only 6 had a prior diagnosis. The underlying diagnoses in these 6 patients included systemic lupus erythematosus (1), dermatomyositis (2), rheumatoid arthritis (1), eosinophilic granulomatosis with polyangiitis (1), and Crohn disease (1). One patient with granulomatous polyangiitis and 1 patient with systemic lupus erythematosus initially presented with GI symptoms. The 8 cases of isolated GI tract vasculopathy consisted of enterocolic lymphocytic phlebitis (4), idiopathic myointimal hyperplasia of the sigmoid colon (1), idiopathic myointimal hyperplasia of the ileum (1), granulomatous vasculitis (1), and polyarteritis nodosa-like arteritis (1). Isolated GI tract vasculopathy is rare, but appears to be almost as common as that associated with systemic disease. The chief primary vasculopathies are enterocolic lymphocytic colitis and idiopathic myointimal hyperplasia. Although the latter occurs predominantly in the left colon, rare examples occur in the small bowel and likely represent a complex, more protean disorder.
非传染性胃肠道（GI）血管病变较为罕见，由于其罕见性，在组织病理学检查或形成鉴别诊断时往往容易被忽视。然而，胃肠道受累可能导致严重并发症，包括缺血和穿孔。由于了解可能累及胃肠道的血管病变类型对于正确诊断至关重要，因此我们回顾了我们机构在胃肠道血管病变方面的经验，以提高这些罕见病变的诊断准确性。我们报告了在 20 年期间诊断的 16 例（不包括 16 例免疫球蛋白 A 血管炎）的临床和组织学特征。在 16 名患者中，有 14 名出现与胃肠道血管病变相关的症状（包括 2 名内镜检查时出现肿块）。其余 2 名患者表现为嵌顿疝和浸润性腺癌。血管病变与系统性疾病无关，在 8 名患者中局限于胃肠道。8 名患者伴有系统性疾病，但仅有 6 名患者有既往诊断。这 6 名患者的潜在诊断包括系统性红斑狼疮（1）、皮肌炎（2）、类风湿关节炎（1）、嗜酸性肉芽肿伴多血管炎（1）和克罗恩病（1）。1 例肉芽肿性多血管炎和 1 例系统性红斑狼疮患者最初表现为胃肠道症状。8 例孤立性胃肠道血管病变包括回肠结肠炎性淋巴细胞性静脉炎（4）、乙状结肠特发性内膜增生（1）、回肠特发性内膜增生（1）、肉芽肿性血管炎（1）和多动脉炎样动脉炎（1）。孤立性胃肠道血管病变较为罕见，但似乎与伴有系统性疾病的血管病变一样常见。主要的原发性血管病变是回肠结肠炎性淋巴细胞性结肠炎和特发性内膜增生。虽然后者主要发生在左结肠，但罕见的病例发生在小肠，可能代表一种复杂、更多变的疾病。
