Departments of Medicine (M.B., J.K.) and Pathology (C.T.), Lahey Hitchcock Medical Center, Burlington, Massachusetts and Harvard Medical School (J.K.), Boston, Massachusetts.
J Clin Rheumatol. 1996 Aug;2(4):221-6. doi: 10.1097/00124743-199608000-00011.
We describe a 37-year-old man with Churg-Strauss syndrome (CSS) and a uniquely diffuse anatomic distribution of gastrointestinal (GI) involvement. Eosinophilic infiltration of the jejunum with granulomatous and necrotizing vasculitis resulted in perforation with peritonitis. Inflammation of the sigmoid colon associated with ulceration was documented in combination with radiographic evidence of a gastric ulcer.Endoscopic biopsy and abdominal angiography were not diagnostic and might be of limited sensitivity in documenting GI vasculitis in patients with CSS. Abdominal involvement in CSS can lead to life-threatening ischemia and perforation of the GI tract. In such cases, surgical intervention may be necessary; as in this case, the addition of cyclophosphamide to systemic corticosteroid therapy may speed resolution and decrease mortality in CSS.
我们描述了一例累及胃肠道(GI)的 Churg-Strauss 综合征(CSS)患者,其 GI 受累具有独特的弥漫性解剖分布。空肠的嗜酸性粒细胞浸润伴有肉芽肿性和坏死性血管炎导致穿孔合并腹膜炎。同时还记录到直肠炎症伴有溃疡,结合胃溃 疡的放射学证据。内镜活检和腹部血管造影术没有诊断价值,并且在诊断 CSS 患者的 GI 血管炎方面可能敏感性有限。CSS 的腹部受累可导致危及生命的胃肠道缺血和穿孔。在这种情况下,可能需要手术干预;就像本例中,在全身性皮质类固醇治疗中加入环磷酰胺可能会加速缓解并降低 CSS 的死亡率。