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[复发性多软骨炎患者的急性肠系膜缺血]

[Acute mesenteric ischemia in a patient with relapsing polychondritis].

作者信息

Schultz O, Ruckert R, Burmester G, Buttgereit F

机构信息

Medizinische Klinik mit Schwerpunkt Rheumatologie und Klinische Immunologie, Universitätsklinikum Charité Berlin, Berlin.

出版信息

Z Rheumatol. 2006 Feb;65(1):63-8. doi: 10.1007/s00393-005-0001-8.

Abstract

We report about a 43-year old woman with relapsing polychondritis, admitted with progressive malaise, fatigue, anorexia and profound weight loss. Two years ago a nasal chondritis with characteristic changes of the nasal profil, scleritis, laryngitis and unspecific general symptoms (fever, fatigue, signs of a systemic inflammation) developed and relapsing polychondritis was diagnosed. The patient was treated initially with azathioprine followed by methotrexate in combination with ciclosporine and glucocorticoids. During the hospitalization her condition slowly worsened, and crampy abdominal pain developed subsequently with massive tenderness, rebound and guarding. The clinically presumed diagnosis of ileus and peritonitis was radiomorphologically confirmed with signs of enteric perforation and a laparatomy was performed. A complete occlusion of the A. mesenterica superior and stenosis of the truncus coeliacus were diagnosed followed by a revascularization with an aorto-mesenteric bypass and subsequent resection of the necrotic ischemic ileum. An association with various autoimmune disorders including vasculitic syndromes is well known for relapsing polychondritis. The case demonstrated a progressive mesenteric ischemia with the acute exacerbation caused by a vasculitic mesenterial occlusion. The unusual presentation should be considered in the differential diagnosis of uncommon abdominal symptoms during the course of relapsing polychondritis.

摘要

我们报告了一名43岁患有复发性多软骨炎的女性,她因进行性不适、疲劳、厌食和体重显著减轻入院。两年前,她出现了具有鼻部轮廓特征性改变的鼻软骨炎、巩膜炎、喉炎以及非特异性全身症状(发热、疲劳、全身炎症体征),并被诊断为复发性多软骨炎。患者最初接受硫唑嘌呤治疗,随后使用甲氨蝶呤联合环孢素和糖皮质激素治疗。住院期间,她的病情逐渐恶化,随后出现痉挛性腹痛,并伴有明显压痛、反跳痛和肌紧张。临床推测的肠梗阻和腹膜炎诊断经影像学检查证实存在肠穿孔迹象,并进行了剖腹手术。术中诊断为肠系膜上动脉完全闭塞和腹腔干狭窄,随后进行了主动脉 - 肠系膜旁路血管重建术,并切除了坏死缺血的回肠。复发性多软骨炎与包括血管炎综合征在内的各种自身免疫性疾病有关联。该病例显示出一种进行性肠系膜缺血,由血管炎性肠系膜闭塞导致急性加重。在复发性多软骨炎病程中出现不常见腹部症状的鉴别诊断时,应考虑这种不寻常的表现。

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