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结节性多动脉炎以腹水为首发表现

Ascites as the first manifestation of polyarteritis nodosa.

作者信息

Hervé Fabien, Héron François, Levesque Hervé, Marie Isabelle

机构信息

Department of Internal Medicine, Centre Hospitalier Universitaire de Rouen-Boisguillaume, Rouen, France.

出版信息

Scand J Gastroenterol. 2006 Apr;41(4):493-5. doi: 10.1080/00365520600575670.

Abstract

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that affects numerous organs, particularly the digestive tract (33-52% of cases). However, isolated ascites has rarely been described in patients with PAN. We observed a patient who had developed ascites revealing PAN, and because of suspected malignancy laparotomy and abdominal surgery were performed, including systematic total abdominal hysterectomy, bilateral salpingo-oophrectomy, submesocolic omontectomy and appendicectomy. Macroscopic examination revealed a nodule involving the left ovary, while histological examination of biopsy specimens demonstrated characteristic damage of necrotizing vasculitis involving the appendix, omentum, uterus, fallopian tubes and right ovary, consistent with PAN. The patient had a favourable outcome of clinical manifestations related to PAN after initiation of combined therapy of prednisone and cyclophosphamide. Our case report therefore reinforces the possibility of an unusual presentation of PAN. As ascites may precede other signs of PAN, we suggest that when unexplained ascites is noted an evaluation for misdiagnosed vasculitis should be carried out, including a search of the constitutional symptoms (e.g. fever, weight loss). Patients undergoing laparoscopy for peritoneal involvement of unknown origin should also have tissue specimens taken for pathologic studies, notably to exclude an underlying vasculitis.

摘要

结节性多动脉炎(PAN)是一种全身性坏死性血管炎,可累及多个器官,尤其是消化道(33%-52%的病例)。然而,PAN患者中孤立性腹水很少被描述。我们观察到一名出现腹水的患者,经检查发现患有PAN,由于怀疑为恶性肿瘤,进行了剖腹术和腹部手术,包括系统性全腹子宫切除术、双侧输卵管卵巢切除术、结肠系膜下大网膜切除术和阑尾切除术。宏观检查发现一个结节累及左侧卵巢,而活检标本的组织学检查显示阑尾、大网膜、子宫、输卵管和右侧卵巢存在坏死性血管炎的特征性损害,符合PAN。在开始泼尼松和环磷酰胺联合治疗后,患者与PAN相关的临床表现有良好转归。因此,我们的病例报告强化了PAN不寻常表现的可能性。由于腹水可能先于PAN的其他体征出现,我们建议当发现不明原因的腹水时,应进行误诊血管炎的评估,包括寻找全身症状(如发热、体重减轻)。因不明原因的腹膜受累而接受腹腔镜检查的患者也应取组织标本进行病理研究,特别是要排除潜在的血管炎。

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