Magro Cynthia M, Crowson A Neil, Ferri Clodoveo
Department of Pathology, Weill College of Medicine, Cornell University, New York, NY 10021, USA.
Hum Pathol. 2007 Jan;38(1):42-9. doi: 10.1016/j.humpath.2006.06.002. Epub 2006 Nov 3.
Viruses have long been held to be of pathogenetic importance in the evolution of autoimmune connective tissue disease. We describe 7 adults who developed cutaneous connective tissue disease stigmata in temporal association with recent cytomegalovirus (CMV) infection but without the classic cytopathic changes of CMV infection. We examined 7 adults with clinical presentations encompassing cutaneous vasculitis in 4 and scleroderma in 3. In all 7 patients, there was either IgM seropositivity for CMV and/or CMV DNA isolation from peripheral blood. Although no CMV inclusions were seen, in situ hybridization studies revealed very focal CMV RNA transcript expression with localization mainly to the endothelium. The patients with vasculitis treated with ganciclovir had improvement or resolution of symptoms, whereas only 1 patient with scleroderma received antiviral therapy, without benefit. Another scleroderma patient responded to infliximab therapy. Abortive/partial CMV reactivation can be associated with a syndrome complex mimicking and/or triggering a primary immune-based cutaneous microvascular injury syndrome. Antiviral therapy appears to be of therapeutic value in those cases associated with active necrotizing vasculitic changes. The role of tumor necrosis factor alpha blockers in scleroderma cases temporally associated with CMV infection requires further evaluation.
长期以来，病毒在自身免疫性结缔组织病的演变中被认为具有致病重要性。我们描述了7名成年人，他们在近期巨细胞病毒（CMV）感染的同时出现了皮肤结缔组织病体征，但没有CMV感染的典型细胞病变改变。我们检查了7名成年人，临床表现包括4例皮肤血管炎和3例硬皮病。在所有7例患者中，要么CMV IgM血清学呈阳性，和/或从外周血中分离出CMV DNA。虽然未见CMV包涵体，但原位杂交研究显示CMV RNA转录本表达非常局限，主要定位于内皮细胞。接受更昔洛韦治疗的血管炎患者症状有所改善或缓解，而只有1例硬皮病患者接受了抗病毒治疗，但未见疗效。另1例硬皮病患者对英夫利昔单抗治疗有反应。CMV的顿挫型/部分再激活可能与一种综合征相关，该综合征模仿和/或引发原发性免疫性皮肤微血管损伤综合征。抗病毒治疗在那些与活动性坏死性血管炎改变相关的病例中似乎具有治疗价值。肿瘤坏死因子α阻滞剂在与CMV感染时间相关的硬皮病病例中的作用需要进一步评估。
