抗中性粒细胞胞浆抗体相关血管炎中的嗜中性皮肤病：一项法国多中心17例病例研究及文献综述
Neutrophilic Dermatoses in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A French Multicenter Study of 17 Cases and Literature Review.
作者信息
de Boysson Hubert, Martin Silva Nicolas, de Moreuil Claire, Néel Antoine, de Menthon Mathilde, Meyer Olivier, Launay David, Pagnoux Christian, Guillevin Loïc, Puéchal Xavier, Bienvenu Boris, Aouba Achille
机构信息
From the Department of Internal Medicine, Caen University Hospital, Caen (HDB, NMS, BB, AA); Department of Internal Medicine, Brest University Hospital (CDM); Department of Internal Medicine, Nantes University Hospital (AN); Department of Internal Medicine, Assistance Publique Hôpitaux de Paris, Saint-Louis Hospital, Paris, France (MDM); Department of Rheumatology, Assistance Publique Hôpitaux de Paris, Bichat University Hospital, Paris, France (OM); Department of Internal Medicine, Lille University Hospital, France (DL); Mount Sinai Hospital, University Health Network, and University of Toronto, Toronto, Ontario, Canada (CP); and Department of Internal Medicine, Assistancec Publique Hôpitaux de Paris, Cochin Hospital, Paris, France (LG, XP).
出版信息
Medicine (Baltimore). 2016 Mar;95(11):e2957. doi: 10.1097/MD.0000000000002957.
A few reports suggest combination of ANCA-associated vasculitis (AAV) and neutrophilic dermatoses (ND). We aimed to describe the main characteristics of patients presenting with both AAV and ND in a French cohort and through a systematic literature review, and to discuss the possible common pathogenic process involved. We conducted a retrospective study of patients with both conditions. Patients were selected via the French Internal Medicine Society (SNFMI) and the French Vasculitis Study Group (FVSG). A literature review focusing on a combination of both conditions, concentrated only on publications with well-established diagnoses and individual detailed data. Seventeen patients diagnosed with AAV and ND were identified in this cohort. Twelve patients had granulomatosis with polyangiitis (GPA), 4 had microscopic polyangiitis (MPA) and one had eosinophilic GPA (EGPA). Eight patients, all with GPA, displayed pyoderma gangrenosum (PG). Sweet's syndrome was observed in 6 patients (4 with MPA, one with GPA and one with EGPA) and erythema elevatum diutinum in the other three (2 with GPA and 1 with MPA). The literature review identified 33 additional patients with both conditions, including 26 with GPA. Altogether, of the 50 patients (17 from our study and 33 from the literature review), 33 (66%) patients presented with PG associated with GPA in 29 cases (89%). Corticosteroids were the first-line treatment in conjunction with an immunosuppressive agent in most cases. Outcomes were good and a total of 15 patients experienced a relapse. Patients who relapsed were more likely to have ear, nose and throat manifestation than patients who did not [12/15 (80%) relapsing patients vs. 15/35 (43%) non-relapsing patients; p = 0.03)]. In our stud, the most frequent association concerned GPA and PG. ND should be considered and specifically researched within the spectrum of cutaneous manifestations observed in AAV.
一些报告提示抗中性粒细胞胞浆抗体相关性血管炎（AAV）与嗜中性皮肤病（ND）存在合并情况。我们旨在描述法国队列中同时患有AAV和ND的患者的主要特征，并通过系统的文献综述进行分析，同时探讨可能涉及的共同致病过程。我们对患有这两种疾病的患者进行了一项回顾性研究。患者通过法国内科协会（SNFMI）和法国血管炎研究组（FVSG）进行筛选。一项聚焦于这两种疾病合并情况的文献综述，仅纳入了诊断明确且有个体详细数据的出版物。在该队列中，共识别出17例诊断为AAV和ND的患者。其中12例患有肉芽肿性多血管炎（GPA），4例患有显微镜下多血管炎（MPA），1例患有嗜酸性肉芽肿性多血管炎（EGPA）。8例患者（均为GPA）出现坏疽性脓皮病（PG）。6例患者出现Sweet综合征（4例MPA、1例GPA和1例EGPA），另外3例（2例GPA和1例MPA）出现持久性隆起性红斑。文献综述又识别出另外33例同时患有这两种疾病的患者，其中26例为GPA。总共50例患者（17例来自我们的研究，33例来自文献综述）中，33例（66%）患者出现与GPA相关的PG，共29例（89%）。大多数情况下，糖皮质激素联合免疫抑制剂作为一线治疗。预后良好，共有15例患者复发。复发患者比未复发患者更易出现耳鼻喉表现[复发患者12/15（80%） vs. 未复发患者15/35（43%）；p = 0.03]。在我们的研究中，最常见的关联是GPA和PG。在AAV观察到的皮肤表现范围内，应考虑并专门研究ND。
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