背景:抗中性粒细胞胞浆抗体相关血管炎(AAV)是一组全身性坏死性小血管自身免疫性疾病,显微镜下多血管炎(MPA)和肉芽肿性多血管炎(GPA)是最常见的两种。AAV与不同的免疫介导的疾病(自身免疫性疾病-AID)的共存可能会影响原发疾病的临床表现。该研究的目的是评估AAV与AID的共存并调查其是否影响AAV的特征和过程。
方法:进行了一项回顾性单中心研究,以确定诊断为MPA或GPA并伴有AID的患者。并探讨其临床特点和特点。该组由在一家大型大学医院接受治疗的连续未选择的AAV患者组成,自1988年起,随访至2022年。
结果:在诊断为GPA(232)或MPA(52)的284例患者中,40人(14,1%)患有共存的艾滋病。最常见的是:桥本甲状腺炎(16例),类风湿性关节炎(8例),其次是银屑病(6例),恶性贫血(3例),脱发(3例)。自身免疫性合并症患者在症状发作和诊断之间的时间明显更长(26vs.11个月,p<0.001)。喉部受累(20.0%vs.9.0%,p=0,05),周围神经系统疾病(35.0%vs.13.9%,p<0.001),和肿瘤(20.0%vs.8.6%,p=0.044)在AID合并症患者中更常见,与没有AID的受试者相比。相比之下,肾脏受累(45.0%vs.70.9%,p=0.001)和结节性肺部病变(27.5%vs.47.5%,p=0.044)在合并症患者中的频率明显较低。按照EUVAS标准,自身免疫性合并症患者具有无器官受累的疾病的广泛性形式(52.5%vs.27.2%,p=0.007),而其他人有器官受累的广泛性形式的比例更高(38.3%vs.20.0%,p=0.007)。
结论:AAV与不同的自身免疫性疾病共存并不常见,但它可能会影响疾病的临床进程。多重自身免疫延长了诊断时间,但是AAV课程似乎更温和。应特别注意这些患者患癌症的风险增加。AAV患者应该接受血清学筛查以排除重叠疾病的发展似乎也是合理的。
BACKGROUND: Antineutrophil cytoplasmic antibody-associated vasculitides (AAV) is a group of systemic necrotizing small vessel autoimmune diseases, with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) being the two most common. The co-existence of AAV with different immune-mediated diseases (autoimmune disesases - AID) might affect the clinical presentation of the primary disease. The purpose of the study was to assess the co-existence of AAV with AID and to investigate whether it affects the characteristics and the course of AAV.
METHODS: A retrospective single-center study was performed to identify patients with a diagnosis of MPA or GPA and concomitant AID, and to investigate their clinical features and characteristics. The group consisted of consecutive unselected AAV patients treated at a large university-based hospital, since 1988 with follow-up until 2022.
RESULTS: Among 284 patients diagnosed either with GPA (232) or MPA (52), 40 (14,1%) had co-existing AIDs. The most frequent were: Hashimoto thyroiditis (16 cases), rheumatoid arthritis (8 cases), followed by psoriasis (6 cases), pernicious anemia (3 cases), and alopecia (3 cases). Patients with autoimmune comorbidities had a significantly longer time between the onset of symptoms and the diagnosis (26 vs. 11 months, p < 0.001). Laryngeal involvement (20.0% vs. 9.0%, p = 0,05), peripheral nervous system disorders (35.0% vs. 13.9%, p < 0.001), and neoplasms (20.0% vs. 8.6%, p = 0,044) were more common in patients with AID comorbidities, compared to subjects without AID. In contrast, renal involvement (45.0% vs. 70.9%, p = 0.001) and nodular lung lesions (27.5% vs. 47.5%, p = 0.044) were significantly less frequent in patients with co-morbidities. Following EUVAS criteria, patients with autoimmune co-morbidities had a generalized form of the disease without organ involvement (52.5% vs. 27.2%, p = 0.007), while the others had a higher percentage of generalized form with organ involvement (38.3% vs. 20.0%, p = 0.007).
CONCLUSIONS: The coexistence of AAV with different autoimmune diseases is not common, but it might affect the clinical course of the disease. Polyautoimmunity prolonged the time to diagnosis, but the AAV course seemed to be milder. Particular attention should be paid to the increased risk of cancer in these patients. It also seems reasonable that AAV patients should receive a serological screening to exclude the development of overlapping diseases.