Department of Rheumatology, Clinical Immunology, Geriatrics and Internal Medicine, Medical University of Gdansk, Gdańsk, Poland.
Department of Pulmonology and Allergology, Medical University of Gdansk, Gdańsk, Poland.
Rheumatol Int. 2024 Aug;44(8):1521-1528. doi: 10.1007/s00296-024-05631-3. Epub 2024 Jun 25.
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.
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.
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).
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.
抗中性粒细胞胞质抗体相关性血管炎（AAV）是一组系统性坏死性小血管自身免疫性疾病，其中显微镜下多血管炎（MPA）和肉芽肿性多血管炎（GPA）是最常见的两种。AAV 与不同的免疫介导性疾病（autoimmune disesases - AID）共存可能会影响主要疾病的临床表现。本研究旨在评估 AAV 与 AID 共存的情况，并探讨其是否会影响 AAV 的特征和病程。
进行了一项回顾性单中心研究，以确定诊断为 MPA 或 GPA 且同时患有 AID 的患者，并调查他们的临床特征和特征。该组由自 1988 年以来在一家大型大学附属医院接受治疗且随访至 2022 年的连续未经选择的 AAV 患者组成。
在 284 名诊断为 GPA（232 名）或 MPA（52 名）的患者中，有 40 名（14.1%）存在合并的 AIDs。最常见的是：桥本甲状腺炎（16 例）、类风湿关节炎（8 例），其次是银屑病（6 例）、恶性贫血（3 例）和脱发（3 例）。自身免疫合并症患者从症状出现到诊断的时间明显延长（26 个月 vs. 11 个月，p<0.001）。与无 AID 组相比，有自身免疫合并症的患者喉受累（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）更为常见。相反，有合并症的患者肾脏受累（45.0% vs. 70.9%，p=0.001）和结节性肺部病变（27.5% vs. 47.5%，p=0.044）明显较少。根据 EUVAS 标准，有自身免疫合并症的患者为无器官受累的全身性疾病（52.5% vs. 27.2%，p=0.007），而其他患者则为全身性疾病伴器官受累的比例较高（38.3% vs. 20.0%，p=0.007）。
AAV 与不同自身免疫性疾病共存并不常见，但可能会影响疾病的病程。多器官自身免疫病延长了诊断时间，但 AAV 病程似乎较轻。这些患者应特别注意癌症风险增加。似乎合理的是，AAV 患者应接受血清学筛查以排除重叠疾病的发展。
