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Abstract：
VEXAS (vacuoles, E1 enzyme, X-linked, auto-inflammatory, somatic) syndrome is a newly described auto-inflammatory disease. Many cases feature pulmonary infiltrates or respiratory failure. This systematic review aimed to summarize respiratory manifestations in VEXAS syndrome described to date.
Databases were searched for articles discussing VEXAS syndrome until May 2022. The research question was: What are the pulmonary manifestations in patients with VEXAS syndrome? The search was restricted to English language and those discussing clinical presentation of disease. Information on basic demographics, type and prevalence of pulmonary manifestations, co-existing disease associations and author conclusions on pulmonary involvement were extracted. The protocol was registered on the PROSPERO register of systematic reviews.
Initially, 219 articles were retrieved with 36 ultimately included (all case reports or series). A total of 269 patients with VEXAS were included, 98.6% male, mean age 66.8 years at disease onset. The most frequently described pulmonary manifestation was infiltrates (43.1%; n = 116), followed by pleural effusion (7.4%; n = 20) and idiopathic interstitial pneumonia (3.3%; n = 9). Other pulmonary manifestations were: nonspecific interstitial pneumonia (n = 1), bronchiolitis obliterans (n = 3), pulmonary vasculitis (n = 6), bronchiectasis (n = 1), alveolar haemorrhage (n = 1), pulmonary embolism (n = 4), bronchial stenosis (n = 1), and alveolitis (n = 1). Several patients had one or more co-existing autoimmune/inflammatory condition. It was not reported which patients had particular pulmonary manifestations.
This is the first systematic review undertaken in VEXAS patients. Our results demonstrate that pulmonary involvement is common in this patient group. It is unclear if respiratory manifestations are part of the primary disease or a co-existing condition. Larger epidemiological analyses will aid further characterisation of pulmonary involvement and disease management.
Databases were searched for articles discussing VEXAS syndrome until May 2022. The research question was: What are the pulmonary manifestations in patients with VEXAS syndrome? The search was restricted to English language and those discussing clinical presentation of disease. Information on basic demographics, type and prevalence of pulmonary manifestations, co-existing disease associations and author conclusions on pulmonary involvement were extracted. The protocol was registered on the PROSPERO register of systematic reviews.
Initially, 219 articles were retrieved with 36 ultimately included (all case reports or series). A total of 269 patients with VEXAS were included, 98.6% male, mean age 66.8 years at disease onset. The most frequently described pulmonary manifestation was infiltrates (43.1%; n = 116), followed by pleural effusion (7.4%; n = 20) and idiopathic interstitial pneumonia (3.3%; n = 9). Other pulmonary manifestations were: nonspecific interstitial pneumonia (n = 1), bronchiolitis obliterans (n = 3), pulmonary vasculitis (n = 6), bronchiectasis (n = 1), alveolar haemorrhage (n = 1), pulmonary embolism (n = 4), bronchial stenosis (n = 1), and alveolitis (n = 1). Several patients had one or more co-existing autoimmune/inflammatory condition. It was not reported which patients had particular pulmonary manifestations.
This is the first systematic review undertaken in VEXAS patients. Our results demonstrate that pulmonary involvement is common in this patient group. It is unclear if respiratory manifestations are part of the primary disease or a co-existing condition. Larger epidemiological analyses will aid further characterisation of pulmonary involvement and disease management.
摘要：
背景：VEXAS（空泡，E1酶，X-linked,自身炎症,躯体)综合征是一种新描述的自身炎症性疾病。许多病例以肺浸润或呼吸衰竭为特征。本系统综述旨在总结迄今描述的VEXAS综合征的呼吸道表现。
方法：在2022年5月之前搜索数据库中讨论VEXAS综合征的文章。研究问题是：VEXAS综合征患者的肺部表现是什么？搜索仅限于英语和讨论疾病临床表现的人。基本人口统计信息，肺部表现的类型和患病率，提取了共存的疾病关联和作者关于肺部受累的结论.该协议已在PROSPERO系统审查登记册上注册。
结果：最初，检索到219篇文章，最终纳入36篇（所有病例报告或系列）。共纳入269例VEXAS患者，98.6%男性,发病时平均年龄66.8岁。最常见的肺部表现是浸润（43.1％；n=116），其次是胸腔积液(7.4%;n=20)和特发性间质性肺炎(3.3%;n=9)。其他肺部表现为：非特异性间质性肺炎（n=1），闭塞性细支气管炎（n=3），肺血管炎（n=6），支气管扩张（n=1），肺泡出血（n=1），肺栓塞（n=4），支气管狭窄（n=1），和肺泡炎（n=1）。一些患者具有一种或多种共存的自身免疫/炎性疾病。没有报道哪些患者有特定的肺部表现。
结论：这是首次对VEXAS患者进行系统评价。我们的结果表明，肺部受累在该患者组中很常见。目前尚不清楚呼吸道表现是原发疾病还是共存疾病的一部分。更大的流行病学分析将有助于进一步表征肺部受累和疾病管理。
方法：在2022年5月之前搜索数据库中讨论VEXAS综合征的文章。研究问题是：VEXAS综合征患者的肺部表现是什么？搜索仅限于英语和讨论疾病临床表现的人。基本人口统计信息，肺部表现的类型和患病率，提取了共存的疾病关联和作者关于肺部受累的结论.该协议已在PROSPERO系统审查登记册上注册。
结果：最初，检索到219篇文章，最终纳入36篇（所有病例报告或系列）。共纳入269例VEXAS患者，98.6%男性,发病时平均年龄66.8岁。最常见的肺部表现是浸润（43.1％；n=116），其次是胸腔积液(7.4%;n=20)和特发性间质性肺炎(3.3%;n=9)。其他肺部表现为：非特异性间质性肺炎（n=1），闭塞性细支气管炎（n=3），肺血管炎（n=6），支气管扩张（n=1），肺泡出血（n=1），肺栓塞（n=4），支气管狭窄（n=1），和肺泡炎（n=1）。一些患者具有一种或多种共存的自身免疫/炎性疾病。没有报道哪些患者有特定的肺部表现。
结论：这是首次对VEXAS患者进行系统评价。我们的结果表明，肺部受累在该患者组中很常见。目前尚不清楚呼吸道表现是原发疾病还是共存疾病的一部分。更大的流行病学分析将有助于进一步表征肺部受累和疾病管理。
