Abstract：
Immunoglobulin G4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition. A consistent feature of many cases is pulmonary infiltrates, or respiratory failure. This systematic literature review aims to summarise the pulmonary manifestations of IgG4-RD, including clinical outcomes and treatment. This review was registered on PROSPERO (CRD42023416410). Medline, Embase and Cochrane databases were searched for articles discussing IgG4-RD syndrome. Information was extracted on demographics, type and prevalence of pulmonary manifestations, treatment and clinical outcomes. Initially, after deduplication, 3123 articles were retrieved with 18 ultimately included. A pooled total of 724 patients with IgG4-RD were included, 68.6% male, mean age 59.4 years (SD 5.8) at disease onset. The most frequently described pulmonary manifestation was mediastinal lymphadenopathy (n = 186, 48.8%), followed by pulmonary nodules (n = 151, 39.6%) and broncho-vascular thickening (n = 85, 22.3%). Where treatment was reported, the majority of patients received glucocorticoids (n = 211, 93.4%). Other immunosuppressive therapy included cyclophosphamide (n = 31), azathioprine (n = 18), with mycophenolate mofetil (n = 6), rituximab (n = 6), methotrexate (n = 5) and other unspecified immunomodulators (50). Clinical outcomes were reported in 263 patients, where 196 patients had remission of their disease, 20 had relapse, 35 had stable disease, four had progression and eight patients died from complications of IgG4-RD. This systematic review summarises pulmonary manifestations, treatments and outcomes in patients with IgG4-RD. Pulmonary involvement in IgG4-RD is relatively common, leading to high levels of morbidity and mortality. Glucocorticoids remain the mainstay of treatment, but further work is required to explore the management of patients with pulmonary manifestations in association with IgG4-RD.
摘要：
免疫球蛋白G4相关疾病（IgG4-RD）是一种多系统纤维炎症性疾病。许多病例的一致特征是肺浸润，或呼吸衰竭。这篇系统的文献综述旨在总结IgG4-RD的肺部表现，包括临床结果和治疗。这篇评论在PROSPERO(CRD42023416410)上注册。Medline,检索Embase和Cochrane数据库中讨论IgG4-RD综合征的文章。信息是根据人口统计学提取的，肺部表现的类型和患病率，治疗和临床结果。最初，重复数据删除后，检索到3123篇文章，最终纳入18篇。共纳入724例IgG4-RD患者，68.6%男性,发病时平均年龄59.4岁（SD5.8）。最常见的肺部表现是纵隔淋巴结肿大（n=186，48.8%），其次是肺结节（n=151，39.6％）和支气管血管增厚（n=85，22.3％）。在报告治疗的地方，大多数患者接受了糖皮质激素(n=211,93.4%).其他免疫抑制治疗包括环磷酰胺（n=31），硫唑嘌呤（n=18），与霉酚酸酯（n=6），利妥昔单抗（n=6），甲氨蝶呤（n=5）和其他未指定的免疫调节剂（50）。263例患者报告了临床结果，196名患者病情缓解，20人复发了，35人病情稳定，4例患者进展,8例死于IgG4-RD并发症.这篇系统综述总结了肺部表现，IgG4-RD患者的治疗和结局。IgG4-RD的肺部受累相对常见，导致高发病率和死亡率。糖皮质激素仍是治疗的主要手段,但还需要进一步的工作来探索与IgG4-RD相关的肺部表现患者的治疗方法.
