Department of Respiratory, The First Affiliated Hospital of Ningbo University, Ningbo, China.
Department of Rheumatology, The First Affiliated Hospital of Ningbo University, Ningbo, China.
Ann Med. 2023 Dec;55(1):2204449. doi: 10.1080/07853890.2023.2204449.
The association between pulmonary involvement and microscopic polyangiitis (MPA) has been increasingly recognized in recent years. Whether interstitial lung disease (ILD) and bronchiectasis (BE) are disease manifestations of MPA, preexisting comorbidities or important complications remains unclear. The purpose of this study was to determine the clinical characteristics and prognosis of MPA with pulmonary involvement to further guide clinical management.
The data for 97 patients with a definitive diagnosis of MPA were retrospectively reviewed. The MPA diagnosis was based on the 2012 revised Chapel Hill Consensus Conference (CHCC) criteria. The baseline clinical information and laboratory parameters were collected and analysed at each patient's initial diagnosis.
Forty-seven out of the 97 (48.5%) patients who were diagnosed with MPA presented with pulmonary involvement, including 37 patients with ILD, 12 patients with BE and two patients with diffuse alveolar haemorrhage (DAH). ILD and BE antedated MPA in 56.76% and 75.00% of the patients, respectively. Compared with that in the MPA-BE group, the serum LDH level (222.86 ± 68.19 vs. 171.58 ± 31.43, = .016) in the MPA-ILD group was significantly higher. In the multivariate Cox analysis, elevated serum creatinine (HR 4.08, confidence interval (CI) 1.38-12.05, = .011) was an independent risk factor for shorter survival in MPA patients with pulmonary involvement, and treatment with glucocorticoid pulse cyclophosphamide therapy (HR 0.095, 95% CI 0.019-0.47, = .004) was independently associated with prolonged survival. Among the patients in the MPA-ILD group, acute exacerbations of ILD (HR 4.55 CI 1.16-17.86, = .029) and elevated serum creatinine (HR 4.95, CI 1.39-17.54, = .014) were independently associated with a poor prognosis, and treatment with glucocorticoids (HR 0.057, 95% CI 0.012-0.28, < .001) was independently associated with significant prolongation of survival.
Patients with MPA have a high prevalence of pulmonary involvement, and ILD is the most common subtype of MPA. ILD and BE can be considered preexisting comorbidities of MPA. Elevated serum creatinine was associated with shorter survival. However, remission induction regimens with glucocorticoids and/or immunosuppressants may improve this outcome.
近年来，人们越来越认识到肺受累与显微镜下多血管炎（MPA）之间的关联。间质性肺病（ILD）和支气管扩张（BE）是 MPA 的疾病表现、预先存在的合并症还是重要的并发症尚不清楚。本研究的目的是确定有肺受累的 MPA 的临床特征和预后，以进一步指导临床管理。
回顾性分析了 97 例明确诊断为 MPA 的患者的数据。MPA 的诊断基于 2012 年修订的查佩尔山共识会议（CHCC）标准。在每位患者的初始诊断时收集并分析基线临床信息和实验室参数。
97 例 MPA 患者中有 47 例（48.5%）出现肺受累，包括 37 例ILD 患者、12 例 BE 患者和 2 例弥漫性肺泡出血（DAH）患者。ILD 和 BE 在分别有 56.76%和 75.00%的患者中先于 MPA。与 MPA-BE 组相比，MPA-ILD 组血清 LDH 水平（222.86±68.19 vs. 171.58±31.43，=0.016）明显更高。多变量 Cox 分析显示，血清肌酐升高（HR 4.08，95%CI 1.38-12.05，=0.011）是 MPA 患者肺受累生存时间较短的独立危险因素，而接受糖皮质激素脉冲环磷酰胺治疗（HR 0.095，95%CI 0.019-0.47，=0.004）与生存时间延长独立相关。在 MPA-ILD 组中，ILD 急性加重（HR 4.55，95%CI 1.16-17.86，=0.029）和血清肌酐升高（HR 4.95，95%CI 1.39-17.54，=0.014）与不良预后独立相关，而接受糖皮质激素治疗（HR 0.057，95%CI 0.012-0.28，<0.001）与生存时间显著延长独立相关。
MPA 患者肺受累的患病率较高，ILD 是 MPA 最常见的亚型。ILD 和 BE 可被视为 MPA 的预先存在的合并症。血清肌酐升高与生存时间缩短相关。然而，用糖皮质激素和/或免疫抑制剂诱导缓解的方案可能会改善这一结果。
