Abstract：
OBJECTIVE: To elucidate the clinical features, long-term survival, and prognostic factors for mortality among patients with microscopic polyangiitis (MPA), including those with anti-neutrophil cytoplasmic antibody-positive interstitial lung disease (ILD) (ANCA-ILD), which could be a subset of its variant phenotype.
METHODS: We retrospectively included 76 consecutive patients between 2006 and 2014, diagnosed with MPA according to the European Medicines Agency algorithm using the Chapel Hill Consensus Conference definitions or ANCA-ILD. ILD was classified as usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia pattern using chest computed tomography.
RESULTS: The mean (standard deviation) age of the patients (female, 68%) was 69 (12) years. The median (interquartile range) follow-up period was 68 (33-95) months. Comorbid ILD and glomerulonephritis were observed in 44 (58%) (68% UIP) and 54 (71%) patients, respectively. Comorbid ILD was associated with low survival (P = .0563). There were 17 (39%) and 5 (16%) deaths in the ILD and non-ILD groups, respectively (P = .0404). In the ILD group, 6 and 5 of the deaths were attributed to infection and ILD progression, respectively. In the non-ILD group, 1 and 2 patients expired from subsequently developed ILD and aspiration pneumonia, respectively. Age ≥ 70 years (hazard ratio = 2.78; 95% confidential interval 1.15-6.70) and UIP (3.95; 1.60-9.77) were independent risk factors for mortality.
CONCLUSIONS: Age ≥ 70 years and ILD with a UIP pattern were associated with high mortality, owing to susceptibility to infection and ILD progression. A more effective and less toxic treatment is required for progressive ILD.
METHODS: We retrospectively included 76 consecutive patients between 2006 and 2014, diagnosed with MPA according to the European Medicines Agency algorithm using the Chapel Hill Consensus Conference definitions or ANCA-ILD. ILD was classified as usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia pattern using chest computed tomography.
RESULTS: The mean (standard deviation) age of the patients (female, 68%) was 69 (12) years. The median (interquartile range) follow-up period was 68 (33-95) months. Comorbid ILD and glomerulonephritis were observed in 44 (58%) (68% UIP) and 54 (71%) patients, respectively. Comorbid ILD was associated with low survival (P = .0563). There were 17 (39%) and 5 (16%) deaths in the ILD and non-ILD groups, respectively (P = .0404). In the ILD group, 6 and 5 of the deaths were attributed to infection and ILD progression, respectively. In the non-ILD group, 1 and 2 patients expired from subsequently developed ILD and aspiration pneumonia, respectively. Age ≥ 70 years (hazard ratio = 2.78; 95% confidential interval 1.15-6.70) and UIP (3.95; 1.60-9.77) were independent risk factors for mortality.
CONCLUSIONS: Age ≥ 70 years and ILD with a UIP pattern were associated with high mortality, owing to susceptibility to infection and ILD progression. A more effective and less toxic treatment is required for progressive ILD.
摘要：
目的：为了阐明临床特征，长期生存,显微镜下多血管炎（MPA）患者死亡率的预后因素，包括抗中性粒细胞胞浆抗体阳性间质性肺病（ILD）（ANCA-ILD），这可能是其变异表型的一个子集。
方法：我们回顾性纳入了2006年至2014年期间的76例连续患者，根据欧洲药品管理局使用ChapelHill共识会议定义或ANCA-ILD的算法诊断为MPA。使用胸部计算机断层扫描将ILD分类为普通间质性肺炎（UIP）或非特异性间质性肺炎。
结果：患者的平均（标准差）年龄（女性，68%)为69(12)年。中位（四分位距）随访期为68（33-95）个月。在44例(58%)(68%UIP)和54例(71%)患者中观察到ILD和肾小球肾炎合并症,分别。合并症ILD与低生存率相关(P=.0563)。ILD和非ILD组中有17例（39%）和5例（16%）死亡，分别（P=.0404）。在ILD组中，6和5例死亡归因于感染和ILD进展，分别。在非ILD组中，1和2例患者因随后发展为ILD和吸入性肺炎而过期，分别。年龄≥70岁（风险比=2.78；95％保密间隔1.15-6.70）和UIP（3.95；1.60-9.77）是死亡的独立危险因素。
结论：年龄≥70岁和具有UIP模式的ILD与高死亡率相关，由于对感染和ILD进展的易感性。进行性ILD需要更有效且毒性更低的治疗。
方法：我们回顾性纳入了2006年至2014年期间的76例连续患者，根据欧洲药品管理局使用ChapelHill共识会议定义或ANCA-ILD的算法诊断为MPA。使用胸部计算机断层扫描将ILD分类为普通间质性肺炎（UIP）或非特异性间质性肺炎。
结果：患者的平均（标准差）年龄（女性，68%)为69(12)年。中位（四分位距）随访期为68（33-95）个月。在44例(58%)(68%UIP)和54例(71%)患者中观察到ILD和肾小球肾炎合并症,分别。合并症ILD与低生存率相关(P=.0563)。ILD和非ILD组中有17例（39%）和5例（16%）死亡，分别（P=.0404）。在ILD组中，6和5例死亡归因于感染和ILD进展，分别。在非ILD组中，1和2例患者因随后发展为ILD和吸入性肺炎而过期，分别。年龄≥70岁（风险比=2.78；95％保密间隔1.15-6.70）和UIP（3.95；1.60-9.77）是死亡的独立危险因素。
结论：年龄≥70岁和具有UIP模式的ILD与高死亡率相关，由于对感染和ILD进展的易感性。进行性ILD需要更有效且毒性更低的治疗。
