Objectives: In the present study, we have sought to establish the clinical and immunological characteristics of Japanese patients with interstitial lung disease (ILD). Methods: Serum samples from 35 patients of ILD we...Objectives: In the present study, we have sought to establish the clinical and immunological characteristics of Japanese patients with interstitial lung disease (ILD). Methods: Serum samples from 35 patients of ILD were screened for autoantibodies using RNA and protein immunoprecipitation assays. Patients with or without serum antibodies to aminoacyl tRNA synthetases (ARS) were assessed clinically and compared. Results: Sera from 12 of 35 (34%) patients with ILD (mean age at onset = 49.7 yrs;range 27 - 65 yrs) were found to contain anti-ARS antibodies (anti-EJ: 3 patients;anti-OJ: 2 patients;anti-PL-12: 3 patients;anti-KS: 4 patients). Nine of the 12 (75%) were female. Six (50%) had Raynaud’s phenomenon, 5 (42%) had arthralgia/arthritis and four (33%) had rheumatoid factor. Lung biopsy specimens of 8 patients with anti-ARS antibodies were examined histologically in detail. The following was determined: Two patients had usual interstitial pneumonia;3 had non-specific interstitial pneumonia;one had organizing pneumonia;one had lymphocyte interstitial pneumonia and the remaining patient had desquamative interstitial pneumonia. Age at disease onset was significantly lower and the frequency of Raynaud’s phenomenon was significantly greater in these patients compared to anti-ARS-negative patients (49.7 yrs vs. 62.6 yrs, p = 0.004;50% vs. 4%, p = 0.003, respectively). Conclusions: These results indicate that the presence of anti-ARS autoantibodies correlates with ILD without definite diagnosis of connective tissue diseases as well as polymyositis/dermatomyositis (PM/DM) with ILD in Japanese patients.展开更多
目的探讨具有自身免疫特征的间质性肺炎(IPAF)的临床特点及其支气管肺泡灌洗液(BALF)细胞分类特征与应用。方法本研究为病例对照研究。采用目的抽样法选取2021年3月至2023年9月于首都医科大学附属北京安贞医院诊治的行支气管肺泡灌洗的I...目的探讨具有自身免疫特征的间质性肺炎(IPAF)的临床特点及其支气管肺泡灌洗液(BALF)细胞分类特征与应用。方法本研究为病例对照研究。采用目的抽样法选取2021年3月至2023年9月于首都医科大学附属北京安贞医院诊治的行支气管肺泡灌洗的IPAF患者56例,根据使用糖皮质激素前后的临床症状和胸部高分辨CT情况分为有效组(39例)和无效组(17例)。收集所有患者的一般情况(性别、年龄、吸烟、合并症),临床表现,辅助检查(血清学、影像特征、BALF分类、肺功能),以及糖皮质激素治疗反应情况,分析疾病临床特点。比较2组患者BALF分类淋巴细胞百分比,以及治疗前后用力肺活量占预计值百分比(FVC%pred)、肺总量占预计值百分比(TLC%pred)和肺一氧化碳弥散量占预计值百分比(DLCO%pred)水平。采用logistic回归分析影响IPAF患者糖皮质激素疗效的因素。结果共纳入IPAF患者56例,其中男22例,女34例;年龄(62.8±8.5)岁,年龄范围为43~78岁。所有患者均存在咳嗽,其中伴气短者51例(91.1%);咳痰35例(62.5%);技工手(即远端手指皮肤裂纹)8例(14.3%);手掌或指腹毛细血管扩张7例(12.5%);炎性关节炎或多关节晨僵>60 min 14例(25.0%);不明原因手指肿胀11例(19.6%);雷诺现象3例(5.4%)。所有IPAF患者血清学指标均为阳性,常见的血清学表现包括:抗核抗体阳性29例(51.8%),类风湿因子≥2倍正常上限3例(5.4%),抗环瓜氨酸肽抗体阳性1例(1.8%),抗SSA抗体阳性6例(10.7%)。IPAF患者胸部高分辨率CT主要表现为磨玻璃影(52/56,92.8%)、网格影(27/56,48.2%)、条索影(15/56,26.8%);影像分型最常见为非特异性间质性肺炎型(37/56,66.1%),其次为机化性肺炎型(9/56,16.1%)。BALF细胞分类以中性粒细胞和淋巴细胞混合升高为主(57.1%)。93.3%(14/15)技工手、手掌和指腹毛细血管扩张患者BALF淋巴细胞比例升高。磨玻璃影患者中86.5%(45/52)BALF淋巴细胞比例升高,94.2%(49/52)BALF中性粒细胞升高。有效组和无效组BALF分类淋巴细胞百分比分别为(26.4±13.8)%和(13.2±4.6)%,差异有统计学意义(t=3.84,P=0.009)。2组患者治疗前FVC%pred、TLC%pred和DLCO%pred比较差异均无统计学意义(均P>0.05);治疗后有效组以上各指标均高于无效组[(71.9±6.2)%比(65.4±6.2)%、(70.5±6.0)%比(64.0±6.1)%、(69.9±6.8)%比(65.1±8.4)%],差异均有统计学意义(均P<0.05)。较高的淋巴细胞比例会得到较好的糖皮质激素疗效(OR=0.407,95%CI:0.203~0.816,P=0.011)。结论IPAF以技工手、手掌或指腹毛细血管扩张较为常见,手部皮肤异常患者的BALF淋巴细胞比例较高;BALF细胞分类以淋巴细胞和中性粒细胞混合升高为主,较高的淋巴细胞比例会得到较好的糖皮质激素疗效。展开更多
文摘Objectives: In the present study, we have sought to establish the clinical and immunological characteristics of Japanese patients with interstitial lung disease (ILD). Methods: Serum samples from 35 patients of ILD were screened for autoantibodies using RNA and protein immunoprecipitation assays. Patients with or without serum antibodies to aminoacyl tRNA synthetases (ARS) were assessed clinically and compared. Results: Sera from 12 of 35 (34%) patients with ILD (mean age at onset = 49.7 yrs;range 27 - 65 yrs) were found to contain anti-ARS antibodies (anti-EJ: 3 patients;anti-OJ: 2 patients;anti-PL-12: 3 patients;anti-KS: 4 patients). Nine of the 12 (75%) were female. Six (50%) had Raynaud’s phenomenon, 5 (42%) had arthralgia/arthritis and four (33%) had rheumatoid factor. Lung biopsy specimens of 8 patients with anti-ARS antibodies were examined histologically in detail. The following was determined: Two patients had usual interstitial pneumonia;3 had non-specific interstitial pneumonia;one had organizing pneumonia;one had lymphocyte interstitial pneumonia and the remaining patient had desquamative interstitial pneumonia. Age at disease onset was significantly lower and the frequency of Raynaud’s phenomenon was significantly greater in these patients compared to anti-ARS-negative patients (49.7 yrs vs. 62.6 yrs, p = 0.004;50% vs. 4%, p = 0.003, respectively). Conclusions: These results indicate that the presence of anti-ARS autoantibodies correlates with ILD without definite diagnosis of connective tissue diseases as well as polymyositis/dermatomyositis (PM/DM) with ILD in Japanese patients.
文摘目的探讨具有自身免疫特征的间质性肺炎(IPAF)的临床特点及其支气管肺泡灌洗液(BALF)细胞分类特征与应用。方法本研究为病例对照研究。采用目的抽样法选取2021年3月至2023年9月于首都医科大学附属北京安贞医院诊治的行支气管肺泡灌洗的IPAF患者56例,根据使用糖皮质激素前后的临床症状和胸部高分辨CT情况分为有效组(39例)和无效组(17例)。收集所有患者的一般情况(性别、年龄、吸烟、合并症),临床表现,辅助检查(血清学、影像特征、BALF分类、肺功能),以及糖皮质激素治疗反应情况,分析疾病临床特点。比较2组患者BALF分类淋巴细胞百分比,以及治疗前后用力肺活量占预计值百分比(FVC%pred)、肺总量占预计值百分比(TLC%pred)和肺一氧化碳弥散量占预计值百分比(DLCO%pred)水平。采用logistic回归分析影响IPAF患者糖皮质激素疗效的因素。结果共纳入IPAF患者56例,其中男22例,女34例;年龄(62.8±8.5)岁,年龄范围为43~78岁。所有患者均存在咳嗽,其中伴气短者51例(91.1%);咳痰35例(62.5%);技工手(即远端手指皮肤裂纹)8例(14.3%);手掌或指腹毛细血管扩张7例(12.5%);炎性关节炎或多关节晨僵>60 min 14例(25.0%);不明原因手指肿胀11例(19.6%);雷诺现象3例(5.4%)。所有IPAF患者血清学指标均为阳性,常见的血清学表现包括:抗核抗体阳性29例(51.8%),类风湿因子≥2倍正常上限3例(5.4%),抗环瓜氨酸肽抗体阳性1例(1.8%),抗SSA抗体阳性6例(10.7%)。IPAF患者胸部高分辨率CT主要表现为磨玻璃影(52/56,92.8%)、网格影(27/56,48.2%)、条索影(15/56,26.8%);影像分型最常见为非特异性间质性肺炎型(37/56,66.1%),其次为机化性肺炎型(9/56,16.1%)。BALF细胞分类以中性粒细胞和淋巴细胞混合升高为主(57.1%)。93.3%(14/15)技工手、手掌和指腹毛细血管扩张患者BALF淋巴细胞比例升高。磨玻璃影患者中86.5%(45/52)BALF淋巴细胞比例升高,94.2%(49/52)BALF中性粒细胞升高。有效组和无效组BALF分类淋巴细胞百分比分别为(26.4±13.8)%和(13.2±4.6)%,差异有统计学意义(t=3.84,P=0.009)。2组患者治疗前FVC%pred、TLC%pred和DLCO%pred比较差异均无统计学意义(均P>0.05);治疗后有效组以上各指标均高于无效组[(71.9±6.2)%比(65.4±6.2)%、(70.5±6.0)%比(64.0±6.1)%、(69.9±6.8)%比(65.1±8.4)%],差异均有统计学意义(均P<0.05)。较高的淋巴细胞比例会得到较好的糖皮质激素疗效(OR=0.407,95%CI:0.203~0.816,P=0.011)。结论IPAF以技工手、手掌或指腹毛细血管扩张较为常见,手部皮肤异常患者的BALF淋巴细胞比例较高;BALF细胞分类以淋巴细胞和中性粒细胞混合升高为主,较高的淋巴细胞比例会得到较好的糖皮质激素疗效。
