Pulmonary manifestations of primary Sjogren's syndrome
Pulmonary manifestations of primary Sjogren's syndrome
复制标题
DOI：
10.1164/rccm.200403-417oc
复制
发表时间：
2005-03-15
影响因子：
24.7
通讯作者：
Mishima, M
中科院分区：
文献类型：
--
作者：
Ito, I;Nagai, S;Mishima, M
基本原理：自从认识到非特异性间质性肺炎（NSIP）是一种独特的组织学模式以来，与原发性干燥综合征相关的临床病理学肺部表现尚未进行大量的回顾。目的：目的：根据NSIP确定原发性干燥综合征相关肺部疾病的临床表现、高分辨率CT（HRCT）和组织学表现，并分析其预后。研究方法：基于从多个中心连续收集的33例病例（31例手术肺活检和2例尸检），我们回顾性评价了该疾病的临床、影像学和病理学表现。通过单因素和多因素分析确定预后因素。测量和主要结果：我们发现NSIP是最常见的组织学类型（33例中的20例[61%]，19例纤维化和1例细胞）。支气管疾病、淀粉样蛋白和恶性淋巴瘤较少见。HRCT-病理相关性导致CT-NSIP模式对NSIP病理诊断的阳性预测值为94%，而HRCT的诊断价值较低（15%），HRCT模式不同于NSIP，这些数据可能影响活检的决定。总体5年生存率为84%，NSIP患者为83%。对所有患者的多变量分析显示，低Pa-o2（P = 0.02）和显微镜下蜂窝样改变（P = 0.04）与生存率独立相关。患有NSIP的患者的肺活量（平均值+/- SD：68.5 +/- 16.6%pred）低于没有NSIP的患者（92.5 +/- 18.6%pred; p < 0.001）。结论：在原发性干燥综合征的多种肺部病变中，NSIP是最常见的组织学类型，预后良好。
Rationale: Clinicopathologic pulmonary manifestations associated with primary Sjogren's syndrome have yet to be reviewed in a large series since the recognition of nonspecific interstitial pneumonia (NSIP) as a distinct histologic pattern. Objectives: To determine clinical presentations, high-resolution computed tomographic (HRCT) and histologic findings of the lung disease associated with primary Sjogren's syndrome in the light of NSIP, and to analyze prognosis of the disease. Methods: On the basis of 33 cases (31 surgical lung biopsies and 2 autopsies) collected consecutively from multiple centers, we have retrospectively evaluated clinical, radiologic, and paithologic manifestations of the disease. Prognostic factors were identified by univariate and multivariate analysis. Measurements and main results: We found that NSIP was the most frequently seen histologic pattern (20 of 33 cases [61%], 19 fibrosing and 1 cellular). Bronchiolar diseases and amyloid and malignant lymphoma were seen less frequently. HRCT-pathologic correlation resulted in a 94% positive predictive value of CT-NSIP pattern for pathologic diagnosis of NSIP, whereas the diagnostic value of HRCT was low (15%) with an HRCT pattern other than NSIP, data that may influence the decision to biopsy. The 5-year survival rate was 84% overall and 83% in patients with NSIP. Multivariate analysis on all patients showed that low Pa-o2 (P = 0.02) and presence of microscopic honeycombing (p = 0.04) were independently associated with survival. Patients with NSIP showed lower vital capacity (mean +/- SD: 68.5 +/- 16.6%pred) than patients without NSIP (92.5 +/- 18.6%pred; p < 0.001). Conclusion: Among a diversity of pulmonary lesions in primary Sjogren's syndrome, NSIP was the commonest histologic pattern and had a favorable prognosis.