Prognostic factors in interstitial lung disease associated with primary Sjögren's syndrome: a retrospective analysis of 33 pathologically-proven cases.

Prognostic factors in interstitial lung disease associated with primary Sjögren's syndrome: a retrospective analysis of 33 pathologically-proven cases.
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DOI:
10.1371/journal.pone.0073774
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发表时间:
2013
期刊:
影响因子:
3.7
通讯作者:
Ogura T
Ogura T
中科院分区:
综合性期刊3区
文献类型:
--
作者:
Enomoto Y;Takemura T;Hagiwara E;Iwasawa T;Fukuda Y;Yanagawa N;Sakai F;Baba T;Nagaoka S;Ogura T

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与原发性干燥综合征相关的间质性肺病(pSS-ILD)表现出几种模式,如非特异性间质性肺炎(NSIP)和普通型间质性肺炎(UIP)。虽然UIP是特发性间质性肺炎的公认预后决定因素,但pSS-ILD是否也是如此尚不清楚。本研究的目的是评估UIP的预后作用,并确定pSS-ILD的预后因素。对病历进行回顾性审查,确定了33例经病理学证实的pSS-ILD连续患者。通过多学科分析将每例患者分类为每种ILD模式。比较ILD类型之间的基线临床-放射学-病理学特征和生存率。最后,使用考克斯比例风险回归模型通过单变量和随后的多变量分析评估pSS-ILD的预后因素。pSS-ILD患者诊断为NSIP(n = 22)或UIP(n = 11)。中位随访时间为110个月,总患者人群的5年生存率为87.3%。UIP患者的预后与NSIP患者的预后无显著差异(NSIP与UIP,风险比[HR]:0.77,95%置信区间[CI]:0.18-3.36,P = 0.73)。多变量分析确定了PaCO 2(HR:1.68/1 Torr增加,95% CI:1.24-2.28,P < 0.01),高分辨率CT显示的网状异常程度(HR:每1级增加4.17,95%CI:1.18-14.73,P = 0.03)和成纤维细胞灶的严重程度(HR:9.26/1级,95% CI:1.74-49.35,P < 0.01)作为pSS-ILD的预后因素。pSS-ILD中UIP与预后差于NSIP无关。评估详细的临床-放射学-病理结果比鉴别UIP更重要,以评估这种疾病的预后。
Interstitial lung disease associated with primary Sjögren’s syndrome (pSS–ILD) shows several patterns such as nonspecific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP). Although UIP is a well–recognized prognostic determinant in idiopathic interstitial pneumonias, whether this is also the case in pSS–ILD is unclear. The objectives of this study were to evaluate the prognostic effect of UIP, and to identify the prognostic factors in pSS–ILD. A retrospective review of medical records identified 33 consecutive patients with pathologically–proven pSS–ILD. Each patient was classified into each ILD pattern by multidisciplinary analysis. Baseline clinical–radiologic–pathologic characteristics and survival rates were compared between the ILD patterns. Finally, the prognostic factors in pSS–ILD were assessed by univariate and subsequent multivariate analyses using Cox’s proportional hazards regression model. pSS–ILD patients were diagnosed with NSIP (n = 22) or UIP (n = 11). The median follow–up period was 110 months, and five-year survival rate was 87.3% in the total patient population. The prognosis of the UIP patients was not significantly different from that of the NSIP patients (NSIP to UIP, hazard ratio [HR]: 0.77, 95% confidence interval [CI]: 0.18–3.36, P = 0.73). Multivariate analysis identified PaCO2 (HR: 1.68 per 1 Torr increase, 95% CI: 1.24–2.28, P < 0.01), extent of reticular abnormality on high–resolution CT (HR: 4.17 per 1-grade increment, 95% CI: 1.18–14.73, P = 0.03), and severity of fibroblastic foci (HR: 9.26 per 1-grade increment, 95% CI: 1.74–49.35, P < 0.01) as prognostic factors in pSS–ILD. UIP in pSS–ILD was not related to poorer prognosis than NSIP. Assessment of detailed clinical–radiologic–pathologic findings is more important than distinguishing UIP to evaluate prognosis in this disease.
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