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特发性间质性肺炎伴自身免疫特征患者隐匿性原发性干燥综合征。

Occult primary Sjögren Syndrome in patients with interstitial pneumonia with autoimmune features.

机构信息

María Ferrer Hospital, Interstitial Lung Disease Unit, Buenos Aires, Argentina.

Pedro Lagleyze Hospital, Buenos Aires, Argentina.

出版信息

Respir Med. 2021 Jun;182:106405. doi: 10.1016/j.rmed.2021.106405. Epub 2021 Apr 20.

DOI:10.1016/j.rmed.2021.106405
PMID:33894442
Abstract

INTRODUCTION/OBJECTIVES: To define the performance of Minor Salivary Gland Biopsy (MSGB) and Dry Eye Tests (DET) to detect occult Sjögren Syndrome (SS) among Interstitial Pneumonia with Autoimmune Features (IPAF) patients.

METHODS

Prospective study. Interstitial Lung Disease (ILD) patients without defined Connective Tissue Disease and one or more IPAF classification domains or xerophthalmia were included. MSGB, Schirmer's test (ST) and Ocular Staining Score (OSS) were performed in a blinded manner by experienced specialists. MSGB with ≥1 focus of lymphocytes and Dry Eye Test (DET) with OSS ≥ 5 and/or ST < 5 s were considered positive. SS was diagnosed according to the ACR 2016 criteria.

RESULTS

534 patients on the first consult were screened. 67 patients had at least one IPAF criteria, 53 (79.1%) female, mean age (SD) 64.2 years old (10.8). Positive ST in 36 (53.7%), positive OSS in 29 (43.3%) and positive MSGB in 36 (53.7%) were found. Finally, 27 (40.3%) met SS diagnostic criteria. 25 (37.3%) and 18 (26.8%) of them did not report dry eyes or dry mouth, respectively. 53 (79.1%) had negative anti SSA/Ro, 57 (85.1%) had negative anti LA/SSB, 30 (44.7%) had negative ANA, and 52 (77.6%) had negative RF, respectively. A significantly higher proportion of ANA (+), anti-SSA/Ro (+), anti-SSB/La (+), positive DET and positive MSGB were found in the SS population.

CONCLUSIONS

A significant proportion of patients with occult SS were found in our study. MSGB and DET may be considered in the evaluation of IPAF patients.

摘要

介绍/目的:确定唾液腺活检(MSGB)和干眼测试(DET)在有自身免疫特征的间质性肺炎(IPAF)患者中检测隐匿性干燥综合征(SS)的性能。

方法

前瞻性研究。纳入无明确结缔组织病且具有一个或多个 IPAF 分类域或干眼症的间质性肺病(ILD)患者。由经验丰富的专家进行 MSGB、泪液分泌试验(ST)和眼染色评分(OSS)的盲法检查。MSGB 有≥1 个淋巴细胞灶,干眼试验(DET)有 OSS≥5 和/或 ST<5s 被认为是阳性。根据 ACR 2016 标准诊断 SS。

结果

在首次就诊时筛选了 534 名患者。67 名患者至少有一个 IPAF 标准,53 名(79.1%)女性,平均年龄(标准差)为 64.2 岁(10.8)。发现 36 例(53.7%)ST 阳性,29 例(43.3%)OSS 阳性,36 例(53.7%)MSGB 阳性。最终,27 例(40.3%)符合 SS 诊断标准。其中 25 例(37.3%)和 18 例(26.8%)分别报告无干眼或口干。53 例(79.1%)抗 SSA/Ro 阴性,57 例(85.1%)抗 LA/SSB 阴性,30 例(44.7%)抗核抗体(ANA)阴性,52 例(77.6%)类风湿因子(RF)阴性。SS 人群中发现 ANA(+)、抗 SSA/Ro(+)、抗 SSB/La(+)、DET 和 MSGB 阳性的比例显著较高。

结论

本研究发现隐匿性 SS 患者比例较高。MSGB 和 DET 可考虑用于评估 IPAF 患者。

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