Fischer Aryeh, Swigris Jeffrey J, du Bois Roland M, Groshong Steve D, Cool Carlyne D, Sahin Hakan, Lynch David A, Gillis JoAnn Z, Cohen Marc D, Meehan Richard T, Brown Kevin K
Autoimmune Lung Disease Center, National Jewish Medical and Research Center, Denver, CO; Department of Rheumatology, National Jewish Medical and Research Center, Denver, CO.
Autoimmune Lung Disease Center, National Jewish Medical and Research Center, Denver, CO.
Chest. 2009 Oct;136(4):1072-1078. doi: 10.1378/chest.08-2839. Epub 2009 May 8.
To describe a cohort of patients who presented with interstitial lung disease (ILD) of unknown cause, features of primary Sjögren syndrome (pSS), and a positive minor salivary gland biopsy (MSGB).
Thirty-eight patients with ILD evaluated at our center underwent an MSGB to confirm a diagnosis of pSS. All of the samples were reviewed by pathologists experienced in the evaluation of salivary gland histology. We defined a positive MSGB finding as a lymphocyte focus score of >1.
At presentation, all patients had ILD, and symptoms of cough and dyspnea. None had a definable connective tissue disease (CTD) or known cause for their ILD. Thirteen patients (34%) had positive MSGB findings. Of these, the median age was 61 years (age range, 33 to 75 years); 7 patients (54%) were women; 8 patients (62%) had a smoking history; and 10 patients (77%) had sicca symptoms. In all patients, a thoracic high-resolution CT scan evaluation demonstrated bibasilar, peripheral-predominant, ground-glass, and reticular opacities. Four patients (31%) were negative for both antinuclear autoantibody (ANA) and rheumatoid factor (RF) autoantibody, and three patients (23%) were negative for ANA, RF, Sjögren syndrome (SS)-A, and SS-B autoantibodies. No patients experienced any complications from the MSGB. The identification of underlying pSS did not affect the management of ILD in these patients.
Confirming a diagnosis of pSS-related ILD by performing MSGB allows for a more precise CTD classification. This study provides evidence that CTD may exist subclinically, and longitudinal studies are needed to determine whether identifying occult CTD impacts on management, longitudinal changes in lung function, or survival.
描述一组病因不明的间质性肺疾病（ILD）患者，其具有原发性干燥综合征（pSS）的特征且小唾液腺活检（MSGB）结果为阳性。
在我们中心接受评估的38例ILD患者接受了MSGB以确诊pSS。所有样本均由在唾液腺组织学评估方面经验丰富的病理学家进行复查。我们将MSGB结果阳性定义为淋巴细胞灶评分>1。
就诊时，所有患者均患有ILD，伴有咳嗽和呼吸困难症状。无一例有明确的结缔组织病（CTD）或已知的ILD病因。13例患者（34%）MSGB结果为阳性。其中，中位年龄为61岁（年龄范围33至75岁）；7例患者（54%）为女性；8例患者（62%）有吸烟史；10例患者（77%）有口干眼干症状。所有患者的胸部高分辨率CT扫描评估均显示双下肺、以周边为主的磨玻璃影和网状阴影。4例患者（31%）抗核自身抗体（ANA）和类风湿因子（RF）自身抗体均为阴性，3例患者（23%）ANA、RF、干燥综合征（SS）-A和SS-B自身抗体均为阴性。没有患者因MSGB出现任何并发症。潜在pSS的确诊并未影响这些患者ILD的治疗。
通过MSGB确诊pSS相关的ILD有助于更精确的CTD分类。本研究提供了证据表明CTD可能以亚临床形式存在，需要进行纵向研究以确定隐匿性CTD的识别是否会影响治疗、肺功能的纵向变化或生存率。
