Department of Pulmonology, Ospedale GB Morgagni, Forlì, Italy.
Eur Respir J. 2011 Aug;38(2):384-91. doi: 10.1183/09031936.00094910. Epub 2011 Jan 27.
Recent evidence suggests that idiopathic nonspecific interstitial pneumonia (iNSIP) is a distinct clinical entity amongst other idiopathic interstitial pneumonias, and some data seem to suggest a possible pathogenetic role of autoimmunity. The aim of the present study was to assess if iNSIP might represent an early lung manifestation of an autoimmune disease. After initial review of cases found in the medical records database by searching for the term "NSIP" (n = 63), 37 iNSIP cases were identified, and were re-evaluated using a dynamic integrated multidisciplinary approach. 27 cases with iNSIP were selected for the study. Mean ± sd age at first respiratory symptom was 54.2 ± 8 yrs, 70% were females, and 59% were never-smokers. At follow-up (mean ± sd 59.7 ± 29 months, range 12-138 months), autoimmune diseases occurred in 14 (52%) patients, with seven (26%) cases of autoimmune thyroiditis, six (22%) of undifferentiated connective tissue disease and three (11%) of connective tissue disease. Patients developing autoimmune diseases were older and more frequently never-smoking females. In >50% of patients diagnosed with iNSIP, evidence of autoimmune diseases develops within 2 yrs, suggesting a probable link between the clinical entity of iNSIP and autoimmune disorders.
最近的证据表明，特发性非特异性间质性肺炎（iNSIP）是特发性间质性肺炎中的一种独特的临床实体，一些数据似乎表明自身免疫可能具有发病作用。本研究的目的是评估 iNSIP 是否可能代表自身免疫性疾病的早期肺部表现。在通过搜索术语“NSIP”（n=63）对病历数据库中的病例进行初步审查后，确定了 37 例 iNSIP 病例，并使用动态综合多学科方法对其进行重新评估。对 27 例 iNSIP 患者进行了研究。首次呼吸系统症状时的平均年龄±标准差为 54.2±8 岁，70%为女性，59%为从不吸烟者。在随访（平均±标准差 59.7±29 个月，范围 12-138 个月）时，14 例（52%）患者发生自身免疫性疾病，7 例（26%）为自身免疫性甲状腺炎，6 例（22%）为未分化结缔组织病，3 例（11%）为结缔组织病。发生自身免疫性疾病的患者年龄较大，且更频繁地从不吸烟。在 50%以上诊断为 iNSIP 的患者中，在 2 年内出现自身免疫性疾病的证据，提示 iNSIP 的临床实体与自身免疫性疾病之间可能存在关联。
