Division of Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First St. SW, Rochester, MN, 55905, USA.
Dipartimento Cardio-Toraco-Vascolare, University of Milan-Bicocca, Respiratory Unit, San Gerardo Hospital, ASST di Monza; via Pergolesi 33, 20900, Monza, Italy.
Respir Res. 2017 Aug 23;18(1):161. doi: 10.1186/s12931-017-0644-4.
Autoimmune serologies are often obtained in the initial evaluation of uncharacterized interstitial lung disease (ILD). Whether this practice is helpful in delineating connective-tissue disease related ILD (CTD-ILD) is not well known. We assessed the frequency of incident CTD-ILD as detected by autoimmune serology testing and presenting clinical signs and symptoms.
Consecutive patients seen at our institution over a four year period with newly diagnosed uncharacterized ILD and autoimmune serologic testing were included. Serologic assessment was performed as a standardized order set of 13 laboratory tests. Presenting demographics and clinical signs or symptoms suggestive of autoimmune disease were correlated with the presence or absence of positive serology studies and final CTD-ILD diagnoses.
Overall prevalence of newly diagnosed CTD-ILD was 6.9% (42 of 605). Positive serology was seen in 35.2% (213 of 605) of screened ILD. CTD-ILD was diagnosed in 19.2% of those with positive serology, and 52.8% of those with both positive serology and suggestive clinical signs or symptoms. Only 1.4% of those with positive serology and negative review of systems were diagnosed with CTD-ILD. CTD-ILD diagnoses were made more frequently in younger patients ≤60 years with no diagnoses made after the age of 80 (P = 0.009). Positive serology in non-CTD-ILD cases did not appear to confer any survival advantage.
The yield of autoimmune serology testing in uncharacterized ILD appears greatest in those with suggestive clinical signs or symptoms on presentation for CTD-ILD.
在不明原因的间质性肺病（ILD）的初始评估中，常进行自身免疫血清学检查。但目前尚不清楚这种方法是否有助于明确结缔组织病相关的ILD（CTD-ILD）。本研究旨在评估通过自身免疫血清学检测发现的、有临床表现的 CTD-ILD 的发生率。
回顾性分析了我院在 4 年内诊断为新发不明原因的 ILD 并进行了自身免疫血清学检测的患者。血清学评估是按照标准化的 13 项实验室检测进行的。我们将患者的人口统计学特征、临床表现或疑似自身免疫性疾病的症状与自身免疫血清学检测结果及最终的 CTD-ILD 诊断结果进行了相关性分析。
新发 CTD-ILD 的总体患病率为 6.9%（42/605）。605 例 ILD 患者中，阳性血清学检查的检出率为 35.2%（213/605）。阳性血清学检查患者中，有 19.2%诊断为 CTD-ILD，而在同时具有阳性血清学检查和疑似临床表现或症状的患者中，这一比例为 52.8%。仅在 1.4%的阳性血清学检查且无系统回顾提示有自身免疫性疾病的患者中诊断为 CTD-ILD。在≤60 岁的年轻患者中，CTD-ILD 的诊断更为常见，而在 80 岁以上的患者中未发现 CTD-ILD（P=0.009）。在非 CTD-ILD 患者中，阳性血清学检查似乎并未带来生存优势。
在具有 CTD-ILD 临床表现的患者中，自身免疫血清学检测对不明原因的 ILD 具有最大的检出率。
