Clinical analysis of 25 cases of interstitial pneumonia with autoimmune features
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摘要: 目的 探讨具有自身免疫特征的间质性肺炎(IPAF)的临床特点、自然病程、危险因素及预后。 方法 回顾性分析2015年9月—2018年3月在宁波大学医学院附属医院诊治的特发性间质性肺炎(IIP)和未分化结缔组织疾病相关性间质性肺疾病(UCTD-ILD)患者,筛选出符合IPAF诊断标准的25例,分析患者的临床表现、血清学特征、形态学特征、肺功能及炎症标记物。 结果 25例IPAF患者中,男性9例,女性16例,发病年龄41~84岁,平均年龄(58.8±13.0)岁,有吸烟史6例。首发症状最常见为咳嗽(21/25)、咳痰(16/25)和气促(18/25)。肺外表现最常见为炎性关节炎/多关节晨僵(6/25),其次是雷诺现象(5/25)。血清学表现一半以上为抗核抗体ANA≥1∶320(13/25)。胸部高分辨CT(HRCT)以非特异性间质性肺炎(NSIP)最常见(10/25),其次为机化性肺炎(OP,5/25)。肺功能主要为弥散功能障碍(15/22)和(或)限制性通气功能障碍(16/22)。IPAF初诊时炎症标记物可出现明显升高,血沉升高(20/25),超敏C-反应蛋白异常(13/25),白细胞计数升高少见(6/25)。 结论 IPAF患者多见于非吸烟女性,高分辨CT以NSIP最常见。临床表现复杂多样,但缺乏特异性,有时与肺部感染难以鉴别。综合其肺外表现、血清学特点及影像学特点,可获得正确诊断。
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关键词:
- 特发性间质性肺炎 /
- 结缔组织疾病 /
- 具有自身免疫性的间质性肺炎
Abstract: Objective To analyze the clinical features, natural course, risk factors and prognosis of interstitial pneumonia with autoimmune features(IPAF). Methods The Clinical manifestations, serological features, morphological features, lung function and inflammatory markers of 25 patients with IPAF in the Affiliated Hospital of Medical School of Ningbo University from September 2015 to March 2018 were retrospectively investigated. Results There were 9 males and 16 females, aging 41 to 84 years(mean 58.8±13.0), and 6 patients had a history of smoking. The most common initial symptoms were cough(21/25), expectoration(16/25), and shortness of breath(18/25). The most prevalent systemic symptoms were inflammatory arthritis and polyarticular morning joint stiffness(6/25), followed by Raynaud`s phenomenon(5/25). More than half of the serological manifestations were ANA ≥ 1:320(13/25). Nonspecific interstitial pneumonia(NSIP, 10/25) was the most common chest high resolution CT(HRCT) pattern, followed by organic pneumonia(OP, 5/25). Pulmonary function was mainly diffuse dysfunction(15/22) and/or restrictive ventilatory dysfunction(16/22). In the initial diagnosis of IPAF, there was a significant increase in inflammatory markers, erythrocyte sedimentation(ESR, 20/25), hypersensitive C-reactive protein(13/25), and leukocyte count(6/25). Conclusion IPAF are more common in non-smoking women, and NSIP is the most common HRCT pattern. The clinical manifestations are complex and diverse, but lack of specificity, sometimes it is difficult to distinguish from pulmonary infection. The correct diagnosis should be based on extrapulmonary manifestations, serological and imaging features.
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