乾燥症相關之尋常性間質性肺炎與其他間質性肺病之比較

Comparison of usual interstitial pneumonia and other interstitial lung disease in patients with primary Sjögren’s syndrome-A single tertiary center observational study in Taiwan

目的：探討乾燥症病患合併有尋常性間質性肺病的臨床特徵、治療、及預後，並與其他乾燥症相關間質性肺病做比較。方法：本研究為病歷回顧分析，對象為西元2004年至2019年間、經胸部電腦斷層確診間質性肺病之原發性乾燥症患者。我們評估其肺部表現、乾燥症臨床及血清學檢查、治療選擇，最後使用統計方法比較尋常性間質性肺病及其他乾燥症相關間質性肺病之差異。結果：本總共有112個乾燥症病患經胸部電腦斷層發現合併有間質性肺病，大部分肺病診斷於乾燥症之前（中位數：1.2個月），其中35（31.3%）位病患的影像學型態為尋常性間質性肺病。相較於其他乾燥症相關間質性肺病，合併有尋常性間質性肺病的病患相對年老（72.0歲比66.7歲，p=0.017），且女性比例較少（51.4%比74%，p=0.01）；患有尋常性間質性肺病之病患之一年及十年存活率分別為85.7%和52.6%；經年齡、性別、用力呼氣肺活量、使用免疫抑制劑校正後，發現尋常性間質性肺病為不良預後因子（風險比率6.13，95%信賴區間1.43-25，p=0.014）。結論：相對其他種類乾燥症間質性肺病，乾燥症合併尋常性間質性肺病之病患普遍較年老，男性比例較多，且預後可能不良。

usual interstitial pneumonia (UIP) pattern and non-UIP patterns among patients with primary Sjögren’s syndrome (pSjS). Methods: Patients with pSjS and ILD between 2004-2019 were retrospectively identified. Demographic data, autoimmune features, ancillary examinations, therapies and treatment response of these patients were recorded. Descriptive and statistical analyses were used to compare groups with different radiologic pattern. Results: A total of 112 patients with pSS were reported having ILD. Most of these patients (81.2%) were recognized having pSS subsequent to the diagnosis of ILD with a median interval of 1.2 month from diagnosis date. Thirty-five (31.3%) patients had HRCT pattern of UIP and 77 (68.7%) patients had non- UIP pattern. Compared with non-UIP group, patients in UIP group were older (72.0 vs. 66.7, p=0.017). There were less female patients in UIP group than non-UIP (51.4% vs. 74.0%, p=0.018). The 1-year and 10-year survival rate in the UIP group were 85.7% and 52.6%, respectively. UIP group had worse prognosis compared with non-UIP group after adjustment by age of ILD diagnosis, FVC%, and use of immunosuppressant (hazard ratio: 6.13, 95% confidence interval 1.43-25, p=0.014). Conclusions: Comparing to non-UIP group, patients of primary Sjögren’s syndrome with UIP pattern were older and more males. The prognosis of UIP pattern seems poor among pSS-ILDs.