Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome
Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome
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DOI：
10.1164/rccm.2106012
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发表时间：
2002-06-15
影响因子：
24.7
通讯作者：
du Bois, RM
中科院分区：
文献类型：
--
作者：
Bouros, D;Wells, AU;du Bois, RM
纤维化肺泡炎合并系统性硬化症（FASSc）的预后优于特发性肺纤维化。鉴于最近有报道称特发性非特异性间质性肺炎（NSIP）比特发性普通间质性肺炎（UIP）预后更好，我们对80例FASSc患者的外科肺活检的组织学表现进行了分类。NSIP (n = 62, 77.5%)，亚分类为细胞性NSIP （n = 15）和纤维化性NSIP (n = 47)，比UIP （n = 6）、终末期肺病（ESL, n = 6）或其他模式（n = 6）更为普遍。死亡25例（NSIP 16/62, 26%; UIP/ESL 6/12, 50%）。NSIP和UIP/IESL的5年生存率差异不大（91%）；死亡率与较低的初始一氧化碳扩散能力（DLCO）和FVC水平相关（p = 0.004和p = 0.007）。细胞性和纤维化性NSIP的生存率和连续FVC和DLCO趋势没有差异。NSIP患者死亡率增加与较低的初始DLCO水平（p = 0.04）、较高的BAL嗜酸性粒细胞水平（p = 0.03）以及未来3年DLCO水平恶化相关（p < 0.005）。我们得出结论，NSIP是大多数FASSc患者的组织病理学模式。然而，结果与发病时的疾病严重程度和连续DLCO趋势的关系比与组织病理学结果的关系更强。
Fibrosing alveolitis associated with systemic sclerosis (FASSc) has a better prognosis than idiopathic pulmonary fibrosis. In view of recent reports that idiopathic nonspecific interstitial pneumonia (NSIP) has a better prognosis than idiopathic usual interstitial pneumonia (UIP), we classified histologic appearances of surgical lung biopsies performed in 80 patients with FASSc. NSIP (n = 62, 77.5%), subcategorized as cellular NSIP (n = 15) and fibrotic NSIP (n = 47) was much more prevalent than UIP (n = 6), end-stage lung disease (ESL, n = 6), or other patterns (n = 6). There were 25 deaths (NSIP 16/62, 26%; UIP/ESL 6/12, 50%). Five-year survival differed little between NSIP (91%) and UIP/IESL (82%); mortality was associated with lower initial carbon monoxide diffusing capacity (DLCO) and FVC levels (p = 0.004 and p = 0.007, respectively). Survival and serial FVC and DLCO trends did not differ between cellular and fibrotic NSIP. Increased mortality in NSIP was associated with lower initial DLCO levels (p = 0.04), higher BAL eosinophil levels (p = 0.03), and deterioration in DLCO levels during the next 3 years (p < 0.005). We conclude that NSIP is the histopathologic pattern in most patients with FASSc. However, outcome is linked more strongly to disease severity at presentation and serial DLCO trends than to histopathologic findings.