Interstitial lung disease in primary Sjogren syndrome
Interstitial lung disease in primary Sjogren syndrome
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DOI：
10.1378/chest.130.5.1489
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发表时间：
2006-11-01
期刊：
影响因子：
9.6
通讯作者：
Ryu, Jay H.
中科院分区：
文献类型：
--
作者：
Parambil, Joseph G.;Myers, Jeffrey L.;Ryu, Jay H.
背景：原发性干燥综合征 (pSS) 与间质性肺疾病 (ILD) 的各种组织学模式相关。方法：我们回顾性鉴定了 1992 年至 2004 年 13 年间在我们机构接受肺活检（14 例手术活检和 9 例支气管镜活检）的患有 pSS 和疑似 ILD 的 IS 患者。 与放射学特征和结果相关。结果：中位年龄为 62 岁（范围为 34 至 78 岁），15 名患者 (83%) 为女性。大多数患者出现呼吸困难和咳嗽。胸部X光片显示双侧浸润，高分辨率CT显示各种类型的异常，包括磨玻璃样、实变样、网状样和结节样混浊。主要组织病理学模式包括非特异性间质性肺炎（NSIP）[5名患者]、机化性肺炎（OP）[4名患者]、普通间质性肺炎（UIP）[3名患者]、淋巴细胞性间质性肺炎（3名患者）、原发性肺淋巴瘤（2名患者）和弥漫性间质性淀粉样变性（1名患者） 病人）。在四名患者中（三名患有 OP，一名患有淀粉样变性），诊断是根据经支气管活检结果确定的。治疗通常包括泼尼松联合或不联合其他免疫抑制剂。在随访期间（中位38个月），除3名UIP患者、1名NSIP患者和1名淀粉样变性患者外，大多数患者病情改善或保持稳定。 7 例患者 (39%) 死亡，其中 3 例死于间质性肺炎急性加重。结论：pSS 相关 ILD 患者可出现多种组织学模式。患有 UIP 的人往往会出现肺部疾病的进展。 pSS 相关 ILD 患者可能会因间质性肺炎急性加重而死亡。
Background: Primary Sjogren syndrome (pSS) has been associated with various histologic patterns of interstitial lung disease (ILD).Methods: We retrospectively identified IS patients with pSS and suspected ILD who underwent lung biopsies (14 surgical biopsies and 9 bronchoscopic biopsies) at our institution during a 13-year period from 1992 through 2004. Histopathologic findings were analyzed and correlated with radiologic features and outcome.Results: Median age was 62 years (range, 34 to 78 years), and 15 patients (83%) were women. Most patients presented with dyspnea and cough. Chest radiographs demonstrated bilateral infiltrates, and high-resolution CT revealed abnormalities of various types including ground-glass, consolidation, reticular, and nodular opacities. The major histopathologic patterns included nonspecific interstitial pneumonia (NSIP) [five patients], organizing pneumonia (OP) [four patients], usual interstitial pneumonia (UIP) [three patients], lymphocytic interstitial pneumonia (three patients), primary pulmonary lymphoma (two patients), and diffuse interstitial amyloidosis (one patient). In four patients (three with OP and one with amyloidosis), the diagnosis was established on transbronchial biopsy results. Treatment commonly included prednisone with or without another immunosuppressive agent. During the follow-up period (median, 38 months), most patients improved or remained stable except three patients with UIP, one patient with NSIP, and one patient with amyloidosis. Seven patients (39%) died, including three deaths from acute exacerbation of interstitial pneumonia.Conclusions: A variety of histologic patterns can be seen in patients with pSS-associated ILD. Those with UIP tended to have progression of lung disease. Death from acute exacerbation of interstitial pneumonia may occur in patients with pSS-associated ILD.