系统性硬皮病合并肺纤维化和肺气肿：一种与高发病率和死亡率相关的综合征。
Combined pulmonary fibrosis and emphysema in systemic sclerosis: A syndrome associated with heavy morbidity and mortality.
机构信息
Department of Internal Medicine, Service de Médecine Interne, Hôpital Cochin, Centre de Référence Maladies Systémiques Autoimmunes Rares d'Ile de France, DHU Authors (Autoimmune and Hormonal Diseases), Université Paris Descartes, Assistance Publique-Hôpitaux de Paris (AP-HP), 27, rue du Faubourg Saint-Jacques, 75679 Paris Cedex 14, France.
Service de Pneumologie, Centre National de Référence des maladies pulmonaire rares, Hospices Civils de Lyon, Hôpital Louis Pradel, Groupe d'Etudes et de Recherche sur les Maladies « Orphelines » Pulmonaires (GERM«O»P), Université Claude Bernard Lyon 1, UMR754, Lyon, France.
出版信息
Semin Arthritis Rheum. 2019 Aug;49(1):98-104. doi: 10.1016/j.semarthrit.2018.10.011. Epub 2018 Oct 13.
BACKGROUND
The syndrome of combined pulmonary fibrosis and emphysema (CPFE) primarily due to tobacco smoking has been reported in connective tissue disease, but little is known about its characteristics in systemic sclerosis (SSc).
METHODS
In this retrospective multi-center case-control study, we identified 36 SSc patients with CPFE, and compared them with 72 SSc controls with interstitial lung disease (ILD) without emphysema.
RESULTS
Rate of CPFE in SSc patients with CT scan was 3.6%, and 7.6% among SSc patients with ILD. CPFE-SSc patients were more likely to be male (75 % vs 18%, p < 0.0001), smokers (83 % vs 33%, p < 0.0001), and to have limited cutaneous SSc (53 % vs 24% p < 0.01) than ILD-SSc controls. No specific autoantibody was significantly associated with CPFE. At diagnosis, CPFE-SSc patients had a greater decrease in carbon monoxide diffusing capacity (DLCO 39 ± 13 % vs 51 ± 12% of predicted value, p < 0.0001) when compared to SSc-ILD controls, whereas lung volumes (total lung capacity and forced vital capacity) were similar. During follow-up, CPFE-SSc patients more frequently developed precapillary pulmonary hypertension (PH) (44 % vs 11%, p < 10), experienced more frequent unscheduled hospitalizations (50 % vs 25%, p < 0.01), and had decreased survival (p < 0.02 by Kaplan-Meier survival analysis) as compared to ILD-SSc controls.
CONCLUSIONS
The CPFE syndrome is a distinct pulmonary manifestation in SSc, with higher morbidity and mortality. Early diagnosis of CPFE by chest CT in SSc patients (especially smokers) may result in earlier smoking cessation, screening for PH, and appropriate management.
背景
联合性肺纤维化和肺气肿（CPFE）综合征主要由烟草引起，已在结缔组织疾病中报道，但在系统性硬化症（SSc）中其特征鲜为人知。
方法
在这项回顾性多中心病例对照研究中，我们确定了 36 例 SSc 合并 CPFE 患者，并将其与 72 例无肺气肿的 SSc 间质性肺病（ILD）对照进行比较。
结果
SSc 患者 CT 扫描的 CPFE 发生率为 3.6%，ILD 患者为 7.6%。CPFE-SSc 患者更可能为男性（75%比 18%，p<0.0001）、吸烟者（83%比 33%，p<0.0001）和局限性皮肤 SSc（53%比 24%，p<0.01），而非 ILD-SSc 对照。没有特定的自身抗体与 CPFE 显著相关。在诊断时，CPFE-SSc 患者与 SSc-ILD 对照相比，一氧化碳弥散量（DLCO）下降更明显（39±13%比预测值的 51±12%，p<0.0001），而肺容积（肺总量和用力肺活量）相似。在随访期间，CPFE-SSc 患者更频繁地发生毛细血管前肺动脉高压（PH）（44%比 11%，p<0.10），更频繁地非计划住院（50%比 25%，p<0.01），并且与ILD-SSc 对照相比生存率降低（p<0.02 由 Kaplan-Meier 生存分析）。
结论
CPFE 综合征是 SSc 的一种独特的肺部表现，发病率和死亡率更高。在 SSc 患者（尤其是吸烟者）中通过胸部 CT 早期诊断 CPFE 可能导致更早戒烟、筛查 PH 和适当的管理。
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