Chartrand Sandra, Swigris Jeffrey J, Stanchev Lina, Lee Joyce S, Brown Kevin K, Fischer Aryeh
Department of Medicine, Hôpital Maisonneuve-Rosemont affiliated to Université de Montréal, Montréal, Québec, Canada.
Department of Medicine, National Jewish Health, Denver, CO, USA; Department of Medicine, University of Colorado School of Medicine, Aurora, CO, USA.
Respir Med. 2016 Oct;119:150-154. doi: 10.1016/j.rmed.2016.09.002. Epub 2016 Sep 3.
To describe the clinical phenotype and natural history of a cohort of patients with interstitial pneumonia with autoimmune features (IPAF).
A retrospective, single center study of 56 patients with IPAF evaluated between February 2008 and August 2014. All clinical data were extracted from the electronic medical record and longitudinal changes in forced vital capacity (FVC) were analyzed with mixed-effects, piecewise linear regression models that considered time as a continuous factor.
All patients fulfilled classification criteria for IPAF. The majority were women (71%) and never smokers (68%). The most frequently identified clinical features were Raynaud's phenomenon (39%), distal digital fissuring (29%), Gottron's sign (18%) and inflammatory arthropathy (16%). The most frequently identified serologies were antinuclear antibody (ANA) (48%), anti-Ro (SSA) (43%) and anti-tRNA-synthetase antibodies (36%). Nonspecific interstitial pneumonia (NSIP) (57.1%) followed by NSIP with organizing pneumonia (18%) were the most common radiologic patterns, while usual interstitial pneumonia was identified in only 9%. All but one patient was treated with immunosuppression: prednisone (82%) and mycophenolate mofetil (76%) were the most frequently used agents. During a follow-up period of 284.9 ± 141.3 days, modeled longitudinal FVC% was stable (slope = 0.69/year) and no deaths were observed in the cohort.
In this single center study, patients with IPAF were predominately non-smoking women with high-resolution computed tomography scans that suggested NSIP. Their pulmonary physiology was stable, and during limited follow-up, no deaths were observed. Prospective and multi-center studies are needed to better inform our understanding of IPAF.
描述一组具有自身免疫特征的间质性肺炎（IPAF）患者的临床表型和自然病史。
对2008年2月至2014年8月期间评估的56例IPAF患者进行回顾性单中心研究。所有临床数据均从电子病历中提取，并使用将时间视为连续因素的混合效应分段线性回归模型分析用力肺活量（FVC）的纵向变化。
所有患者均符合IPAF分类标准。大多数为女性（71%）且从不吸烟（68%）。最常发现的临床特征为雷诺现象（39%）、手指末端干裂（29%）、Gottron征（18%）和炎性关节病（16%）。最常发现的血清学检查结果为抗核抗体（ANA）（48%）、抗Ro（SSA）（43%）和抗tRNA合成酶抗体（36%）。非特异性间质性肺炎（NSIP）（57.1%）其次是伴机化性肺炎的NSIP（18%）是最常见的放射学模式，而普通间质性肺炎仅占9%。除1例患者外，所有患者均接受了免疫抑制治疗：泼尼松（82%）和霉酚酸酯（76%）是最常用的药物。在284.9±141.3天的随访期内，模拟的纵向FVC%稳定（斜率=0.69/年），且该队列中未观察到死亡病例。
在这项单中心研究中，IPAF患者主要为不吸烟女性，高分辨率计算机断层扫描显示为NSIP。他们的肺生理学稳定，在有限的随访期间未观察到死亡病例。需要进行前瞻性多中心研究以更好地了解IPAF。
