Pereira Daniel Antunes Silva, Dias Olívia Meira, Almeida Guilherme Eler de, Araujo Mariana Sponholz, Kawano-Dourado Letícia Barbosa, Baldi Bruno Guedes, Kairalla Ronaldo Adib, Carvalho Carlos Roberto Ribeiro
University of São Paulo, School of Medicine, Hospital das Clínicas, São Paulo, Brazil. Department of Pulmonology, Instituto do Coração - InCor, Heart Institute - University of São Paulo School of Medicine Hospital das Clínicas, São Paulo, Brazil.
University of São Paulo, School of Medicine, Hospital das Clínicas, São Paulo, Brazil, Department of Cardiorespiratory Diseases, University of São Paulo School of Medicine, São Paulo, Brazil; and Director. Department of Pulmonology, Instituto do Coração - InCor, Heart Institute - University of São Paulo School of Medicine Hospital das Clínicas, São Paulo, Brazil.
J Bras Pneumol. 2015 Mar-Apr;41(2):151-60. doi: 10.1590/S1806-37132015000004443.
To describe the characteristics of a cohort of patients with lung-dominant connective tissue disease (LD-CTD).
This was a retrospective study of patients with interstitial lung disease (ILD), positive antinuclear antibody (ANA) results (≥ 1/320), with or without specific autoantibodies, and at least one clinical feature suggestive of connective tissue disease (CTD).
Of the 1,998 patients screened, 52 initially met the criteria for a diagnosis of LD-CTD: 37% were male; the mean age at diagnosis was 56 years; and the median follow-up period was 48 months. During follow-up, 8 patients met the criteria for a definitive diagnosis of a CTD. The remaining 44 patients comprised the LD-CTD group, in which the most prevalent extrathoracic features were arthralgia, gastroesophageal reflux disease, and Raynaud's phenomenon. The most prevalent autoantibodies in this group were ANA (89%) and anti-SSA (anti-Ro, 27%). The mean baseline and final FVC was 69.5% and 74.0% of the predicted values, respectively (p > 0.05). Nonspecific interstitial pneumonia and usual interstitial pneumonia patterns were found in 45% and 9% of HRCT scans, respectively; 36% of the scans were unclassifiable. A similar prevalence was noted in histological samples. Diffuse esophageal dilatation was identified in 52% of HRCT scans. Nailfold capillaroscopy was performed in 22 patients; 17 showed a scleroderma pattern.
In our LD-CTD group, there was predominance of females and the patients showed mild spirometric abnormalities at diagnosis, with differing underlying ILD patterns that were mostly unclassifiable on HRCT and by histology. We found functional stability on follow-up. Esophageal dilatation on HRCT and scleroderma pattern on nailfold capillaroscopy were frequent findings and might come to serve as diagnostic criteria.
描述以肺部为主的结缔组织病（LD-CTD）患者队列的特征。
这是一项对间质性肺疾病（ILD）患者的回顾性研究，这些患者抗核抗体（ANA）结果呈阳性（≥1/320），有或无特异性自身抗体，且至少有一项提示结缔组织病（CTD）的临床特征。
在筛查的1998例患者中，52例最初符合LD-CTD诊断标准：37%为男性；诊断时的平均年龄为56岁；中位随访期为48个月。随访期间，8例患者符合CTD确诊标准。其余44例患者组成LD-CTD组，其中最常见的胸外特征为关节痛、胃食管反流病和雷诺现象。该组中最常见的自身抗体为ANA（89%）和抗SSA（抗Ro，27%）。平均基线和最终用力肺活量（FVC）分别为预测值的69.5%和74.0%（p>0.05）。高分辨率CT（HRCT）扫描分别有45%和9%显示非特异性间质性肺炎和普通间质性肺炎模式；36%的扫描无法分类。组织学样本中也观察到类似的患病率。52%的HRCT扫描发现弥漫性食管扩张。对22例患者进行了甲襞毛细血管镜检查；17例显示硬皮病模式。
在我们的LD-CTD组中，女性占多数，患者诊断时肺功能轻度异常，潜在的ILD模式不同，大多在HRCT和组织学上无法分类。随访中发现功能稳定。HRCT上的食管扩张和甲襞毛细血管镜检查的硬皮病模式是常见表现，可能可作为诊断标准。
