Veiga C S, Coutinho D S, Nakaie C M A, Campos L M A, Suzuki L, Cunha M T, Leone C, Silva C A, Rodrigues J C
Pediatric Pulmonology Unit, Instituto da Criança do Hospital das Clínicas, University of São Paulo Medical School, São Paulo, Brazil
Pediatric Pulmonology Unit, Instituto da Criança do Hospital das Clínicas, University of São Paulo Medical School, São Paulo, Brazil.
Lupus. 2016 May;25(6):645-51. doi: 10.1177/0961203316629554. Epub 2016 Feb 4.
The aims of this study were to analyze the pulmonary function of childhood-onset systemic lupus erythematosus (cSLE) patients and to identify possible correlations between the high-resolution computed chest tomography (HRCT) score, disease activity, disease cumulative damage, and the participants' quality of life.
Forty cSLE patients, median age: 14.1 years (range: 7.4-17.9), underwent spirometry and plethysmography. Carbon monoxide diffusing capacity (DLCO), HRCT, disease activity, disease cumulative damage, and quality of life were assessed.
Pulmonary abnormalities were evident in 19/40 (47.5%) cSLE patients according to spirometry/DLCO. Forced expired volume in one second (FEV1%) was the parameter most affected (30%). The HRCT showed some abnormality in 22/30 patients (73%), which were minimal in 43%. Signs of airway affects were found in 50%. Twelve patients were hospitalized due to cSLE-related pulmonary complications before the study began (median discharge: 2.1 years earlier). Total lung capacity (TLC%), vital capacity (VC%), forced vital capacity (FVC%), and FEV1% were significantly lower in the group with hospitalization compared to the group without hospitalization (p = 0.0025, p = 0.0022, p = 0.0032, and p = 0.0004, respectively). Of note, DLCO was positively correlated with disease duration (r = +0.4; p = 0.01). The HRCT-score was negatively correlated with FEV1/VC (r = -0.63; p = 0.0002), FEV1 (r = -0.54; p = 0.018), FEF25%-75% (r = -0.67; p < 0.0001), and HRCT-score was positively correlated with resistance (r = +0.49; p = 0.0056).
Almost half of patients with cSLE had subclinical pulmonary abnormalities, especially airway abnormalities. The cSLE-related pulmonary complications seem to determine long-term functional damage.
本研究旨在分析儿童期起病的系统性红斑狼疮（cSLE）患者的肺功能，并确定高分辨率胸部计算机断层扫描（HRCT）评分、疾病活动度、疾病累积损伤与参与者生活质量之间的可能相关性。
40例cSLE患者，中位年龄：14.1岁（范围：7.4 - 17.9岁），接受了肺量计检查和体积描记法检查。评估了一氧化碳弥散量（DLCO）、HRCT、疾病活动度、疾病累积损伤和生活质量。
根据肺量计/DLCO检查，19/40（47.5%）的cSLE患者存在明显的肺部异常。一秒用力呼气容积（FEV1%）是受影响最大的参数（30%）。HRCT显示22/30例患者（73%）有一些异常，其中43%为轻微异常。50%的患者有气道受累迹象。在研究开始前，有12例患者因cSLE相关肺部并发症住院（中位出院时间：2.1年前）。与未住院组相比，住院组的肺总量（TLC%）、肺活量（VC%）、用力肺活量（FVC%）和FEV1%显著降低（分别为p = 0.0025、p = 0.0022、p = 0.0032和p = 0.0004）。值得注意的是，DLCO与疾病持续时间呈正相关（r = +0.4；p = 0.01）。HRCT评分与FEV1/VC（r = -0.63；p = 0.0002）、FEV1（r = -0.54；p = 0.018）、FEF25%-75%（r = -0.67；p < 0.0001）呈负相关，HRCT评分与阻力呈正相关（r = +0.49；p = 0.0056）。
几乎一半的cSLE患者存在亚临床肺部异常，尤其是气道异常。cSLE相关肺部并发症似乎决定了长期功能损害。
