Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, USA.
Lupus. 2023 Apr;32(5):688-693. doi: 10.1177/09612033231163831. Epub 2023 Mar 13.
Pulmonary involvement in childhood-onset systemic lupus erythematosus (cSLE), contributes to significant morbidity and mortality. Manifestations include chronic interstitial pneumonitis, pneumonia, pleuritis, alveolar hemorrhage, and shrinking lung syndrome. However, many patients can be asymptomatic from a respiratory standpoint and still have pulmonary function test (PFT) abnormalities. Our aim is to describe PFT abnormalities in patients with cSLE.
We completed a retrospective review of 42 patients with cSLE followed at our center. These patients were at least 6 years old (so they could complete PFTs). We collected data from July 2015 to July 2020.
Out of the 42 patients, 10 (23.8%) had abnormal PFTs. These 10 patients had a mean age at diagnosis of 13 ± 2.9 years. Nine were female. One-fifth (20%) self-identified as Hispanic, 20% as Asian, 10% as Black or African American, and the remaining 50% as "Other." Of the 10, 3 had restrictive disease only, 3 with diffusion impairment only, and 4 with both restrictive lung disease and diffusion impairment. Patients with restrictive patterns had a mean total lung capacity (TLC) of 72.5 ± 5.8 throughout the study period. The average diffusing capacity for carbon monoxide corrected for hemoglobin (DsbHb) among patients with diffusion limitation during the study period was 64.8 ± 8.3.
The most common PFT abnormalities seen in patients with cSLE are alterations in diffusing capacity as well as restrictive lung disease.
儿童起病的系统性红斑狼疮（cSLE）肺部受累可导致显著的发病率和死亡率。表现包括慢性间质性肺炎、肺炎、胸膜炎、肺泡出血和缩肺综合征。然而，许多患者从呼吸角度来看可能无症状，但仍存在肺功能测试（PFT）异常。我们的目的是描述 cSLE 患者的 PFT 异常。
我们对在我们中心接受治疗的 42 例 cSLE 患者进行了回顾性研究。这些患者年龄至少为 6 岁（以便能够完成 PFT）。我们收集了 2015 年 7 月至 2020 年 7 月的数据。
在 42 例患者中，有 10 例（23.8%）PFT 异常。这 10 例患者的诊断年龄平均为 13 ± 2.9 岁。9 例为女性。五分之一（20%）患者自我认定为西班牙裔，20%为亚裔，10%为黑人和非裔美国人，其余 50%为“其他”。其中，3 例为单纯限制性疾病，3 例为单纯弥散障碍，4 例为限制性肺疾病和弥散障碍并存。具有限制性模式的患者在整个研究期间的平均总肺容量（TLC）为 72.5 ± 5.8。在研究期间存在弥散受限的患者中，血红蛋白校正后的一氧化碳弥散量（DsbHb）的平均值为 64.8 ± 8.3。
cSLE 患者最常见的 PFT 异常是弥散能力改变和限制性肺疾病。
