Lung involvement in children with newly diagnosed rheumatic diseases: characteristics and associations

Abstract

Background Pulmonary complications of rheumatic diseases may cause functional impairment and increase mortality. However, reports regarding detection of lung involvement in children with treatment-naive, newly diagnosed rheumatic diseases are scarce. Herein, we aimed to describe the characteristics of such patients and explore the association between lung involvement and rheumatic disease. Methods From January 2019 to June 2021, 48 pediatric patients with treatment-naive, newly diagnosed rheumatic diseases at Department of Rheumatology and Immunology, Shanghai Children's Medical Center, School of Medicine, Shanghai Jiao Tong University were included with pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) findings, and 51 age-matched healthy controls were examined based on PFTs. Univariate and multivariable logistic regression analyses were used to investigate the clinical characters and laboratory parameters associated with lung involvement in these patients. Results Asymptomatic patients had a faster respiratory rate and a higher ratio of forced expiratory volume in 1 s/forced vital capacity than the controls (P < 0.05). More patients than controls were observed to have a decreased DLCO below the lower limit of normal (18 of 45 [40.0%] vs. 6 of 36, respectively; P = 0.041). Among the 48 patients, 8 (16.7%) had abnormal HRCT findings and 27 (56.3%) had abnormal PFT results. Thirty-one (64.6%) patients had lung involvement. Logistic regression revealed that increases in the erythrocyte sedimentation rate (ESR) and CD4/CD8 ratio were associated with increased odds ratio of lung involvement (1.037, 95% CI: 1.003–1.072; 9.875, 95% CI: 1.296–75.243, respectively). Conclusions Pediatric patients with treatment-naive, newly diagnosed rheumatic diseases are prone to pulmonary involvement. Increased ESR and CD4/CD8 are associated with elevated odds of lung involvement in patients. We recommend routine pulmonary evaluation in such patients, especially in high-risk patients, even in the absence of respiratory symptoms, once they are diagnosed with rheumatic disease.

New developments related to lung complications in pediatric rheumatic disease

“…In regard to pulmonary imaging at presentation, abnormal HRCT findings were observed in 16.7% of the patients, including ground glass opacities, nodules, pleural thickening, and linear opacities [4 ▪▪ ]. Diagnoses noted among these patients were limited to SLE, JDM, Sjogren's syndrome, and JIA.…”

“…Huang et al recently published a study highlighting the frequency of abnormal pulmonary findings in 48 treatment-naive patients with rheumatic disease at the time of diagnosis, compared with 51 age-appropriate healthy controls in Shanghai, China. The patients were evaluated by pulmonary function tests (PFTs) and high-resolution chest computed tomography (HRCT) imaging, while controls were only evaluated by PFTs [4 ▪▪ ]. Patients had a diagnosis of SLE, JDM, JIA, Sjogren's syndrome, SSc, or Behcet's disease.…”

Janus Kinase Inhibitors and Interstitial Lung Disease Associated With Pediatric Rheumatic Diseases: An Unexplored Field

“…In RA, the prevalence of ILD is approximately 10%, although rates as high as 67% have been reported depending on the method used to diagnose ILD (e.g., CT versus high-resolution CT (HRCT)) [23]. While RA-ILD appears to predominantly affect male patients [24], a study in pediatric patients showed that lung involvement in rheumatic disease is more strongly associated with female sex [4].…”

“…Nevertheless, these children are often clinically asymptomatic until the disease is significantly advanced [30]. Indeed, Huang et al [4] showed a relatively high prevalence of lung involvement in pediatric patients with newly diagnosed rheumatic diseases, the majority of whom had abnormal pulmonary function tests and HRCT findings, even though most were asymptomatic. The most common finding in these patients was a reduction in lung diffusion for carbon monoxide values, a prominent marker of ILD [31].…”

“…Although uncommon, pulmonary involvement may be observed in almost all pediatric rheumatic conditions [4]. In adult patients with various types of rheumatic diseases, interstitial lung disease (ILD) is the primary manifestation of lung involvement, causing progressive respiratory failure that significantly impairs the patient's quality of life and increases their morbidity and mortality [5,6].…”