Lung Ultrasound in Children With Systemic Juvenile Idiopathic Arthritis–Associated Interstitial Lung Disease

Abstract

Objective. Systemic juvenile idiopathic arthritis (JIA) associated with lung disease (JIA-LD) is a potentially life threating complication in children with systemic JIA. Although high-resolution computed tomography (HRCT) is considered the gold standard imaging modality for evaluating interstitial lung disease (ILD), lung ultrasound (US) has shown utility for ILD screening in adults with connective tissue diseases at lower cost and without using ionizing radiation. The goals of this pilot study were to describe lung US features in children with known systemic JIA-LD and to assess the feasibility of lung US in this population.Methods. Children age <18 years with systemic JIA-LD and healthy controls were enrolled. Lung US acquisition was performed at 14 lung positions. Demographic, clinical, and HRCT data were collected and reviewed. Feasibility was assessed through patient surveys. Lung US findings were qualitatively and semiquantitatively assessed and compared to HRCT findings.Results. Lung US was performed in 9 children with systemic JIA-LD and 6 healthy controls and took 12 minutes on average to perform. Lung US findings in systemic JIA-LD included focal to diffuse pleural irregularity, granularity, and thickening, with associated scattered or coalesced B-lines, and subpleural consolidations. Lung US findings appeared to correspond to HRCT findings. Conclusion.Lung US in systemic JIA-LD reveals highly conspicuous abnormalities in the pleura and subpleura that appear to correlate with peripheral lung findings on HRCT. Lung US is a feasible imaging tool in children even from an early age. This study suggests a potential role of lung US in systemic JIA-LD screening, diagnosis, and/or prognostication.

New developments related to lung complications in pediatric rheumatic disease

“…One promising option is utilizing lung ultrasound, which was demonstrated in a small pilot study by Vega-Fernandez et al They compared the lung ultrasound findings of nine children with SJIA-LD with six healthy controls. At least in SJIA-LD, lung ultrasound appeared to correspond with HRCT findings and showed very distinct abnormalities in the pleura and subpleura [20 ▪ ]. It will be interesting to follow this research further and see if lung ultrasound is applicable to other pediatric rheumatic lung diseases as well, as has been shown in adult connective tissue disease [21].…”

Lung Involvement in Systemic Juvenile Idiopathic Arthritis: A Narrative Review

“…Recently, Vega Fernandez et al [ 51 ] evaluated the pulmonary ultrasonography findings in nine patients with sJIA-LD compared to healthy controls. Seven patients were also eligible for evaluation with HRCT.…”

“…HRCT is more sensitive at detecting changes in ILD than chest X-rays and represents the gold standard imaging for ILD. However, the significant cumulative radiation and costs associated with serial HRCT preclude its routine use for screening, follow-up and for monitoring responses to therapy [ 51 ]. Lung ultrasound (LUS) is a non-invasive, quick, ionizing radiation-free and low-cost imaging technique.…”

“…Traditionally used for pleural assessment or guided procedures, LUS has recently been proposed for the assessment of ILD in childhood rheumatic diseases. Vega Fernandez et al [ 51 ] found that 106/122 (87%) lung zones assessed using LUS were abnormal, often with severely abnormal subpleural pleural architecture. These findings often spared the axillary zones and affected anterior and posterior fields, as observed in HRCT.…”

“…Moreover, sJIA-LD may have been present since a patient was 2 years of age as children are unable to perform the recommended screening tests for sJIA-LD, including the 6-minute walk test, the lung function tests or an unsedated HRCT. These considerations, together with the costs and exposure to the ionizing radiation of HRCT, suggest a potential role of LUS as a screening tool for sJIA-LD [ 51 ].…”