Developments In Lung Disease in Pediatric Rheumatic Disease

Abstract and Introduction

Abstract

Purpose of Review: While substantial progress has been made understanding lung disease in adult patients with rheumatic disease, pediatric lung disease has not been well addressed. Several recent studies provide new insights into diagnosis, management and treatment of lung disease in children with rheumatic disease.

Recent Findings: Building on previous research, newly diagnosed patients may have abnormalities in pulmonary function tests and chest computed tomography imaging even when asymptomatic. New guidelines for screening for rheumatic-associated lung disease provide important recommendations for clinicians. New theories have been proposed about immunologic shifts leading to the development of lung disease in children with systemic juvenile idiopathic arthritis. Additionally, there are new antifibrotic agents that are being explored as treatments in pediatric patients with fibrotic lung diseases.

Summary: Patients appear to have frequent lung function abnormalities while being clinically asymptomatic, emphasizing importance for rheumatologists to refer for pulmonary function tests and imaging at diagnosis. New advances are helping define optimal approaches to treatment of lung disease, including use of biologic agents and antifibrotic medicines for pediatric patients with rheumatologic diseases.

Introduction

Pulmonary complications are a major source of morbidity and mortality in pediatric and adult rheumatologic diseases.^[1] These pulmonary manifestations relate to the underlying disease process, progression of disease, and/or side effects of disease treatment.^[2] Although pulmonary injury theoretically results from an immune-mediated process in rheumatologic diseases, the absolute mechanism of injury is often not fully elucidated.^[2,3] A better understanding of pulmonary complications will help us recognize and further manage these conditions in the future. This review highlights recent articles published since June 2021 that increase our understanding of pulmonary complication frequency, diagnostic advances, lung disease pathogenesis, and potential future treatments for patients with lung injury and fibrosis.