Respiratory complications of the rheumatological diseases in childhood

Abstract

Pleuropulmonary manifestations of rheumatological diseases are rare in children but pose a significant risk to overall morbidity and mortality. We have reviewed the literature to provide an overview of the respiratory complications of the commonest rheumatological diseases to occur in children (juvenile systemic lupus erythematosus, scleroderma, juvenile dermatomyositis, mixed connective tissue disease, granulomatosis with polyangitis and juvenile idiopathic arthritis). Pulmonary function testing in these patients can be used to refine the differential diagnosis and establish disease severity, but also has a role in ongoing monitoring for respiratory complications. Early detection of pulmonary involvement allows for prompt and targeted therapies to achieve the best outcome for the child. This is best achieved with joint specialist paediatric rheumatology and respiratory reviews in a multidisciplinary setting.

Lung function in children with juvenile idiopathic arthritis: A cross‐sectional analysis

“…25 However, data on the incidence and prevalence of lung impairment in JIA children are scarce. 26 DLCO is a reliable test and preliminary studies documented a reduction in DLCO in JIA patients, 10,27 suggesting abnormalities of the alveolar-capillary interface, compatible with diffuse vascular and/or parenchymal lung disease. 28 The prevalence of pulmonary impairment observed in our study population was consistent with previous studies reporting a significant 22 Therefore, we hypothesized that our JIA group might hide an early onset of ILD, also considering that we included only outpatients, without severe disease and with a relatively short disease duration.…”

“…Anecdotal descriptions of pulmonary involvement in JIA children report pleural involvement, interstitial pneumonia, fibrosis, rheumatoid nodules, obstructive bronchiolitis, pulmonary vasculitis, and pulmonary hypertension . However, data on the incidence and prevalence of lung impairment in JIA children are scarce . DLCO is a reliable test and preliminary studies documented a reduction in DLCO in JIA patients, suggesting abnormalities of the alveolar‐capillary interface, compatible with diffuse vascular and/or parenchymal lung disease …”

Exhaled breath analysis in childhood rheumatic disorders—a longitudinal study

“…However, clinically significant lung involvement is usually rare in the most common disease entity, juvenile idiopathic arthritis (JIA) [1], and the advent of potent diseasemodifying antirheumatic drugs (DMARDs) and biologicals has reduced the burden of disease due to pulmonary involvement across the board in most childhood rheumatic disorders. On the other hand, new technologies have made it possible to noninvasively monitor relatively subtle changes in pulmonary inflammation, even in asymptomatic periods [2].…”

Hemorragia pulmonar em pacientes com lúpus eritematoso sistêmico juvenil

“…A prevalência de HP em pacientes com LESJ neste estudo foi similar a outros relatos ou séries de caso, variando de 2% a 5% 8,9,32 . O diagnóstico dessa complicação inclui sintomas/sinais respiratórios típicos, queda dos níveis de hemoglobina, e evidencia radiográfica de infiltrado pulmonar intersticial/alveolar, assim como hipoxemia devido à insuficiência respiratória aguda 9 .…”

“…O diagnóstico dessa complicação inclui sintomas/sinais respiratórios típicos, queda dos níveis de hemoglobina, e evidencia radiográfica de infiltrado pulmonar intersticial/alveolar, assim como hipoxemia devido à insuficiência respiratória aguda 9 . Em dois dos pacientes com LESJ com HP foi encontrado presença de macrófagos com hemossiderina em lavado broncoalveolar, sem evidencia de outras causas de sangramento 8 .…”