Pulmonary manifestations of systemic vasculitis in childhood

Abstract

The systemic vasculitides are a heterogenous group of rare conditions with an incompletely understood aetiology. Any of the systemic vasculitides may cause respiratory disease, but some conditions are more likely to affect the pulmonary system, often through pulmonary infarction and diffuse alveolar haemorrhage. These conditions are often difficult to diagnose due to their rarity and significant clinical overlap with common respiratory conditions. Prompt diagnosis and management can significantly reduce morbidity and mortality.

Innovations in the Care of chILD Associated with Connective Tissue Disease and Immune Mediated Disorders

“…The most common age of onset is in early teenage years, although some children present in infancy. Lung involvement, considered to be a hallmark of MPA and GPA, is associated with increased morbidity and mortality -in one cohort of 28 patients, half of children required ICU admission and one third of these admissions were for respiratory failure, with five patient requiring mechanical ventilation [131][132][133].…”

“…In pediatric GPA, pulmonary involvement can range from asymptomatic with radiographic abnormalities (a prior report noted 41% of radiographic abnormalities found in patients with pediatric GPA were observed in asymptomatic patients [135]) to presentation with fulminant DAH [133]. In the ARChiVe cohort, including 183 patients with pediatric GPA, 74% had pulmonary involvement, with 54% reporting chronic cough and 42% with massive hemoptysis or alveolar hemorrhage [136,137].…”

“…PFTs may show abnormal DLCO (related to parenchymal disease or hemorrhage), restrictive lung disease, and/or obstructive disease (related to airway stenosis). CT findings may include nodular lung lesions (secondary to necrotizing, granulomatous inflammation, which may demonstrate cavitation and/or a "halo sign" related to adjacent hemorrhage), fixed pulmonary infiltrates, and more rarely (< 10% of cases), fibrosis, septal thickening and pneumothorax [133,136]. Bronchoalveolar lavage (BAL) with serial aliquots can be useful in evaluation for pulmonary hemorrhage.…”