Lung manifestations in MPO-ANCA associated vasculitides in children.

OBJECTIVE

To describe lung manifestations in MPO-ANCA associated vasculitides in children.

METHODS

We retrospectively reviewed the medical records of patients with MPO-ANCA associated vasculitis, who were followed in two pediatric nephrology departments from January 2000 to December 2010.

RESULTS

Twelve patients were identified with MPO-ANCA over the study period. Their median age (IQR) at diagnosis was 10.5 (6.3-12.0) years, and their median duration of follow-up was 4.8 (1.2-7.5) years. Only five of them had pulmonary involvement with diffuse alveolar hemorrhage. Lung involvement was inaugural for four of five children. One child with severe chronic respiratory disease and renal failure died after 6 years of disease progression. Pulmonary function tests were available for 10 children. They were within normal ranges in four of five patients without clinical lung manifestations, and no significant impairment was observed in children with pulmonary complications.

CONCLUSIONS

Diffuse alveolar hemorrhage complicates 40% of cases of MPO-ANCA associated vasculitides in children with renal involvement. After an acute potentially severe phase, a complete recovery without significant functional impairment was observed in four of five affected children.