Subclinical pulmonary haemorrhage causing a restrictive lung defect in three siblings with a unique urticarial vasculitis syndrome

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Abstract
Three siblings with urticarial vasculitis syndrome (UVS) are described. All had restrictive lung function abnormalities caused by subclinical pulmonary haemorrhage. The latter was suspected after finding haemosiderin-laden macrophages and a friable bronchial mucosa during elective bronchopulmonary alveolar lavage (BAL). The chest radiographs were normal at presentation but after steroid withdrawal symptoms worsened, haemoglobin levels fell, and Case 1 developed acute pulmonary haemorrhage. This was documented by lung biopsy, which also revealed evidence of old haemorrhage and fibrosis. We concluded that these patients had a unique familial variant of UVS with a previously unreported restrictive lung disease due to subclinical pulmonary haemorrhage.

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