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

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.