Severe alveolar haemorrhage in Churg-Strauss syndrome.

Abstract

We report two patients with Churg-Strauss syndrome (CSS), who presented with life-threatening alveolar haemorrhage as the major manifestation of their disease. One improved on high doses of steroids and cyclophosphamide; the other deteriorated on prednisolone alone, but responded rapidly to the addition of cyclophosphamide and plasma exchange. On follow-up, neither showed further evidence of lung disease except for well-controlled asthma. Pulmonary haemorrhage is a well-recognised phenomenon in patients with other forms of systemic vasculitis, and CSS must now be considered in the differential diagnosis of such cases.