Severe alveolar haemorrhage in Churg-Strauss syndrome.
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.
Clutterbuck
E J
EJ
Department of Medicine, Royal Postgraduate Medical School, Hammersmith Hospital, London, U.K.
Pusey
C D
CD
D000328:Adult; D001249:Asthma / Q000150:complications; D001379:Azathioprine / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D008171:Lung Diseases / Q000209:etiology*; D008875:Middle Aged; D011650:Pulmonary Alveoli; D013577:Syndrome; D014657:Vasculitis / Q000150:complications* / Q000188:drug therapy
D005938:Glucocorticoids; D003520:Cyclophosphamide; D001379:Azathioprine