[Wegener granulomatosis with severe alveolar hemorrhagic syndrome]

Abstract

The article presents the case of a female admitted for the suspicion of pulmonary TB (clinical and radiological pattern compatible with this diagnosis), in which the lack of bacteriological confirmation together with a rapid and dramatic deterioration of clinical, radiological and functional status excluded tuberculosis and oriented the diagnosis towards a severe alveolar hemorrhage; further tests confirmed a Wegener granulomatosis with pulmonary, renal and ORL manifestations. The evolution was rapidly favorable using pulse-therapy with cyclophosphamide and methylprednisolone i.v. The particularities of this case were: the use of helmet device for correction of the extremely severe hypoxemia for 3 weeks (no other studies reported such a long continuous duration of use of this device) and the lag between the pulmonary and the renal impairment.