ARDS and Wegener's granulomatosis

The following report describes a case of fulminant ARDS in a 36-year-old woman. The underlying cause of this condition was an isolated manifestation of Wegener's granulomatosis. The acute impairment of gas exchange was treated by a prompt protective ventilation strategy that included rapid restoration of spontaneous breathing and prone positioning. Within a few days it proved possible to wean the patient from the ventilator and extubate her on day 7. The rapid recovery of the patient was due not only to the ventilation strategy, but more important, to the early diagnosis and treatment of the underlying disease. The diagnosis of Wegener's granulomatosis - a rare cause of ARDS - was based on an interdisciplinary effort that included specific immunological tests. Subsequent immunosuppressive therapy with cyclophosphamide and prednisolone during mechanical ventilation resulted in a marked improvement. Of critical importance was the fact that in addition to common causes of lung disease, differential diagnostic consideration was also given to rare causes. Determination of antineutrophil cytoplasmic antibodies, rheumatoid factors and antiphospholipid antibodies rapidly clarifies the vasculitis question. The specific treatment of Wegener's granulomatosis is discussed.