Pulmonary-renal syndrome

Abstract

Pulmonary-renal syndrome is characterized by lung and kidney diseases due to small-vessel vasculitis. It is mainly caused by antineutrophil cytoplasmic antibodies (ANCA)-related vasculitis and Goodpasture’s syndrome, but several other auto-immune diseases can be associated with this clinical presentation. Diffuse alveolar haemorrhage is caused by haemoptysis, dyspnoea, and anaemia, whereas acute renal failure with proteinuria and haematuria occurs due to rapidly progressive glomerulonephritis. Diagnosis is based on positivity of specific auto-antibodies, presence of blood on bronchoscopic alveolar lavage, and presence of specific glomerular lesions on the renal biopsy. The underlying auto-immune disorder must be identified as soon as possible in order to initiate specific treatment. Recent therapeutic protocols involving corticosteroids and immunosuppressive drugs such as cyclophosphamide or rituximab, associated with plasma exchanges have significantly improved the prognosis of this syndrome, which is still a difficult diagnostic and therapeutic challenge in the intensive care unit.