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Hemocoagulative Problems in the Critically Ill Patient pp 217–226Cite as

Pulmonary-Renal Syndrome

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Abstract

Pulmonary-renal syndrome is defined as the combination of diffuse alveolar haemorrhage (DAH) and glomerulonephritis. The majority of cases are initiated by small vessel vasculitides presenting antineutrophil cytoplasmic autoantibodies (ANCA): Wegener’s granulomatosis (WG), microscopic polyangiitis (MPA) and Churg–Strauss–syndrome (CSS). Up to 25% of these ANCA-associated vasculitides (AAV) have also an increased venous thromboembolic risk due to circulating anti-plasminogen antibodies. Pulmonary-renal syndrome in AAV is often fatal without treatment, which still relies on immunosuppression by cytotoxic agents. As such therapy may be lethal itself, accurate assessment of disease severity is mandatory. In this scenario, plasma exchange (PLEX) has proven a fast-acting therapeutic adjunct during severe disease as it limits the use of immunosuppressives and their life-threatening side effects. Novel biomarkers of AAV activity, as well as data from randomized trials investigating new agents, will be essential to improve treatment of severe and refractory forms in the future.

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  1. Anesthesia and Intensive Care, Cattinara Hospital, University of Trieste, Strada di Fiume, 447, 34141, Trieste, Italy

    Marco Zambon, Davide Cappelli & Giorgio Berlot

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  1. Marco Zambon
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  2. Davide Cappelli
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  1. , Department of Anesthesia and Intensive C, University of Trieste, Strada di Fiume 447, Trieste, I-34149, Italy

    Giorgio Berlot

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Zambon, M., Cappelli, D., Berlot, G. (2012). Pulmonary-Renal Syndrome. In: Berlot, G. (eds) Hemocoagulative Problems in the Critically Ill Patient. Springer, Milano. https://doi.org/10.1007/978-88-470-2448-9_14

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