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Orthotopic liver transplantation substantially reduces the supply of the amyloid fibril precursor protein in hereditary apoAI amyloidosis, and the excellent outcome in this patient probably reflects the balance between deposition and turnover of amyloid having been altered in favor of the latter. These findings support the use of liver transplantation in patients with hereditary apoAI amyloidosis who develop hepatic dysfunction.

The prolonged bleeding times of 12 patients with chronic renal failure were significantly shortened at 1 and 2 h after an infusion of 0.4 µg/kg 1-deamino-8-D-arginine vasopressin (DDAVP). In 5 of these patients, the bleeding times 24 h after infusion had returned to approximate baseline values, suggesting that the effect of DDAVP is a temporary one. The levels of factor VIII coagulant activity, factor VIII related antigen activity, and factor VIII ristocetin cofactor activity were all normal or elevated prior to infusion, and a tendency to further elevation, although significant only for the factor VIII ristocetin cofactor activity, was observed after infusion of DDAVP. The latter observation suggests that the effect of DDAVP infusion on the bleeding time is unrelated to alteration of factor VIII activities.

193 ANCA = antineutrophil cytoplasmic antibody; CSS = Churg-Strauss syndrome; ICU = intensive care unit; MPA = microscopic polyangiitis; PAN = polyarteritis nodosa; PE = plasma exchange; WG = Wegener's granulomatosis. Available online http://ccforum.com/content/9/2/193 AbstractThe second part of this review addresses the treatment and prognosis of the vasculitides Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and polyarteritis nodosa. Treatment regimens consist of an initial remission phase with aggressive immunosuppression, followed by a more prolonged maintenance phase using less toxic agents and doses. This review focuses on the initial treatment of fulminant vasculitis, the mainstay of which remains immunosuppression with steroids and cyclophosphamide. For Wegener's granulomatosis and microscopic polyangiitis plasma exchange can be considered for first-line therapy in patients with acute renal failure and/or pulmonary haemorrhage. Refractory disease is rare and is usually due to inadequate treatment. The vasculitides provide a particular challenge for the critical care team. Particular aspects of major organ support related to these conditions are discussed. Effective treatment has revolutionized the prognosis of these conditions. However, mortality is still approximately 50% for those requiring admission to intensive care unit. Furthermore, there is a high morbidity associated with both the diseases themselves and the treatment.