title: Clinical review: Vasculitis on the intensive care unit -- part 2: treatment and prognosis.
abstract: The 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.
authors.affiliation: Worthing Hospital, Worthing, UK. david.semple@wash.nhs.uk
authors.forename: David
authors.initials: D
authors.lastname: Semple
authors.forename: James
authors.initials: J
authors.lastname: Keogh
authors.forename: Luigi
authors.initials: L
authors.lastname: Forni
authors.forename: Richard
authors.initials: R
authors.lastname: Venn
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology; D000305:Adrenal Cortex Hormones / Q000008:administration & dosage / Q000627:therapeutic use; D015267:Churg-Strauss Syndrome / Q000188:drug therapy / Q000401:mortality / Q000628:therapy; D003422:Critical Care; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000188:drug therapy / Q000401:mortality / Q000628:therapy; D006509:Hepatitis B / Q000150:complications; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D007362:Intensive Care Units; D015337:Multicenter Studies as Topic; D010951:Plasma Exchange; D010488:Polyarteritis Nodosa / Q000188:drug therapy / Q000401:mortality / Q000628:therapy; D011379:Prognosis; D011446:Prospective Studies; D016032:Randomized Controlled Trials as Topic; D012074:Remission Induction; D012128:Respiratory Distress Syndrome / Q000209:etiology; D013997:Time Factors; D014657:Vasculitis / Q000150:complications / Q000188:drug therapy / Q000401:mortality / Q000628:therapy*
chemical_list: D000305:Adrenal Cortex Hormones; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide