Cyclophosphamide(Cytoxan) in the treatment of Pulmonary Vasculitis – pro

Pulmonary vasculitis describes a number of distinct disorders that are pathologically characterized by the destruction of blood vessels. Before the institution of immunosuppressive therapy, the mortality rate of patients with a systemic vasculitis was 75%, with a median survival of 5 mo. The addition of corticosteroid therapy improved mortality at 1 yr but had no impact at 3 yr (there was significant steroid-associated mortality and morbidity). The 5-yr mortality remained at 50%. The major breakthrough occurred when cyclophosphamide was added to corticosteroids, and this lowered the 5-yr mortality to 12%.

EUVAS study, Non-Renal Alternative With Methotrexate (NORAM) trial compared methotrexate with cyclophosphamide for the induction of remission in early disease. There were similar rates of induction of remission at 6 months; however, the time to remission was longer in the methotrexate arm (5.2 months vs 3.2 months), and the relapse rate was statistically higher in the methotrexate arm (74% vs 42%). On the other hand, the methotrexate was better tolerated than the cyclophosphamide. Hence, cycophosphamide with steroids is first line and methotrexate, in combination with corticosteroids, is now considered an alternative first-line regimen for the treatment of early, generalized disease.

Stephen K. Frankel, MD, FCCP, The Diagnosis and Treatment of Pulmonary Vasculitis
PCCSU Article | 04.01.08,3

CURRENT Medical Diagnosis & Treatment 2013
Pulmonary Vasculitis, The McGraw-Hill Companies

Stephen K. Frankel, MD, FCCP; Gregory P. Cosgrove, MD, FCCP; Aryeh Fischer, MD; Richard T. Meehan, MD; Kevin K. Brown, MD, FCCP February 2006, Vol 129, No. 2Update in the Diagnosis and Management of Pulmonary Vasculitis.
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