pulmonary disorders
Pulmonary Renal Syndrome
Treatment of Pulmonary Renal Syndrome
Corticosteroids Sometimescyclophosphamide Plasma exchange Immunosuppression is the cornerstone of treatment of pulmonary-renal syndrome. Standard induction-remission regimens include pulse IVmethylprednisolone(eg, 500 to 1000 mg IV once a day for 3 to 5 days). As life-threatening features subside, the corticosteroid dose can be reduced; for example, 1 mg/kgprednisone(or equivalent) orally once a day is given for the first month, then tapered over the next 3 to 4 months. Cyclophosphamideshould be added to corticosteroid therapy in critically ill patients with generalized disease.Cyclophosphamide, at a dose of 0.5 to 1 g/m2IV, is given as a pulse once a month or orally (1 to 2 mg/kg once a day). Depending on the underlying type of the pulmonary-renal syndrome,rituximabmay be used instead ofcyclophosphamide; it is non-inferior and causes fewer adverse effects. Plasma exchange is also often used, particularly in Transition to maintenance therapy may occur 6 to 12 months after the initiation of induction therapy or after clinical remission. Maintenance therapy includes low-dose corticosteroids coupled with cytotoxic agents. However, relapse may occur despite ongoing therapy. 