PubMed · 14056914
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G H FAIRLEY. 1963-11-16. CYCLOPHOSPHAMIDE.. https://doi.org/10.1136/bmj.2.5367.1269-b
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Systemic lupus erythematosus in infants born to healthy mothers is a rare entity. We describe a male infant who presented at 1 month of age with pulmonary hemorrhage and glomerulonephritis due to systemic lupus erythematosus, confirmed serologically and histologically. He was managed with a combination of prednisone and intermittent cyclophosphamide, but also received mycophenolate mofetil, with a complete serological and clinical remission at 30-month follow-up. This case underscores the importance of a broad approach to the evaluation of pulmonary hemorrhage and glomerulonephritis in the very young and the need for aggressive immunosuppressive therapy to achieve sustained serological and clinical remission.
BACKGROUND: The majority of patients with Wegener's granulomatosis have disease flares after conventional medications are tapered. There is no consistently safe, effective treatment for the maintenance of remission. METHODS: We conducted a randomized, placebo-controlled trial at eight centers to evaluate etanercept for the maintenance of remission in 180 patients with Wegener's granulomatosis. The primary outcome was sustained remission, defined as a Birmingham Vasculitis Activity Score for Wegener's Granulomatosis of 0 for at least six months (scores can range from 0 to 67, with higher scores indicating more active disease). In addition to etanercept or placebo, patients received standard therapy (glucocorticoids plus cyclophosphamide or methotrexate). After remission, standard medications were tapered according to the protocol. RESULTS: The mean follow-up for the overall cohort was 27 months. Of the 174 patients who could be evaluated, 126 (72.4 percent) had a sustained remission, but only 86 (49.4 percent) remained in remission for the remainder of the trial. There were no significant differences between the etanercept and control groups in the rates of sustained remission (69.7 percent vs. 75.3 percent, P=0.39), sustained periods of low-level disease activity (86.5 percent vs. 90.6 percent, P=0.32), or the time required to achieve those measures. Disease flares were common in both groups, with 118 flares in the etanercept group (23 severe and 95 limited) and 134 in the control group (25 severe and 109 limited). There was no significant difference between the etanercept and control groups in the relative risk of disease flares per 100 person-years of follow-up (0.89, P=0.54). During the study, 56.2 percent of patients in the etanercept group and 57.1 percent of those in the control group had at least one severe or life-threatening adverse event or died (P=0.90). Solid cancers developed in six patients in the etanercept group, as compared with none in the control group (P=0.01). CONCLUSIONS: Etanercept is not effective for the maintenance of remission in patients with Wegener's granulomatosis. Durable remissions were achieved in only a minority of the patients, and there was a high rate of treatment-related complications.