Case 28-2005: A Case of Systemic Necrotizing Vasculitis
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《新英格兰医药杂志》
To the Editor: In Case 28-2005 (Sept. 15 issue),1 the authors describe a 42-year-old man who had a systemic necrotizing vasculitis secondary to the Churg–Strauss syndrome with rapidly progressive glomerulonephritis (with a serum creatinine level of 5.7 mg per deciliter at admission), systemic symptoms, eosinophilia, asthma, hemoptysis, sinusitis, purpura, and peripheral-nerve disease. The patient was successfully treated with corticosteroids and oral cyclophosphamide, with normalization of the serum creatinine level, erythrocyte sedimentation rate, urine sediment, and blood eosinophil count. The patient felt well, he was able to return to work, and tests for antineutrophil cytoplasmic antibodies became negative. Yet the patient continued to receive cyclophosphamide well over a year after treatment was begun. In view of the potential toxicity of cyclophosphamide, this approach is no longer the norm for the treatment of a systemic necrotizing vasculitis.2,3 The preferred current approach is to induce remission with three to six months of cyclophosphamide therapy and then to use a less toxic maintenance regimen of azathioprine, methotrexate, or mycophenolate mofetil. Since relapses are common, the current induction–maintenance approach, by minimizing the initial exposure to cyclophosphamide, allows for further cyclophosphamide treatment for late relapses.
Rajiv K. Dixit, M.D.
Northern California Arthritis Center
Walnut Creek, CA 94598
References
Case Records of the Massachusetts General Hospital (Case 28-2005). N Engl J Med 2005;353:1148-1157.
Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 2003;349:36-44.
Langford CA. Treatment of ANCA-associated vasculitis. N Engl J Med 2003;349:3-4.
The discussant replies: I agree that a shorter course is desirable, but two factors led us to prolong this patient's cytoxan therapy for the Churg–Strauss syndrome. The first was that the patient had prolonged multisystem problems, including persistent neuropathy and recurrent asthma; the second factor was that the patient declined to transition to azathioprine.
Henry Rose, M.D.
Berkshire Medical Center
Pittsfield, MA 01201
Rajiv K. Dixit, M.D.
Northern California Arthritis Center
Walnut Creek, CA 94598
References
Case Records of the Massachusetts General Hospital (Case 28-2005). N Engl J Med 2005;353:1148-1157.
Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 2003;349:36-44.
Langford CA. Treatment of ANCA-associated vasculitis. N Engl J Med 2003;349:3-4.
The discussant replies: I agree that a shorter course is desirable, but two factors led us to prolong this patient's cytoxan therapy for the Churg–Strauss syndrome. The first was that the patient had prolonged multisystem problems, including persistent neuropathy and recurrent asthma; the second factor was that the patient declined to transition to azathioprine.
Henry Rose, M.D.
Berkshire Medical Center
Pittsfield, MA 01201