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15 June 1995 | Volume 122 Issue 12 | Pages 940-950
Purpose: To review advances and controversies in the diagnosis and management of systemic lupus erythematosus with visceral involvement (renal, neuropsychiatric, cardiopulmonary, and hematologic disease).
Data Sources and Study Selection: Review of the English-language medical literature with emphasis on articles published in the last 5 years. More than 400 articles were reviewed.
Data Synthesis: Recent debates pertaining to lupus nephritis have focused on the value of kidney biopsy data and the role of cytotoxic drug therapies. Many studies have shown that estimates of prognosis are enhanced by consideration of clinical, demographic, and histologic features. For patients with severe lupus nephritis, an extended course of pulse cyclophosphamide therapy is more effective than a 6-month course of pulse methylprednisolone therapy in preserving renal function. Adding a quarterly maintenance regimen to monthly pulse cyclophosphamide therapy reduces the rate of exacerbations. Plasmapheresis appears not to enhance the effectiveness of prednisone and daily oral cyclophosphamide. Small case series have shown pulses of cyclophosphamide to be beneficial in patients with lupus and neuropsychiatric disease refractory to glucocorticoid therapy, acute pulmonary disease (pneumonitis or hemorrhage), and thrombocytopenia. Patients with systemic lupus erythematosus have an increased prevalence of valvular and atherosclerotic heart disease, apparently because of factors related to the disease itself and to drug therapy.
Conclusions: Cytotoxic agents are superior to glucocorticoid therapy for the treatment of proliferative lupus nephritis, but the optimal duration and intensity of cytotoxic therapy remain undefined. Definitive studies of the treatment of autoimmune thrombocytopenia and acute pulmonary disease and of the diagnosis and treatment of neuropsychiatric disease are not available.
Author and Article Information
From the National Institutes of Health, Bethesda, Maryland.
REVIEW
Systemic Lupus Erythematosus: Emerging Concepts: Part 1: Renal, Neuropsychiatric, Cardiovascular, Pulmonary, and Hematologic Disease
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Requests for Reprints: Dimitrios T. Boumpas, MD, National Institutes of Health, Building 10, Room 3N-112, Bethesda, MD 20892.
Acknowledgments: The authors thank Drs. John L. Decker, Alfred D. Steinberg, and Paul H. Plotz for their leadership in planning and implementing studies of systemic lupus erythematosus at the National Institutes of Health; Dr. Nicholas J. Patronas for invaluable assistance and useful comments in the discussion of neuroimaging studies in patients with systemic lupus erythematosus; Dr. Catherine K. Chow for useful discussions on the role of computed tomography in the diagnosis of pulmonary disease; Drs. W. Travis and C.L. Phillips for their assistance with the interpretation of the pathology of Figure 3; and Ms. Lisa A. Miller and Mr. Andrew S. Lerner for manuscript preparation.
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