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Systemic Lupus Erythematosus: Emerging Concepts: Part 1: Renal, Neuropsychiatric, Cardiovascular, Pulmonary, and Hematologic Disease

Dimitrios T. Boumpas, MD; Howard A. Austin, MD; Barri J. Fessler, MD; James E. Balow, MD; John H. Klippel, MD; and Michael D. Lockshin, MD
[+] Article, Author, and Disclosure Information

From the National Institutes of Health, Bethesda, Maryland. 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.

Copyright ©2004 by the American College of Physicians

Ann Intern Med. 1995;122(12):940-950. doi:10.7326/0003-4819-122-12-199506150-00009
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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.


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Figure 1.
Treatment of severe lupus nephritis. Top.222PBottom.P[39]

Probability of not doubling serum creatinine levels in 65 patients with severe active lupus nephritis randomly assigned to receive MP (intravenous methylprednisolone, 1.0 g/m body surface area monthly for 6 months), CY-S (intravenous cyclophosphamide, 0.5 to 1.0 g/m monthly for 6 months), or CY-L (intravenous cyclophosphamide, 0.5 to 1.0 g/m monthly for 6 months followed by quarterly infusions for 24 months) (Gehan test comparing CY-L with MP, = 0.037). Probability of no exacerbation of lupus activity on completion of monthly pulses in groups randomly assigned to receive CY-L and CY-S (Gehan test, = 0.006). Numbers of patients that remain at risk at various times are shown along the abscissa. Reproduced with permission from Boumpas and colleagues .

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Figure 2.
Magnetic resonance imaging in neuropsychiatric lupus.Left.2arrowsRight.22[71]

Axial T -weighted scan of the brain in a woman 24 years of age with systemic lupus erythematosus. Small foci of increased signal intensity are identified at the periventricular area of the white matter ( ). These lesions are caused by microvascular injury in the peripheral branches of the cerebral arteries resulting in ischemia, edema, and infarction. If the underlying process persists, these lesions may increase in number and coalesce, forming larger lesions. In contrast to the smaller lesions, these larger lesions may also be detected as hypodense areas on computed tomographic scans. Axial T -weighted scan shows increased signal intensity in the gray matter of the left basal ganglia. Abnormalities of the cortical or the deep gray matter are usually due to involvement of more proximal branches of the cerebral arteries, resulting in edema with high-signal intensity on T -weighted and proton density images. These lesions may resolve spontaneously or with therapy or may progress to infarction, in which case they may also be detected by computed tomographic scanning. In this patient, similar lesions were also seen in the left cerebral peduncle and in the cervical spinal cord. After treatment with oral glucocorticoids and pulses of cyclophosphamide, these lesions resolved within approximately 6 months (Reproduced with permission from Boumpas and colleagues .).

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Figure 3.
Computed tomographic scan of the lung in a patient with systemic lupus erythematosus and interstitial pneumonia.

This patient presented with several weeks of dry cough and dyspnea on exertion. Note the bilateral ground-glass opacities that do not obscure underlying vessels (6-mm axial section through lung bases). Open lung biopsy showed lymphocytic interstitial pneumonia with numerous germinal centers situated adjacent to bronchial epithelium and a less prominent inflammatory background of lymphocytes and plasma cells within the alveolar septae. These abnormalities resolved after therapy with high-dose glucocorticoids.

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