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Systemic lupus erythematosus: Clinical, pathological and therapeutic aspects
Tram/us. Sci. 1992; 13:127-128 Printedin Great Britain. All rights reserved
0955-3886192 $5.00+0.00 Copyright0 1992 PergamonPress Ltd.
Editorial Review Systemic Lupus Erythematosus: Clinical, Pathological and Therapeutic Aspects Systemic lupus erythematosus (SLE) has become a major therapeutic challenge for the 1990s. On the one hand, numerous studies have shown a remarkable increase in the survival of cohorts of patients with SLE, when compared with historical controls.‘” On the other hand, patients with the most active, or the most resistant disease are being treated with more aggressive measures.4 A bimodal mortality pattern has been described for patients followed over long periods,5t6 and, for long term survivors, a proportion of the mortality is now associated with the development of atherosclerotic disease, probably as a complication of corticosteroid treatment.5 It therefore becomes important to attempt to define disease activity in patients with SLE, and to determine what factors may be of prognostic importance. This topic is addressed by Drs Gladman and Urowitz in this issue. They have been instrumental in developing a disease activity index for patients with SLE? further studies will be required to determine its value as a long term instrument of prognosis. Two of the major residual causes of death in SLE are involvement of the kidneys and of the central nervous system. Dr Kashgarian reviews the latest classification of renal involvement in SLE, discusses clinico-pathological correlations, and outlines their implications for therapy. Dr J. G. Hanly describes the patterns of nervous system involvement in SLE, draws attention to some of the proposed mechanisms of pathogenesis, and outlines treatment options. There is a brief review of some immunopathological aspects of SLE, with emphasis on the features which make it a paradigm of multisystem autoimmune disease, and an update on the role of immune complexes. Turning to therapeutic aspects, Drs Euler and Schroeder review the theoretical basis and practical results of their new approach to the therapy of severe SLE, which employs a combination of cyclical plasmapheresis and subsequent pulses of a cytotoxic agent. Finally, MS Nance reviews aspects of transfusion medicine which have become important in the management of the patient with SLE, and which will become even more critical if plasma exchange proves to have an important role in the management of this disease. This issue of Transfusion Science illustrates dramatically the collaborative nature of basic and therapeutic research in SLE. It has been suggested that Lupus has replaced syphilis as ‘The Great Imitator’. In this role it can be seen to act as a focus of interest for epidemiologists, immunologists, pathologists, transfusion scientists, nephrologists, neurologists and rheumatologists. Let us hope that this interaction will continue to lead to advances in therapy. REFERENCES 1. Wallace DJ, Dubois EL: Lupus Eryfhematosus,3rd edn. Philadelphia, Lea & Febiger, 1987, p. 592.
2.
Gladman DD: Prognosis of systemic lupus erythematosus and factors that affect it. CUR Opin Rheumatol 1990; 2:694-702. 3. Ginzler EM, Schom R: Outcome and
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prognosis in systemic lupus erythematoSUS. Rheumatol Dis Clin North Am 1988; 1467-78. 4. McCune WJ, Golbus J, Zeldes W, Bohlke P, Dunne R, Fox DA: Clinical and immunoionic effects of monthly administration of inkavenous cyclophoshhamide in severe systemic lupus erythematosus. N Engl J Med 1988; 18:1423-1431.
5. Urowitz MB, Bookman ASM, Koehler BE, Gordon DA, Smythe HA, Ogryzlo MA: The bimodal mortality pattern of systemic lupus erythematosus. Am 1 Med 1976; 60:221-225. 6. Rubin LA, Urowitz MB, Gladman DD: Mortality in systemic lupus erythematosus: the bimodal pattern revisited. Q \Med 1985; 55:87-98.
John Verrier Jones, BM, FRCP, FRCP(C) senior Editor Dalhousie University, Halifax Nova Scotia, Canada
0955-3886192 $5.00+0.00 Copyright0 1992 PergamonPress Ltd.
Editorial Review Systemic Lupus Erythematosus: Clinical, Pathological and Therapeutic Aspects Systemic lupus erythematosus (SLE) has become a major therapeutic challenge for the 1990s. On the one hand, numerous studies have shown a remarkable increase in the survival of cohorts of patients with SLE, when compared with historical controls.‘” On the other hand, patients with the most active, or the most resistant disease are being treated with more aggressive measures.4 A bimodal mortality pattern has been described for patients followed over long periods,5t6 and, for long term survivors, a proportion of the mortality is now associated with the development of atherosclerotic disease, probably as a complication of corticosteroid treatment.5 It therefore becomes important to attempt to define disease activity in patients with SLE, and to determine what factors may be of prognostic importance. This topic is addressed by Drs Gladman and Urowitz in this issue. They have been instrumental in developing a disease activity index for patients with SLE? further studies will be required to determine its value as a long term instrument of prognosis. Two of the major residual causes of death in SLE are involvement of the kidneys and of the central nervous system. Dr Kashgarian reviews the latest classification of renal involvement in SLE, discusses clinico-pathological correlations, and outlines their implications for therapy. Dr J. G. Hanly describes the patterns of nervous system involvement in SLE, draws attention to some of the proposed mechanisms of pathogenesis, and outlines treatment options. There is a brief review of some immunopathological aspects of SLE, with emphasis on the features which make it a paradigm of multisystem autoimmune disease, and an update on the role of immune complexes. Turning to therapeutic aspects, Drs Euler and Schroeder review the theoretical basis and practical results of their new approach to the therapy of severe SLE, which employs a combination of cyclical plasmapheresis and subsequent pulses of a cytotoxic agent. Finally, MS Nance reviews aspects of transfusion medicine which have become important in the management of the patient with SLE, and which will become even more critical if plasma exchange proves to have an important role in the management of this disease. This issue of Transfusion Science illustrates dramatically the collaborative nature of basic and therapeutic research in SLE. It has been suggested that Lupus has replaced syphilis as ‘The Great Imitator’. In this role it can be seen to act as a focus of interest for epidemiologists, immunologists, pathologists, transfusion scientists, nephrologists, neurologists and rheumatologists. Let us hope that this interaction will continue to lead to advances in therapy. REFERENCES 1. Wallace DJ, Dubois EL: Lupus Eryfhematosus,3rd edn. Philadelphia, Lea & Febiger, 1987, p. 592.
2.
Gladman DD: Prognosis of systemic lupus erythematosus and factors that affect it. CUR Opin Rheumatol 1990; 2:694-702. 3. Ginzler EM, Schom R: Outcome and
128
Txansfus. Sci.
Vol. 13, No. 2
prognosis in systemic lupus erythematoSUS. Rheumatol Dis Clin North Am 1988; 1467-78. 4. McCune WJ, Golbus J, Zeldes W, Bohlke P, Dunne R, Fox DA: Clinical and immunoionic effects of monthly administration of inkavenous cyclophoshhamide in severe systemic lupus erythematosus. N Engl J Med 1988; 18:1423-1431.
5. Urowitz MB, Bookman ASM, Koehler BE, Gordon DA, Smythe HA, Ogryzlo MA: The bimodal mortality pattern of systemic lupus erythematosus. Am 1 Med 1976; 60:221-225. 6. Rubin LA, Urowitz MB, Gladman DD: Mortality in systemic lupus erythematosus: the bimodal pattern revisited. Q \Med 1985; 55:87-98.
John Verrier Jones, BM, FRCP, FRCP(C) senior Editor Dalhousie University, Halifax Nova Scotia, Canada