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Lupus No Longer An Acute Fatal Disease But A Chronic Living Disease

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– Lupus is a relatively common multi-system autoimmune disorder – The prognosis of lupus is changing with its evolution from an acute fatal to a chronic living disease – New challenges include the emergence of cardiovascular disease as a common cause of morbidity and mortality in long term survivors. – A change in emphasis in treatment is required with more attention being given to identifying and correcting cardiovascular risk factors.

Systemic lupus erythematosus (SLE), also known as lupus, is the prototype multi-system autoimmune disease. Once thought to be a rare, frequently fatal acute condition, it is now appreciated that it is relatively common (prevalence is approximately 1 in 2000).The Changing Prognosis of LupusThe prognosis of lupus has changed over the past five decades. In the 1950s lupus was associated with a 50% survival at 5 years. This has improved with each subsequent decade such that by the 1990s 10 year survival was 85% and the first report of 20 year survival appeared (68%) (1) (To see the figure please click here.)Reasons for Improved SurvivalIt is unclear why improved patient survival has occurred. Several factors may have contributed including improved community (including physician) awareness. Lupus Awareness Month occurs each October and brings this illness to the attention of the public via the media. Antinuclear antibody (ANA) testing is now readily available and has facilitated early diagnosis. Added to this is earlier and more aggressive treatment of patients with severe disease.What Challenges Remain?Despite the improved prognosis patient survival lags behind that of age-and sex-matched control populations. Nevertheless it is now uncommon for lupus patients to die in the early acute phase of disease, providing appropriate therapy is instituted promptly.Although the prognosis of lupus has improved dramatically, as treating doctors we should not be complacent about what has been achieved. Most lupus patients are in their 20s at the time of diagnosis. By the end of their first decade of disease they are only in their 30s. What does the future hold for these predominantly young women?The Second DecadeThe challenges of the second decade and beyond include a dramatically increased rate of cardiovascular and cerebrovascular disease (2). This is largely due to the effects of chronic inflammation as part of lupus. Superimposed are traditional cardiovascular risk factors such as hypertension, hyperlipidaemia, obesity and smoking. Some lupus patients also have antiphospholipid antibodies and/or raised homocysteine levels which contribute to the thrombotic risk.What other challenges do long term lupus patient survivors face?Infection, including opportunistic infections, remains a persistent threat, particularly for patients who require immunosuppression to control disease activity.Malignancy, especially lymphoma, occurs at an increased frequency in lupus patients. This is especially so in those who require prolonged immunosuppressive treatment to control disease activity.Other causes of morbidity include adverse effects from prolonged corticosteroid therapy such as cataracts, osteoporosis and avascular necrosis of bone.What can be done to reduce the risks?Greater attention needs to be paid to controlling disease activity, normalising blood pressure and serum lipid levels and maintenance of ideal body weight. Cessation of cigarette smoking is essential. There may be a role for prophylactic low dose aspirin.Bisphosphonates play a valuable role in preventing the development and progression of osteoporotic bone loss.ConclusionsThese challenges should not fill either the patient or their treating doctors with despair. 50 years ago most lupus patients didn’t live long enough to face the challenges of the second decade. It is now necessary to consider the management of lupus in a different light, recognising that the majority of patients will live a long time (3).Associate Professor Leslie Schrieber, MBBS (Hons)(USyd), Grad Cert Clin Ed (UNSW), MD (UNSW), FRACPUniversity of SydneyVirtual Rheumatology Centre, Editorial Advisory Board MemberReferences1. Abu-Shakra M, Urowitz MB, Gladman DD, Gough J. Mortality studies in systemic lupus erythematosus. Results from a single centre. 11. Predictor variables for mortality. J Rheumatol 1995;22:1265-70.2. Roman MJ, Shanker BA, Davis A et al. Prevalence and correlates of accelerated atherosclerosis in systemic lupus erythematosus. N Engl J Med 2003;349:2399-2406.3. Schrieber L The changing face of lupus. Int Med J 2004;34:3003-04.

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Posted On: 19 December, 2005
Modified On: 16 January, 2014

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