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Lupus in the developing world – is it any different?

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Systemic lupus erythematosus (SLE) is known to occur in all populations across the globe. In many respects SLE is similar across regions in its spectrum of clinical features, but the severity of the disease and comorbidity are appreciably different in the developing and industrialized worlds. Although data on the prevalence of SLE among Africans and Asians living in the tropics are limited, SLE is reportedly more common and more severe in people of African and Asian extraction living in industrialized countries. Renal disease is especially common in SLE patients in the developing world and is a major cause of morbidity and mortality. Discoid lupus and lymphopenia are frequent clinical features of SLE in patients of African extraction. Thrombotic complications and associated anti-phospholipid antibodies are less common in the Chinese and Black African SLE patients than in Caucasian patients. High frequencies of antibodies to extractable nuclear antigens, especially anti-Sm and anti-U1RNP, have been reported in SLE patients in many developing countries. Infections, including tuberculosis, are among the most important causes of comorbidity and mortality in SLE across all regions of the developing world. With a few exceptions, survival rates for SLE patients in developing countries are substantially lower than those reported in industrialized countries, with early death from infection and active disease. The bimodal pattern of mortality observed in many industrialized countries, is also less evident. While the poorer outcome in developing countries is partly related to inherent genetic differences, better outcomes can be achieved if new initiatives are undertaken to define the burden of disease, increase public awareness, and establish algorithms for diagnosis and treatment based on the available resources and local health-care delivery systems.

Section snippets

Epidemiology

Over the past 50–60 years there has been a rise of more than ten-fold in the annual incidence of SLE in industrialized Western countries.1 This temporal increase in SLE is thought to be due to both a true increase in incidence, related to exposure to environmental factors, and increased recognition of the disease. The epidemiology of SLE in the developing world remains largely unknown. Where formal epidemiological studies have been undertaken, the occurrence of SLE has been found to be within

Clinical and serological features

Studies from Western industrialized countries consistently show that the severity and burden of SLE is greatest in non-Caucasian ethnic groups of African and Asian extraction.*1, 22, *23 The clinical spectrum (Table 1), autoantibody profile, and comorbidity of SLE in the developing world are not dissimilar to those in non-Caucasian SLE patients living in industrialized countries.

Co-morbidity of infections

Infections are among the most important causes of morbidity and mortality in SLE across all regions.58 Several series in developing countries rank infections as either first or second to SLE disease activity as a cause or contributor to mortality (Table 2).*19, 20, *27, 40, 59, 60, 61, 62 This susceptibility to infection may be explained by several intrinsic and acquired defects in the immune system related to the disease itself or to the immunosuppressive therapies.20, 63, 64 Among the genetic

Variations in therapeutic approach due to resource constraints

The outcomes – including survival – in SLE are largely influenced by factors which include socioeconomic status, educational attainment, access to health care, physician availability, and treatment adherence.12, *77, 78 These interdependent factors are very important in developing countries where poverty remains widespread, and many patients live in rural areas where access to medical care is often rudimentary.12 The economic costs due to loss in productivity, both in household and workforce

Factors contributing to differences in outcome

Two major features characterize the divergence of outcome between the two ‘worlds’. Firstly, with few exceptions, survival rates in developing countries are substantially lower (Table 2) than in the industrialized world where 5-year survival rates for SLE are well in excess of 90%.82, 83 Secondly, the bimodal pattern of mortality that has emerged in industrialized countries in recent years, where active lupus and infections accounting for most early deaths (within 5 years of onset of disease)

Summary

In summary, there is clear evidence that SLE varies in spectrum and severity in the developing world compared to the industrialized world. While these differences are to some extent the result of inter-ethnic genetic differences, many of the differences in morbidity and mortality reflect disparities in health services across the globe.

The outlook for SLE patients in the developing world can be improved only by new initiatives undertaken by both private and government sectors: to better define

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