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The rise and rise of lupus

Lupus can, in many ways be considered a modern disease. Even as recently as 1970, lupus was considered rare. In the 1960s, the world map of lupus had huge empty spaces. It now seems remarkable that lupus was barely thought to exist in Africa, Australia, South America or China!

Key points

  • Lupus is more common than multiple sclerosis

  • Prevalence of 1 in 800 in some population groups

  • Increasingly diagnosed worldwide

  • New drugs becoming available

All that has changed dramatically. Lupus is now recognised in centres throughout the globe. The international journal LUPUS runs a regular feature called lupus around the world'. One of the messages from this series of articles is the broad similarity in the clinical features of lupus in different lupus clinics.

Ethnic differences

While the broad clinical picture of lupus is consistent, there may well be differences in the severity and frequency of lupus in different ethnic groups.

In the US, for example, lupus has long been considered more severe in Afro-Caribbean and Hispanic populations, with kidney disease and blood pressure being more common. In the UK, lupus may be more common in Asian and Afro-Caribbean populations, rising to 1 in 800 of women in these populations.

On of the big mysteries of lupus has been its apparent rarity in Africa. To some extent this may be because of missed diagnoses, however recent studies in London have shown that lupus is certainly emerging in African immigrants.

The history of lupus

The skin features of lupus, particularly the more disfiguring forms of discoid lupus, have been recognised for over two centuries. However, it was only in the late 19" century that a number of physicians, notably the great William Osler, painted a wider picture of the disease.

In 1948, recognition of the disease was given a great impetus with the discovery of a lupus blood test - the so-called LE cell test. This test is based on the observation that in lupus patients an abnormal or characteristic blood cell can be seen under the microscope.

Some years later, the more sensitive anti-nuclear antibody (ANA) test was developed and this test became the standard screening test for lupus.

The next major breakthrough in the late 1960s was the development of the anti-DNA test - a highly specific test for lupus. Finally, in 1983 the anti-cardiolipin test was developed, in parallel with a description of 'sticky blood' (or antiphospholipid) syndrome.

An early milestone in the treatment of lupus was the recognition in 1896 by Dr Thomas Payne of St Thomas' Hospital, London, that quinine could have a beneficial effect both on the fever and joint pains in lupus, as well as the skin rash.

In the 1940s Hench and his co-workers discovered the remarkable effects of steroids, lupus being one of the first diseases to benefit from this Nobel prize-winning discovery.

In the 1970s, the use of immunosuppressive drugs, notably pulse (intravenous) cyclophosphamide, was a major advance in the treatment of severe lupus, especially lupus nephritis.

Despite these advances, the list of drugs available for the treatment of lupus has remained disappointingly small, until recently. Now there are two new, promising drugs: mycophenolate (Cellcept® and rituximab (Mabthera®).

Mycophenolate mofetil (MMF or Celleept® has been an extremely successful drug in the transplant world for many years and is already having a major impact in the treatment of lupus.

Rituximab is one of the family of monoclonals or designer drugs, having a specific action on the immune system's B cells (the cells responsible for the production of antibodies). Not only is this drug proving clinically useful, but the good news is that there are a number of similar agents in the pipeline.

Recent advances

What are the three major advances of recent times? Opinions will obviously differ - but here are ours:

  1. The more conservative approach to treatment - reducing over-usage of steroids, and fine tuning of drugs such as cyclophosphamide.

  2. The enormous improvement in prognosis - due not only to the direct treatment of the disease itself, but also paying attention to other related medical conditions such as high blood pressure, raised cholesterol and osteoporosis.

  3. The impact of antiphospholipid syndrome on the management of lupus - many features previously thought to be due directly to lupus (eg, strokes, seizures, balance disorders, leg ulcers) are now known to be due to 'sticky' blood. This has meant, for many lupus patients, a treatment directed against the development of blood clots, rather than more and more steroids.

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