Between 4 and 250 per 100,000 people worldwide have systemic lupus erythematosus (SLE), a syndrome in which the body's immune system erroneously attacks the body's own cells rather than protecting them from outside invaders. The vast majority (90%) of people with lupus are women, usually of childbearing age. There is also some evidence that lupus is more common in people of African, West Indian, and Chinese descent.
No one knows for sure what causes SLE, but predisposing factors include genetic factors, environmental factors (including sun exposure), some drugs such as sulfa antibiotics, and hormonal factors.
Patients with mild disease who don’t respond to anti-inflammatory drugs present their own set of challenges.
Patients with SLE may suffer for years before receiving a diagnosis and treatment - especially when the disease is mild. Because symptoms are non-specific and resemble those of so many other disorders, physicians often don't suspect SLE initially. But it is important to consider the possibility when a patient complains of recurrent bouts of malaise, fever, loss of appetite, weight loss, muscle aches, hair loss, and painful ulcers in the mouth and nose (see sidebar overleaf for additional symptoms).
Untreated, severe SLE can result in renal failure. It can also lead to blood disorders, including anaemia, leucopoenia, and thrombocytopenia; neurological disorders, including seizures or psychosis; myocarditis; and immune disorder. Musculoskeletal complications include disabling arthritis, osteonecrosis, and osteoporosis secondary to steroid therapy. So it's important to diagnose the disease and refer patients to a rheumatologist for ongoing treatment in order to improve quality of life, and in severe cases, life expectancy.
Because SLE is a chronic disease, patients also need extensive health education so they can learn to best manage their condition. They will need to comply with office visits and medications and may need to make lifestyle modifications to reduce or prevent associated problems such as hyperlipidemia, obesity and hypertension. An ongoing partnership between the primary care physician and the rheumatologist is essential in the long-term management of patients with SLE.
The good news is that many promising new treatments are in clinical trails, and geneticists are making rapid progress in determining which genes may be responsible for causing the SLE, says Dr. Gary Gilkeson, Professor of Medicine/Microbiology and Immunology at the Medical University of South Carolina.
In this ‘In Practice' article, Gilkeson, who has authored over 100 articles on lupus and is Chairman of the Medical and Scientific Advisory Board for the Lupus Foundation of America, talks about how to diagnose and treat lupus and why he is optimistic about the future prospects for lupus patients worldwide.
There is no single symptom or finding that is sufficient in itself for making the diagnosis of SLE, says Gilkeson. Diagnosis requires a thorough physical examination and laboratory tests including complete blood count, platelets, erythrocyte sedimentation rate (ESR), anti-nuclear antibodies, and urinalysis. Serologic tests are useful to confirm the diagnosis.
Lab findings for SLE are diverse. ESR is usually elevated, C-reactive protein value is often normal; mild or moderate anaemia is common. There may also be leucocytopenia (lymphocytopenia), and mild thrombocytopenia. Antinuclear antibodies are found in over 90% of patients.
Patients suspected of having SLE should always be referred to a rheumatologist for confirmation of the diagnosis, says Gilkeson. If there are signs of kidney involvement, the patient should also be referred to a nephrologist for their input.
SLE is a chronic disease that evolves over time. Patients who have skin and joint disease remain at risk for kidney disease even after having SLE for decades. So physicians need to continually monitor patients, even when the disease appears to be inactive. It is also important to establish a close partnership with the rheumatologist and any other physicians caring for the patient.
Treatment for SLE depends on the organ system involved and disease severity. All patients should be discouraged from sunbathing and encouraged to use sunscreens.
The most common drugs used to treat SLE include non-steroidal anti-inflammatory drugs (NSAIDS), anti-malarials (such as hydroxychloroquine, chloroquine, or quinacrine), corticosteroids (such as prednisone, hydrocortisone, methylprednisolone, or dexamethasone), immunosuppressive drugs (such as azathioprine, cyclosphamide, or methotrexate). In addition, medications such as rituximab and mycophenolate mofetil are increasingly being used off label to treat patients with SLE.
There is also a lot of interest in the potential role of vitamin D in treating SLE. A clinical trial is poised to begin in four centres in the U.S.
The main challenge for physicians, says Gilkeson, is treating the active phase of severe disease without allowing the treatment itself to cause long-term damage. "We still don't have great treatments for patients with central nervous system or renal involvement," he explains. "We can treat effectively 50 to 60% of them, but there is a large proportion that progress despite therapy.
Patients with mild disease who don't respond to anti-inflammatory drugs present their own set of challenges. "We don't really have a middle ground treatment," says Gilkeson, so physicians need to carefully weigh the benefits against the side effects of the more potent toxic medications.
A hopeful time
Fortunately, there may soon be additional therapies available for treating patients with SLE. There are around 15 under investigation and several are expected to be approved, says Gilkeson. These new treatments include anti B cell therapies (rituximab) and anti-BLyS (B lymphocyte stimulator) treatments, which target the B-lymphocyte stimulator. Anti-BLyS may be used for treating milder disease. In addition, treatments using drugs that block alpha interferon have the potential to be useful. "It is a very optimistic time," he adds.
He mentions another positive development: within the next six months a widely agreed upon set of treatment guidelines should be available from the Lupus Foundation, through the Systemic Lupus International Collaborating Clinics.
Last, SLE researchers from around the world have gotten together and combined the DNA and clinical data they've compiled into large datasets. Today, geneticists can investigate the genetics of 10,000 patients rather than a few hundred, and the analyses are being done across ethnic groups. "Most of the genetics of lupus will be worked out over the next two to three years," says Gilkeson. "There will not be a single gene, but a complex array of genes involved. But it will give us further insight into what is causing the disease" -- which should ultimately lead to even more novel treatments.
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