Systemic Lupus Erythematosus...
How to outsmart it

by Nathan Wei, MD, FACP, FACR

Nathan Wei is a nationally known board-certified rheumatologist and author of the Second Opinion Arthritis Treatment Kit. It's available exclusively at this website... not available in stores.

Click here: Second Opinion Arthritis Treatment Kit

Information, in part, from the Americna College of Rheumatology and the Arthritis Foundation

Systemic lupus erythematosus (SLE) is the prototypical autoimmune inflammatory disease.

In SLE, the body’s immune system gets its wires crossed and, instead of protecting the body, attacks the patient. The immune system manufactures antibodies that are directed against various organ systems.

The cause of SLE remains unknown. Since women are affected more often than men (10:1), hormonal factors are felt to play a role. Patients with SLE apparently share some genetic similarities and there is a higher incidence of SLE among identical twins.

Peak incidence of lupus is in the 15 to 40 year age range. African Americans are affected more often than Caucasians. The prevalence of lupus is approximately 2.5-5.0 per cent.

The most important clinical findings are:

Skin: A classic presentation is the butterfly rash across the cheeks and bridge of the nose. Other sun-exposed areas are also affected. Most lupus rashes are aggravated by sunlight exposure.

Kidney: This is very common and is a major cause of both morbidity and mortality. Immune complexes, which are combinations of patient DNA and antibodies against the DNA, form deposits in the kidneys. These immune complexes also attract and bind another protein in the blood called complement. The end result is a progressive inflammatory reaction that causes the kidney to leak protein and to lose function. Abnormalities in the urinalysis as well as the 24 hour urine collections for protein and creatinine clearance (a measure of kidney function) provide clues to the presence of lupus kidney disease.

Central nervous system: This organ is involved in almost 50 per cent of SLE patients. It is often difficult to tell whether the symptoms a patient has are due to the lupus, or whether other problems are causing the symptoms. These other possible causes include infection, medications (e.g., steroids), metabolic problems (such as kidney failure), other medical conditions (such as severe hypertension), and primary psychiatric problems. There are no definite diagnostic tests for central nervous system lupus. Analysis of spinal fluid, EEG, and MRI may assist in ruling out some of these other causes of abnormal central nervous system function.

Musculoskeletal: About 90 per cent of patients have musculoskeletal signs and symptoms. Most patients have joint pain but few have active inflammation of the joints. The arthritis in lupus does not have the erosive destructive potential that rheumatoid arthritis does. The most commonly involved joints are the hands, wrists, and knees. In the hands, reversible deformity, called “Jacoud’s arthropathy” may be seen. Another joint problem seen in SLE patients is avascular necrosis. This is a condition where the ends of long bones, typically in the hip, knee, and shoulder, “die” from lack of blood supply. Corticosteroid therapy is usually the cause. This condition is associated with much pain and disability and often requires joint replacement surgery.

Blood vessels: SLE can affect the blood vessels in many ways. Kidney disease may lead to hypertension with subsequent risk of stroke and heart attack. Inflammation of blood vessels (vasculitis) may affect virtually any set of blood vessels and causes significant and possibly life threatening damage. Prolonged steroid therapy can increase the risk of cardiovascular disease. Finally, the presence of abnormal antibodies (anticardiolipin antibodies) predispose patients to stroke.

Lungs: Inflammation of the lung tissue can compromise lung function. In addition, patients with lupus are at increased risk for developing pneumonia due to opportunistic infections because of immunosuppressive drug therapy.

The major diagnostic screening test for lupus is the anti nuclear antibody (ANA). The ANA is very sensitive but not specific. Positive ANA tests are seen in other disease states and occasionally even in normal people. Generally, the higher the level of ANA, the more likely the patient has “something.” In our lab, a titer of 1:160 or higher is considered positive and should be followed up on, depending on the clinical signs and symptoms a patient has. If a patient has virtually no symptoms, we may decide to wait a bit longer. Levels of ANA lower than this are equivocal.

ANA patterns may also present clues. However, it is beyond the scope of this website to go into a detailed discussion of ANA patterns.

Antibodies to double stranded DNA are considered very specific for SLE since they are uncommon in other disease states. High titers of anti-DNA also indicate a poorer prognosis. Following the level of anti DNA antibodies may be helpful in assessing the course of disease and response to treatment.

Other autoantibody tests such as anti-Ro, anti-La, anti-Sm and anti-RNP may also be helpful in further evaluating SLE. And like anti DNA, the levels of these antibodies tend to fluctuate with disease activity.

American College of Rheumatology Criteria for the Classification of SLE is a helpful tool for classification of patients. A patient may be classified as having SLE if they have 4 or more of the 11 criteria. These criteria are 95 per cent specific and sensitive. This means that a patient with SLE may not have 4 criteria and that a patient with more than 4 criteria may not have SLE. The moral of the story…Be careful.

1. Malar rash
2. Discoid lupus
3. Photosensitivity
4. Oral ulcers
5. Arthritis
6. Serositis (Inflammation of the lining outside the lungs or abdomen)
7. Renal disorder
8. Neurologic disorder
9. Hematologic disorder (anemia, low platelets, low white blood cell count)
10. Immunologic disorder (antibodies to double stranded DNA)
11. Antinuclear antibody

Treatment depends on the signs and symptoms a patient with SLE might present with. For instance, a patient with aches and pains or with rash might do well with hydroxychloroquine (Plaquenil). Adding a non steroidal anti inflammatory drug for a patient with aches and pains is also helpful. Special attention to kidney function is required because patients with SLE are very sensitive to the kidney altering effects of anti inflammatory drugs. Skin manifestations also respond to topical steroids.

Oral or intravenous steroids are used for patients with severe internal organ system involvement. The dose will depend on the severity of the problem. In patients where steroids are ineffective or where the dose needed is extremely high and needs to be tapered, use of immunosuppressive drugs such as azathioprine, cyclophosphamide, and mycophenolate mofitil (Cellcept) are indicated.

Recently, a B-cell therapy, Benlysta, was approved by the FDA for use in lupus. Reviews on its effectiveness are mixed.

Careful attention to tapering steroids, managing hypertension, treating clotting disorders, and generally attending to other co-morbid conditions is crucial.

Further information on drug therapy may be found in the treatment section.

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