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Discussing SLE (Lupus)

I am particularly interested in Systemic Lupus Erythematosus, an autoimmune disease that occurs when the body's tissues are attacked by its own immune system. Systemic lupus erythematosus is also known as the great mimic. This disease can affect almost any organ in the body, not just the joints. Because it affects so many different parts of the body, it can be confused with many other diseases. I am pleased to have you join me as we discuss systemic lupus erythematosus.

What is Systemic Lupus Erythematosus?

The name Systemic Lupus Erythematosus comes from the characteristic rash that can occur across the cheek and over the bridge of the nose. The name "Lupus" comes from the rash that mimics the facial pattern of the Wolf. The rash is red, hence the term erythematosus, and since the disease is more than skin deep, we use the term systemic.

Recognizing SLE

When Lupus occurs as a skin deep condition only, it is called cutaneous lupus erythematosus. The skin rash looks the same whether it is cutaneous or systemic, but there is no internal organ or joint involvement in skin deep Lupus erythematosus. Systemic lupus erythematosus or SLE is a relatively rare disease. SLE affects about 290,000 people in the United States, which translates to about 1:1000 white women. SLE can cause arthritis, and inflammation of the lining of the lungs, the heart, the blood vessels, and nerves. Serious internal organ involvement includes pleurisy, pericarditis, kidney inflammation or nephritis, vasculitis, and prominent vasospasm, also known as Raynauds phenomenon. Raynauds phenomenon is the blanching of fingertips, with cold exposure, followed by deep purple discoloration and a brighter red infusion of the skin as the fingertip warms. Other less common features of systemic lupus erythematosus include seizure, depression, psychotic behavior, unexpected blood clots, miscarriages, low blood counts, and even dryness of the eyes and mouth. The dryness of the mouth and eyes is sometimes referred to as secondary Sjogren's syndrome.

Organ involvement in SLE

Joint disease and skin rash of the most common features of systemic lupus erythematosus. But internal organ involvement is also a concern in up to 60% of individuals with lupus. We always need to be on guard for the development of systemic involvement. CNS means the central nervous system, and up to 15% of patients with systemic lupus erythematosus will have some inflammation that affects the brain, n including psychosis, depression, stroke, or even dementia.

What causes SLE?

Systemic lupus erythematosus is a complex disease and it is also complicated to explain what causes it. There is clearly a genetic component, but it is not inherited in the same fashion as blue eyes or curly hair. Many genes increase susceptibility to SLE. These genes increase immune system reactivity, with antibodies that clump with our own DNA to produce large molecules called immune complexes. Immune complexes deposit on blood vessel walls in joints, and internal organs like the lungs and kidneys, stimulating severe inflammatory reactions that damage tissues. These inflammatory reactions consume a protein in the blood called complement. Sun exposure or tobacco use enhances the formation of immune complexes. There's many more young women than men with SLE. There is also more SLE in blacks, Asians, and in individuals who come from lower economic strata.

Lupus Genetics

The genetic influence on SLE is complex, as twin studies have shown. If twins are identical, and one has SLE, the other twins has 25 to 50% chance of getting SLE too. If the twins are just fraternal however, that risk of both having lupus fall to about 5%. Even if you do not have a close relative with systemic lupus erythematosus, there may be a higher risk of autoimmunity running in the family, including thyroid disease, diabetes, or rheumatoid arthritis.

Signs of Lupus

Discoid lupus skin rashes are red, raised, and aggravated by sun exposures. They will frequently scar and when they occur on the scalp, it can be associated with permanent hair loss (alopecia). It is much harder to control discoid lupus in patients who continue to smoke.

Additional lupus rashes include non-scarring, reversible hair loss or a malar rash across the bridge of the nose and cheeks which does not scar. Sometimes we do see a very diffuse red flat rash with sun exposure (photodermatitis).

The arthritis associated with SLE is a small joint symmetric arthritis that absolutely loves the middle row of knuckles. Sometimes, you can get a redness over the skin between the knuckles. If the arthritis is allowed to continue without adequate anti-inflammatory treatment, a characteristic deformity known as Jaccoud arthropathy occurs as seen in the lower right-hand corner.

A variety of eye troubles occur, from simple eye dryness which can still be quite disabling, to inflammation of the eye that threatens vision.

Mouth sores, mouth dryness, and blisters can all occur as a complication of SLE.

These are pictures of Raynauds phenomenon, and on the right we see classic white attacks. White attacks represent a complete lack of blood flow and indicate tissue damage is occurring whereas the dark purple discoloration is actually a milder form of Raynauds phenomenon with residual blood flow.

When blood vessels become inflamed, they can clot off, and the tissues that are fed by those blood vessels die. This is known as vasculitis and it can be life-threatening in SLE.

Treatment

Treatment for skin deep lupus and the rash associated with SLE, starts with sun avoidance and the discontinuation of tobacco. A variety of topical creams can help with skin inflammation, and if the rash remains difficult to manage, oral medications can be helpful.

The treatment of SLE has not changed much for the last 40 years. It includes anti-inflammatory therapy like aspirin and corticosteroids like prednisone. Immunomodulators and immunosuppressive medications like hydroxychloroquine, azathioprine, methotrexate, and more recently Mycophenolate are used for systemic lupus erythematosus. Life-threatening systemic lupus erythematosus may require chemotherapy drugs like cyclophosphamide.

In conclusion:

Today, the diagnosis of SLE rarely means a life threatening problem, and usually is a mild systemic inflammation that is quite compatible with a long normal life. A good reason for why people do so well today it is that more people are diagnosed with mild SLE on the basis of sophisticated blood tests, and there are better treatments, used by rheumatologists who specialize in the treatment of lupus.

Thanks for taking time to learn more about SLE. Our team of Mayo Clinic trained, board certified rheumatologists at the Arthritis Center of NE are ready to serve you in the diagnosis and treatment of arthritis and autoimmune diseases. We invite you to call 402-420-1212 to schedule an appointment with one of us.

Categorized in: Lupus
About the Author

Dr. Robert Valente earned a BS in Microbiology from the University of Minnesota, then graduated from Mayo Medical School. After finishing his internal medicine internship and residency… Read more.