In 1986, President Ronald Reagan signed a law that designated October of that year as Lupus Awareness Month. According to the Lupus Foundation of America, at least 5 million people worldwide have a form of lupus. This autoimmune disease is more prevalent in women, especially women of color. While there is no cure for lupus, we now know more about the disease than ever before. It is important to understand the different forms of lupus and how they affect one’s body and lifestyle.
Lupus is a chronic disease that can severely impact a patient’s life. Depending on the form of the disease, lupus can affect nearly every organ system in the body. It is an inflammatory condition in which the patient develops an immune response against
oneself (autoantibodies). Lupus can be widespread, involving both the skin and internal organs, or limited, involving the skin alone. The manifestations of the disease can be quite varied, from a benign, uncomplicated course to one that is very severe, even resulting in death.
Lupus that is limited to only the skin, without any evidence of systemic involvement, is termed discoid lupus erythematosus (DLE). This form of lupus manifests as scarring lesions on the skin that are commonly found in areas that are typically exposed to the sun (photodistributed) and are made worse with exposure to UV light (photosensitive). Often there is hair loss when lesions involve the scalp. The vast
majority of patients with DLE do not show any systemic effects; however, 5% of patients can develop signs of systemic involvement. Treatment of DLE is carefully guided and is directly related to the amount of skin involvement and the patient’s symptoms. (For example, patients often experience lesions that are tender or painful, and treatment will be tailored to such symptoms.) Generally, topical or intralesional steroids are used for DLE lesions in addition to strict sunscreen use and sun avoidance. In more widespread or symptomatic cases, oral immunosuppressant medications can be used.
When lupus affects the internal organs, it is referred to as systemic lupus erythematosus (SLE). The presentation can be quite varied and can affect the skin (creating a red rash across the cheeks and nasal bridge – a photodistributed distribution, as noted above), the kidneys, the heart, and cause muscle and joint pain. Often patients will feel fatigue and experience fevers and weight loss. Chest pain – due to heart or lung involvement – can be experienced, as well as abnormalities in the levels of different cells seen in the blood. Patients who are diagnosed with SLE will most often show abnormally high levels of circulating autoimmune antibodies (antinuclear antibodies, ANAs). If high levels of ANAs are identified in the blood, then further specific testing is required to define the diagnosis, as ANAs can be elevated in multiple diseases. Treatment of SLE is determined on a case-by-case basis, depending on the severity of the disease, but it often includes prescription drugs that either prevent or inhibit activity of the immune system (immunosuppressant agents).
Subacute cutaneous lupus erythematosus (SCLE) is a third form of lupus. In this form, a photodistributed skin rash is also seen. Often lesions in this type of the disease show flaking of the skin. There can be a genetic predisposition to the development of this disease, or it can develop as a side effect of certain medications (hydrochlorothiazide – a blood pressure medication – being the most common). Again, treatment is focused on decreasing sun exposure through sun avoidance and use of sun blocks. Treatment with topical steroids or other oral medications that alter the immune system behavior is often effective. In cases of drug-induced forms of SCLE, discontinuation of the offending medication is crucial.
In all cases, the impact of lupus erythematosus can be severe on a patient’s life. The development of scarred lesions on the face or scalp of a young patient can be difficult to overcome emotionally. Serious problems can develop in patients with SLE, leading to heart and kidney issues, sometimes necessitating dialysis due to loss of kidney function. Pregnancies can sometimes be complicated in patients with SLE, and risks of miscarriage may be higher than in the general population. Aggressive treatment and early diagnosis, however, can be keys to avoiding some of these complicated side effects.