An Overview of Lupus

Show Article Table of Contents

Lupus is an autoimmune disease that takes on several forms, of which systemic lupus erythematosus (SLE) is one. Lupus can affect any part of the body, but it most commonly attacks your skin, joints, heart, lungs, blood cells, kidneys, and brain. Around 1.5 million Americans have some form of lupus, according to the Lupus Foundation of America, with an estimated 16,000 newly diagnosed each year. Anyone at any age can acquire the disease, though most lupus patients are women between the ages of 15 and 45.

The Immune System

Normally, our immune system protects our body against microbial invaders like viruses and bacteria. With autoimmune disease, this normally protective immune system malfunctions and begins to attack the body.

When this happens, one can experience inflammation (the primary feature of lupus), pain, and tissue damage. Inflammation in and of itself can cause pain, heat, redness, swelling, and loss of function, either internally (certain organs), externally (primarily the skin), or both.

Autoimmune diseases like lupus can be difficult to diagnose and differentiate.

Types of Lupus

There are four types of lupus that affect different parts of the body. Even if you have the same type of lupus as someone else, your symptoms won't necessarily be the same, as the disease is highly individualized.

Systemic Lupus Erythematosus (SLE)

The most common form of lupus is systemic lupus erythematosus (SLE), which can affect many parts of the body, including the joints, skin, blood vessels, and organs. This type affects around 70 percent of people with the disease and is typically what is being referred to when people mention "lupus."

SLE sufferers may go through years of red rashes, extreme fatigue, painful or swollen joints, or fevers without ever noticing a pattern to the flares or a physician ever being able to diagnose SLE. These symptoms may come and go all at once and can range from mild to severe.

SLE is often diagnosed using an anti-nuclear antibody blood test (ANA), which identifies autoantibodies that attack your body's own tissues and cells. They're found in the blood along with healthy antibodies, red blood cells, white blood cells, and other blood matter.

A positive ANA does not automatically mean you have lupus, but rather that result is one piece of the puzzle of a lupus diagnosis that includes your symptoms, a physical examination, and other, more specific laboratory tests.

Drug-Induced Lupus

Drug-induced lupus is associated with similar symptoms, but they are specifically brought on by certain types of drugs, usually taken over long periods of time. Drug-induced lupus is completely reversible once the drug is discontinued, and symptoms usually go away within six months. This type accounts for around 10 percent of lupus cases.

Many drugs have been known to cause this form of the disease, but several are considered primary culprits. They are mainly anti-inflammatories, anticonvulsants, or drugs used to treat chronic conditions such as heart disease, thyroid disease, hypertension (high blood pressure), and neuropsychiatric disorders. The three drugs mostly to blame for drug-induced lupus are:

  • procainamide - used to treat heart arrhythmias
  • hydralazine - used to treat hypertension
  • isoniazid - used to treat tuberculosis

Cutaneous Lupus

Some people have only skin manifestations of lupus, and they are diagnosed with cutaneous lupus—a separate type of lupus that, alone, accounts for around 10 percent of lupus cases. However, cutaneous lupus lesions also occur in two-thirds of people diagnosed with SLE.

As with other forms of lupus, it's a case of your body attacking its own normal skin. The exact cause of this form is unknown, although women are more likely to have it and it can run in families. Cigarette smoking and sunlight have been shown to exacerbate the condition.

There are, generally speaking, three types of cutaneous lupus, including:

  • Chronic cutaneous (discoid lupus): In discoid lupus, the most common form of chronic cutaneous lupus, inflammatory sores develop on your face, ears, scalp, and on other body areas. These lesions can be crusty or scaly and often scar. They usually don't hurt or itch. Some patients report lesions and scarring on the scalp, making hair re-growth impossible in those areas. Most people with discoid lupus do not have SLE. In fact, discoid lupus is more common in men than in women. 
  • Subacute cutaneous: The skin symptoms of subacute cutaneous lupus are usually mild. People with this condition, which is also its own form of lupus, present with reddish-purple plaques, which are firm and raised, flattened skin lesions. These plaques can be found alone or in groups and range in size from 5 mm to 20 mm, usually appearing on the trunk, including the upper chest and back. About 10 percent of people with SLE have subacute cutaneous lupus. Certain drugs may also cause subacute cutaneous lupus. 
  • Acute cutaneous: This is the type of skin flare that occurs when your SLE is active. Lesions associated with acute cutaneous lupus appear as flattened areas of red skin on the face, reminiscent of a sunburn—the telltale butterfly rash. These lesions can appear on the arms, legs, and body, and are photosensitive. Though the lesions may discolor the skin, they don't scar. Lesions typically appear during a flare or after sun exposure.

    Note that both chronic/discoid and subacute cutaneous lupus may occur independently, or they may be manifestations of SLE, whereas acute cutaneous lupus does not occur outside of SLE.

    Neonatal Lupus

    Neonatal lupus is a rare form of temporary lupus affecting a fetus or newborn. It's not true lupus: It occurs when the mother’s autoantibodies are passed to her child in utero. These autoantibodies can affect the skin, heart, and blood of the baby. Fortunately, infants born with neonatal lupus are not at an increased risk of developing SLE later in life.

    Many babies with neonatal lupus will have skin rashes at birth. The rest will break out typically within two to five months. Sun exposure tends to bring on the outbreak.

    Rashes, on average, will disappear at about six months or soon after, as the mothers' autoantibodies disappear from the infant. Treatment for skin lesions is typically no more than ointments to help relieve the severity of the breakouts.

    Though it's rare, some babies of mothers with lupus can be born with a heart condition that is permanent but treatable using a pacemaker. This abnormality can be detected as early as the 18th week of pregnancy.

    Childhood Lupus

    Childhood lupus affects somewhere between 5,000 and 10,000 children under 18 years of age in the United States. It is most often diagnosed between ages 11 and 15. Children of any age can have lupus, however, and lupus can strike children of any race or ethnicity.

    Lupus affects children similarly to the way it affects adults in that it expresses itself differently in each person, though it seems to have more organ involvement. This may be because children have often been sick for a longer period of time when they are finally diagnosed.

    Symptoms in children are similar to symptoms in adults, the most common of which are fatigue and achiness. Obvious disease symptoms include fever, butterfly rash, and kidney involvement. Most cases are successfully diagnosed when an ANA blood test is included in diagnostic tests.

    Treatment may need to be a little more aggressive for kids, but doctors also have to be careful regarding the long-term side effects of medications, particularly corticosteroids like prednisone. Most kids lead a normal childhood with the right treatment and care.

    Symptoms

    Early and chronic lupus symptoms mimic the symptoms of several diseases, which can lead to misdiagnosis. Initial symptoms often include:

    • Fever
    • Malaise, or general discomfort
    • Joint pain
    • Muscle pain
    • Fatigue

    Usually, it isn't until those symptoms are coupled with other, more suggestive signs that doctors tend to walk down the lupus path. Those signs include, but are not limited to:

    • Skin ulcers, lesions, and rashes, most significantly the malar rash, commonly called a butterfly rash
    • Anemia
    • Iron deficiency
    • Cardiac issues, such as pericarditis, myocarditis, and endocarditis
    • Pleuritis and other lung inflammation
    • Renal issues, including painless hematuria or proteinuria (blood or protein in your urine)
    • Seizures
    Signs and Symptoms of Lupus

    Causes

    Lupus is still a relative mystery to medical researchers. An exact cause of the disease remains unknown, and many still debate whether lupus is one disease or the combination of several similar diseases.

    But the consensus on the most likely ways in which lupus develops include:

    • Genetics (risk factors you inherit from your parents)
    • Environmental triggers (from medications, stress, infections, and/or exposure to sunlight)
    • Reaction to drugs (in the case of drug-induced lupus)
    Causes of Lupus

    Diagnosis

    If your doctor suspects that you have either cutaneous lupus erythematosus alone or in combination with systemic lupus erythematosus, several factors are considered, including:

    • Physical exam findings
    • Laboratory findings
    • Antibody tests
    • Tissue biopsy
    • Direct immunofluorescence

    Ultimately, because of the wide range of symptoms, it can be challenging for your doctor to diagnose lupus, and most people are first diagnosed five years after first developing symptoms. Thus, if you feel that you may be experiencing symptoms consistent with a diagnosis of lupus, be sure to visit your physician and express this concern.

    The prognosis for those with lupus often depends on the amount of organ involvement. In other words, is the disease targeting organs rather than skin and joints? Survival for lupus patients with central nervous system symptoms, major organ involvement, and/or kidney disease, is likely to be shorter than those with only skin and/or joint disease related to lupus. The most common cause of death associated with lupus is an infection due to immunosuppression, caused by medications used to manage the disease, especially early in ​the disease.

    How Lupus Is Diagnosed

    Treatment

    Currently, there is no cure for lupus. Treatment focuses on controlling the autoimmune response, limiting inflammation and organ damage, and relieving symptoms:

    • Drug therapy, such as anti-inflammatory drugs and immune system modulators
    • Lifestyle moderation, including weight loss and protection from the sun
    • Acupuncture (and other alternative treatments)
    • Physical therapy

    For some patients who are mildly afflicted, lupus symptoms can be managed as a chronic illness, but the disease can be quite serious and even life-threatening for others. The disease does not follow a common path, so lupus patients often suffer unpredictable bouts (flares) followed by periods of remission—even with treatment.

    How Lupus Is Treated

    Coping

    Lupus can bring all sorts of physical and emotional challenges, especially if you're newly diagnosed. Learning to cope with your disease takes time and practice, and includes things like educating yourself and your loved ones about your disease, taking care of yourself by getting enough rest and eating well, learning how to manage your flares, and getting support.

    Could It Be Lupus? Signs and Symptoms
    Was this page helpful?
    Article Sources