Lupus

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Overview

Lupus is associated with multisystemic inflammation resulting from abnormal immunological function. Patients experience periodic flares of varying severity or instances in which no observable signs or symptoms are present. The four main types of lupus are neonatal and pediatric lupus erythematosus (NLE); discoid lupus erythematosus (DLE); drug-induced lupus (DIL); and systemic lupus erythematosus (SLE).

As a rare form of lupus observed in newborns, NLE is thought to result from maternal autoantibodies passing through the placenta. However, of those pediatric patients who have positive maternal autoantibodies, only about 1% develop NLE. Common clinical presentations involve the heart, liver, and skin. Significant morbidity and mortality, along with cardiac manifestations, have been noted; however, in most NLE patients with other organ involvement (e.g. skin, liver, and blood), signs and symptoms sometimes resolve spontaneously within  to 6 months.1

DLE is manifested as a chronic scarring and atrophic photosensitive dermatosis, which may progress to SLE or may occur in patients with SLE. The cause is thought to be genetic, with the highest prevalence in women, African-Americans, and persons between 20 and 40 years of age. The diagnosis is frequently made by biopsy of a rash on the scalp, face, neck, or arms. Chemical and physical sunblocks, topical corticosteroids, or antimalarial agents are commonly used to prevent disease flares and to manage the clinical manifestations associated with DLE.2

DIL occurs after exposure to a medication, causing an autoimmune response. Various organ systems may be affected, but clinical manifestations usually subside upon discontinuation of the responsible agent

SLE is the most common type of lupus and is therefore the focus of this review. SLE is commonly referred to simply as “lupus,” but it is differentiated from other types by its multi-organ system effects. SLE is diagnosed in approximately 20 to 150 persons per 100,000 and is typically seen in females of child-bearing age; however, it may affect male or female patients at any age.4–6 SLE is more commonly observed in African-Americans, Asians, Hispanics, and Native Americans.

Arriving at the correct diagnosis of lupus is a challenge, considering the multitude of clinical presentations observed. The disease can affect the kidneys, lungs, skin, nervous system, and musculoskeletal system as well as other organs of the body. If SLE is suspected, patients’ subjective complaints, as well as laboratory abnormalities and demographic characteristics, may help to pinpoint the diagnosis.

In recent decades, mortality rates attributed to SLE have declined as a result of earlier disease detection and advances in treatment. The average 10-year survival rate now exceeds 90%; three decades ago, the 10-year average survival rate was 76%. The most common causes of death are related to early active SLE include SLE-induced and immunosuppressant-induced infectious complications. A common cause of late mortality related to SLE is an accelerated atherosclerosis that is associated with either the disease or the treatment.9


Symptoms

Fatigue.

Fever.

Joint pain, stiffness and swelling.

Butterfly-shaped rash on the face that covers the cheeks and bridge of the nose or rashes elsewhere on the body.

Skin lesions that appear or worsen with sun exposure.

Fingers and toes that turn white or blue when exposed to cold or during stressful periods.

Causes

Lupus is an autoimmune disease. That means your immune system mistakenly turns against and attacks your own tissues.

Normally, your immune system protects your body against foreign invaders like bacteria and viruses. When it detects these germs, it attacks with a combination of immune cells and specific proteins called antibodies. In an autoimmune disease, your immune system mistakes your own tissues — such as your skin, joints, or heart — as foreign and attacks them.

Experts think a few different factors trigger this immune system assault, including:

Your genes. Lupus sometimes runs in families. Researchers have found more than 50 genes that they believe are linked to the condition. Although most of these genes are unlikely to cause lupus alone, they may make you more vulnerable to developing lupus if you’re exposed to other risk factors.

Your environment.If you have lupus, certain factors around you can set off your symptoms. This includes ultraviolet light from the sun, infections such as the Epstein-Barr virus, and exposure to certain chemicals or medicationsTrusted Source.

Your hormones.Because lupus is much more common in women, researchers suspect female hormones may have something to do with the disease. Women do tend to have worse symptoms before their menstrual periods, when estrogen levels rise. However, the link between estrogen and lupus has not been provenTrusted Source.

Risk factors

Your sex. Women are more likely to develop lupus.

Your age. Symptoms and diagnosis occur most often between the ages of 15 and 44. ...

Your race/ethnicity. In the United States, lupus is more common in people of color than in the Caucasian population. ...

Your family history.

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Complications

Skin scarring.

Joint deformities.

Kidney failure.

Stroke.

Heart attack.

Pregnancy complications.

Hip destruction (also called avascular necrosis)

Cataracts.

Prevention

You can’t necessarily prevent lupus, but you can avoid the factors that trigger your symptoms. For example, you can:

Limit your time in direct sunlight if sun exposure causes a rash. You should always wear a sunscreen with an SPF of 70 or higher that blocks both UVA and UVB rays.

Try to avoid medications, if feasible, that make you even more sensitive to the sun.Trusted Source This includes the antibiotics minocycline (Minocin) and trimethoprim-sulfamethoxazole (Bactrim), and diuretics such as furosemide (Lasix) or hydrochlorothiazide.

Develop stress management techniques. Meditate, practice yoga, or get massages — whatever helps calm your mind.

Stay away from people who are sick with colds and other infections.

Get enough sleep. Go to bed early enough each night to guarantee yourself seven to nine hours of rest.