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Murad Yakushev
Murad Yakushev

Exactly What Is Lupus

Lupus is a widespread and chronic (lifelong) autoimmune disease that, for unknown reasons, causes the immune system to attack the body’s own tissue and organs, including the joints, kidneys, heart, lungs, brain, blood, or skin. The immune system normally protects the body against viruses, bacteria, and other foreign materials. In an autoimmune disease like lupus, the immune system loses its ability to tell the difference between foreign substances and its own cells and tissue. The immune system then makes antibodies directed against “self.”

Exactly what is Lupus

Symptoms of lupus often mimicother less serious illnesses. They can range from mild to life threatening.Lupus can go into periods where symptoms are not present, called remission.Although lupus can affect any part of the body, most people experience symptomsin only a few organs. Other common symptoms include: achy joints (arthralgia),frequent fevers of more than 100 degrees Fahrenheit, arthritis (swollenjoints), prolonged or extreme fatigue, skin rashes and anemia.

There are several forms oflupus: discoid, systemic, drug-induced, and overlap syndrome or mixedconnective tissue disease. Discoid (cutaneous) lupus is always limited to theskin and is identified by a rash that may appear on the face, neck and scalp.Discoid lupus accounts for approximately 10 percent of all cases. Systemiclupus is usually more severe than discoid lupus, and can affect the skin,joints, and almost any organ or system of the body, including the lungs,kidneys, heart or brain. Approximately 70 percent of lupus cases are systemic.In about half of these cases, a major organ will be affected.

Drug-induced lupus occurs afterthe use of certain prescribed drugs. The symptoms of drug-induced lupus aresimilar to systemic lupus. The drugs most commonly connected with drug-inducedlupus are hydralazine (used to treat high blood pressure or hypertension) andprocainamide (used to treat irregular heart rhythms). The percentage ofindividuals using these drugs who develop drug-induced lupus is extremelysmall, and the symptoms usually fade when the medications are discontinued. Inapproximately 10 percent of all lupus cases, individuals will have symptoms andsigns of more than one connective tissue disease, including lupus. A physicianmay use the term “overlap syndrome” or “mixed connective tissuedisease” to describe the illness.

It is difficult to determine anexact number of lupus cases, and estimates vary widely. Based on the results ofseveral nationwide telephone surveys, the Lupus Foundation of America estimatesthat approximately 1.4 million Americans have a form of the disease.

Despite the fact that lupus canaffect men and women of all ages, lupus occurs 10 to 15 times more frequentlyamong adult females than adult males. It develops most often between ages 15and 44 and is two-to-three times more common among African Americans,Hispanics, Asians, and Native Americans. Only 10 percent of people with lupuswill have a close relative (parent or sibling) who already has lupus or maydevelop lupus and only about 5 percent of the children born to individuals withlupus will develop the illness.

Lupus is NOT infectious, rare orcancerous. Researchers do not know what causes lupus. While scientists believethere is a genetic predisposition to the disease, it is known thatenvironmental factors also play a role in triggering the disease. Some of thefactors that may trigger lupus include infections, antibiotics, ultravioletlight, extreme stress, certain drugs, and hormones. Hormonal factors mayexplain why lupus occurs more frequently in females than in males.

Because many lupus symptomsmimic other illnesses, are sometimes vague, and may come and go, lupus can bedifficult to diagnose. Diagnosis is usually made by a careful review of aperson’s entire medical history and a physical examination, coupled withan analysis of the results obtained in routine laboratory tests and somespecialized tests related to immune status. Currently, there is no singlelaboratory test that can determine whether a person has lupus or not. It maytake months or even years for doctors to piece together evolving symptoms andaccurately diagnose lupus.

For the vast majority of peoplewith lupus, effective treatment can minimize symptoms, reduce inflammation, andmaintain normal bodily functions. Treatment approaches are based on thespecific needs and symptoms of each person. Because the characteristics andcourse of lupus may vary significantly among individuals, it is important toemphasize that a thorough medical evaluation and ongoing medical supervisionare essential to ensure proper diagnosis and treatment. Medications are oftenprescribed for people with lupus, depending on which organs are involved andthe severity of involvement. Commonly prescribed medications include:nonsteroidal anti-inflammatory drugs (NSAIDs), acetaminophen, corticosteroids,antimalarials, and immunomodulating drugs.

Increased professional awarenessand improved diagnostic techniques and evaluation methods are contributing tothe early diagnosis and treatment of lupus. With current methods of therapy80-90 percent of people with lupus can look forward to a normal lifespan.

Duarte-García A, Hocaoglu M, Valenzuela-Almada M, et al. Rising incidence and prevalence of systemic lupus erythematosus: a population-based study over four decades. Ann Rheum Dis. 2022;annrheumdis-2022-222276. doi:10.1136/annrheumdis-2022-222276

Somers EC, Marder W, Cagnoli P, et al. Population-based incidence and prevalence of systemic lupus erythematosus: the Michigan Lupus Epidemiology and Surveillance program. Arthritis Rheumatol. 2014;66(2):369-378. doi:10.1002/art.38238

Parks CG, de Souza Espindola Santos A, Barbhaiya M, Costenbader KH. Understanding the role of environmental factors in the development of systemic lupus erythematosus. Best Pract Res Clin Rheumatol. 2017;31(3):306-320. doi:10.1016/j.berh.2017.09.005

Jorge AM, Lu N, Zhang Y, Rai SK, Choi HK. Unchanging premature mortality trends in systemic lupus erythematosus: a general population-based study (1999-2014). Rheumatology (Oxford). 2018;57(2):337-344. doi:10.1093/rheumatology/kex412

Cutaneous lupus erythematous (CLE or cutaneous lupus) is the skin form of lupus, an autoimmune disease. Cutaneous lupus can occur along with lupus in other parts of the body (like the kidneys or lungs, which is called systemic lupus erythematosus, or SLE), and sometimes the rash of cutaneous lupus occurs by itself.

Autoimmune diseases are a type of problem where the immune system (your white blood cells) start attacking your own body parts instead of fighting off bacteria, viruses and other infections. When the immune system attacks your skin there is inflammation and the rash of cutaneous lupus.

There are many different types of cutaneous lupus. Dermatologists can usually tell apart the different types by how the rash looks. Sometimes a biopsy of the skin is needed to be sure a rash is cutaneous lupus and to know exactly which type of cutaneous lupus it is. The rash of cutaneous lupus tends to be most pronounced on sun-exposed skin, like the face and cheeks.

Another common form of lupus is acute cutaneous lupus, which presents with a butterfly-shaped rash on the central cheeks and nose. This type of cutaneous lupus is commonly seen along with signs of lupus in other parts of the body.

The third major subtype of lupus is subacute cutaneous lupus, or SCLE. This type of lupus usually presents as red scaling rings on the chest, upper outer arms and upper back. Subacute cutaneous lupus is sometimes caused by a medication reaction.

Sometimes the type of rash (like the acute butterfly rash we just discussed) can give us an idea of how likely you are to also have systemic lupus. It is important for your doctor to ask you questions about how you are feeling and do some tests (like bloodwork and urine tests) to better answer this question

The bottom line is maybe yes and maybe no. Although most people who have systemic lupus will have a rash of cutaneous lupus at one point or another, many people with cutaneous lupus will never have systemic lupus. Fortunately for many people with cutaneous lupus, even if they have joint aches and fatigue, they will never develop lupus in other organs like their kidneys, lungs, brain or heart. However, people with cutaneous lupus should be screened by their doctor on a regular basis for any signs or symptoms or systemic lupus, because there is a small risk of progressing onto systemic lupus.

The cause of cutaneous lupus (and systemic lupus for that matter!) is unknown at this time. There are some rare forms of cutaneous lupus that run in families, so there is probably a genetic link. Also women are more commonly affected than men, so hormones may be involved. Cutaneous lupus (and systemic lupus) flares after sun exposure, suggesting that ultraviolet light may be a disease trigger. Sometimes medications, including over the counter acid reflux medicines have been associated with a form cutaneous lupus.

Because sun avoidance and sunscreen are effective treatments for lupus, patients with lupus are at risk of becoming vitamin D deficient. Your doctor may test your vitamin D levels during the winter months and recommend supplementation if your levels are low.

Sun protection is a very important treatment for almost every type of lupus, cutaneous and systemic. For this reason, you should avoid sun exposure. You can do this by limiting your outdoor activities to early morning or late afternoon (prior to 10am and after 3pm), and wearing sun-protective clothing. You should wear a sunscreen on exposed skin daily and reapply every two hours as instructed on the sunscreen bottle. This sunscreen should have both UVB protection (reported as SPF) and UVA protection (called broad spectrum protection). Daily sunscreen is important because UVA rays can pass through clouds and windows (including most car windows).

According to the Lupus Foundation of America, approximately 1.5 million Americans and five million people globally have some form of lupus. While lupus can affect both men and women, about 90 percent of those with diagnosed lupus are women between the ages of 15 to 44. Even though this chronic autoimmune disease affects millions, significantly less than half of people are actually somewhat familiar with the disease.


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