Lupus is a chronic autoimmune disease that causes inflammation in various parts of the body. The immune system, which normally protects the body from infection, begins attacking the body’s own tissues and organs instead. The most common symptoms of lupus include fatigue, joint pain, rash, and fever. Lupus can also affect many parts of the body, including the skin, kidneys, heart, lungs, and brain. There is no single test to diagnose lupus, and it may take months or even years for symptoms to develop enough to prompt a doctor to suspect lupus. About 1.5 million Americans have lupus, and 16,000 new cases are reported each year, according to the Lupus Foundation of America.
What causes lupus?
The exact causes of lupus are unknown, but research suggests that genetics, hormones, and environmental factors all play a role.
Genetics
Lupus tends to run in families. Someone with a family member who has lupus has an increased risk of developing the disease. The genes you inherit from your parents may make you more susceptible to lupus. However, most people with lupus do not have a family history of the disease. Even identical twins may have different rates of developing lupus.
Hormones
Because lupus is more common in women, especially during childbearing years, hormones may play a role. Estrogen likely contributes to the development of lupus. Flares of lupus symptoms may also occur more often just before menstrual periods when estrogen levels are highest.
Environmental factors
Sunlight: Exposure to UV rays from the sun or artificial lights causes a lupus rash to appear or worsen on sun-exposed skin. UV light is thought to trigger increased activity in the immune system that causes skin rashes in people with lupus.
Infections: An infection, such as the Epstein-Barr virus that causes mono, can be a trigger for lupus in some people. The immune system responds to the viral infection, but then has trouble shutting down, leading to autoimmunity.
Medications: Certain prescription drugs, such as hydralazine (used to treat high blood pressure) and procainamide (for irregular heart rhythms) can induce lupus in some people. These drug-induced cases usually resolve once the medication is stopped.
Who gets lupus?
About 90% of people diagnosed with lupus are women between the ages of 15 and 45. However, men and people of all ages, including thousands of children, can get lupus too. Some key facts about who develops lupus:
– African Americans are three times more likely to be diagnosed with lupus than Caucasians. The disease is also more severe in African Americans.
– Hispanics, Asians, and Native Americans are more susceptible to lupus than Caucasians.
– People who have a family member with lupus have an increased risk, but the majority of people with lupus do not have a family history of the disease.
Table 1. Lupus rates by gender and ethnicity
| Group | Rate of lupus cases |
|---|---|
| Caucasian women | 1 in 2,000 |
| African American women | 1 in 300 |
| Caucasian men | 1 in 15,000 |
| African American men | 1 in 5,000 |
Common symptoms of lupus
Most people with lupus experience episodes of illness called flares, which alternate with periods of remission where they have minimal symptoms. The signs and symptoms of lupus vary greatly from person to person. Some of the most common include:
Fatigue and fever
Extreme fatigue and tiredness are among the most prevalent symptoms, occurring in over 80% of people with lupus. Low-grade fever may accompany the fatigue.
Joint pain
Joint pain, stiffness, and swelling are common lupus symptoms. The small joints of the hands, wrists, and feet are often affected. About 90% of people with lupus experience joint pain and swelling during flares.
Butterfly rash
A butterfly-shaped rash across the cheeks and bridge of the nose affects about half of people with lupus. It usually appears after exposure to sunlight. The rash may be flat or raised.
Raynaud’s phenomenon
Raynaud’s causes fingers and toes to turn white or blue when exposed to cold temperatures. The affected extremities feel numb, prickly, and painful.
Hair loss
Many lupus patients experience mild to severe hair loss and thinning due to the autoimmune attack on hair follicles. Hair may fall out in clumps.
Chest pain
Some people have sharp chest pain due to inflammation of the lining around the lungs (pleuritis) or heart (pericarditis). Lupus can also increase risk of coronary artery disease.
Kidney problems
Inflammation of the kidneys (lupus nephritis) affects about half of those with lupus. It can lead to blood, protein, or casts in the urine and sometimes kidney failure.
Diagnosing lupus
There is no single test to definitively diagnose lupus. Doctors diagnose lupus by examining symptoms, conducting lab tests, and ruling out other conditions. Steps in the diagnosis may include:
Physical exam
Checking for rashes, swollen joints, and other symptoms
Blood and urine tests
Antinuclear antibody (ANA) test – positive in almost all lupus patients
Complete blood count – low white blood cell, red blood cell, platelet counts common in lupus
Erythrocyte sedimentation rate (ESR) test – measures inflammation levels
Kidney and liver function blood tests
Antibodies related to lupus and inflammation
Urine protein levels increased with lupus nephritis
Skin biopsy
Examining skin samples under a microscope can help confirm discoid lupus
Imaging tests
Chest x-rays, echocardiograms, MRIs to assess heart, lungs, and brain
What are the treatment options for lupus?
While there is no cure for lupus, treatments can help control symptoms, reduce inflammation and pain, prevent flares, and minimize organ damage. Lupus treatment depends on the type and severity of symptoms. Some common treatments include:
NSAIDs and pain relievers
Non-steroidal anti-inflammatory drugs (NSAIDs) like ibuprofen help relieve joint pain, swelling, and fever. Stronger prescription pain medicines may be used for severe pain.
Corticosteroids
Corticosteroid drugs like prednisone reduce swelling and inflammation. They suppress the overactive immune system in lupus. Oral or injected forms may be prescribed.
Antimalarial drugs
Hydroxychloroquine has an anti-inflammatory effect and also protects against damage from UV light. It can reduce likelihood of flares.
Immune-suppressing drugs
Mycophenolate, methotrexate, azathioprine, cyclosporine, and cyclophosphamide restrain the overactive immune system. They help control severe or treatment-resistant lupus.
Biologics
Newer drugs like belimumab (Benlysta) target specific cells and proteins involved in lupus autoimmunity. Biologics may be prescribed for severe lupus unresponsive to other medications.
Blood thinners
People with increased risk of blood clots may take low-dose aspirin or prescription anticoagulants. Blood thinners help prevent cardiovascular problems.
Plasmapheresis
This procedure filters the blood and separates out autoantibodies. It’s used to treat severe lupus cases involving the kidneys or central nervous system.
Besides medications, self-care for lupus includes protecting skin from sunlight, staying active, eating healthy, reducing stress, and seeing doctors regularly to monitor for organ involvement.
What triggers lupus flares?
It’s not always obvious what causes a lupus flare. But certain triggers are known to provoke flare-ups of symptoms in susceptible individuals. Monitoring and avoiding possible lupus flare triggers can help prevent the recurrence of symptoms. Some potential flare triggers include:
Sunlight
Sun exposure often causes rashes to appear or worsen in people with lupus. UV light invokes the skin lesions and joint pain of a flare.
Stress
High stress levels place strain on the body and seem to increase immune activity. Stress management helps prevent flares.
Infections
Any viral or bacterial infection can trigger an immune response that may spiral into a lupus flare. Infections should be treated promptly.
Certain medications
Some drugs like hydralazine, procainamide, isoniazid, and Dilantin may induce symptoms of drug-induced lupus. The lupus symptoms usually resolve after stopping the medication.
Hormones
Female sex hormones like estrogen can stimulate the immune system. Lupus flares coincide with estrogen level peaks before periods. Oral contraceptives and hormone therapy at menopause may also worsen lupus.
Fatigue
Low energy itself doesn’t cause flares, but the resulting lowered capacity to cope with stress may contribute. Rest is essential during flares.
Pregnancy
Lupus symptoms tend to improve during pregnancy, but risk of flares increases after giving birth. Pregnancy in lupus also carries increased risks of miscarriage, preeclampsia, and stillbirth.
Learning individual flare triggers allows people with lupus to take steps to prevent recurrence of symptoms. Keeping lupus well-controlled lessens the chance of a flare.
Can men get lupus?
Lupus occurs about 9 times more frequently in women than men. However, thousands of men are diagnosed with lupus each year. Male hormones like testosterone seem to play a protective role. But men are still susceptible to lupus, and tend to develop the disease at a later age than women.
Some key facts about lupus in men:
– An estimated 1 in 15,000 Caucasian men will develop lupus. The rate is higher in African American and Hispanic men.
– Men tend to develop lupus after age 50, while peak onset for women is 15-45.
– Men often have more severe lupus symptoms like kidney and brain involvement.
– The mortality rate of lupus is higher in men than women.
– Medications like corticosteroids that are used to treat lupus can have stronger side effects in men.
– Men with lupus have a higher rate of heart attacks and strokes.
– Rashes, arthritis, Raynaud’s phenomenon and sensitivity to sunlight also occur in men with lupus.
– Anti-male antibodies are found in some male lupus patients, showing the immune system is attacking the body’s own cells and tissues.
While women are more likely to get lupus, the disease should not be dismissed in men. Many men with lupus are misdiagnosed because doctors don’t consider the possibility of the disease. Increased awareness that lupus can affect both sexes is important for prompt diagnosis and treatment in men.
Is lupus fatal?
Advances in lupus treatment over the past several decades have greatly improved the outlook for people with lupus. In the 1950s, only about 50% of lupus patients survived four years after diagnosis. Today, over 90% of people with lupus survive at least 10 years, and many live relatively long lives with properly controlled symptoms.
However, lupus can sometimes be fatal. Complications like kidney failure, heart attacks, strokes, blood clots, sepsis and seizures increase the risk of death in some lupus patients.
The mortality rate of lupus varies depending on severity of symptoms. Approximately 5-30% of people with lupus die from complications of the disease. The risk of dying prematurely is 2 to 3 times higher for people with lupus than the general population.
Some factors associated with higher mortality rates include:
– Male sex
– African American race
– Later age at lupus diagnosis
– Kidney, brain or central nervous system involvement
– High rate of lupus flares
– Poor access to medical care
Early detection and attentive treatment of lupus decreases chances of organ damage and death. Close monitoring of medications, blood work, and any signs of complications are key to reducing mortality risk. With proactive management, most people with lupus can live a normal or nearly normal lifespan.
Conclusion
Lupus is a complex autoimmune condition with a variety of symptoms and health impacts. Inherited genes, hormones, and environmental triggers like infections and sunlight seem to interact to cause immune system dysfunction in lupus. The disease disproportionately affects women during their childbearing years, but can occur in people of any age or sex. Symptoms range from rashes and joint pain to potentially fatal kidney failure and strokes.
While lupus cannot yet be cured, medical science has made huge strides in treating and managing the disease. Avoiding known flare triggers and promptly treating complications are vital to reducing the chances of organ damage and death. With diligent monitoring and care, the majority of lupus patients today can live a normal lifespan despite the challenges of this unpredictable condition. Increased research funding and improved understanding of the fundamental causes of lupus offer hope for even more effective therapies and perhaps ultimately a cure.
