What is Lupus?

Lupus is a chronic autoimmune disease that causes the body to attack its own tissues and organs. It can affect any part of the body causing widespread pain and inflammation. Common symptoms include fatigue, fever, and joint pain. Lupus affects each patient differently, and it may go into periods of flares and remissions. Although there is no cure for lupus, there are many treatments available to help decrease disease activity. It is important to keep in mind that following medication regimens, maintaining a tolerable amount of daily activity, and making healthy lifestyle choices can help to decrease disease progression. The Michigan Lupus Foundation can provide resources for patients, caregivers, and healthcare providers.

Systemic Lupus Erythematosus (SLE)

SLE is the most common type of lupus. Systemic lupus is so-named because it affects many different organ systems in the body. It is usually more severe than discoid lupus and can affect almost any organ in the body. It causes chronic inflammation, especially of the kidneys, joints, skin, and in some patients it may affect the lungs, heart, blood, and nervous system. Sometimes patients living with lupus may develop a malar rash, often referred to as a butterfly rash. This rash extends from one cheek over the nose to the other cheek.

Discoid Lupus (common form of Cutaneous Lupus)

Discoid lupus is a form of lupus that only affects the skin. It causes rashes, and is characterized by persistent flushing of the cheeks or disk-like lesions that appear on the face, neck, scalp and other areas. The rash is usually scaly red, not itchy, and may be exacerbated by exposure to UV sunlight or fluorescent lights. If the rash is not adequately treated, permanent scars may develop. Although this type of lupus does not affect any of the internal body organs, about 1 in 10 people living with discoid lupus will develop systemic lupus.

Drug-Induced Lupus

Drug-Induced Lupus (DIL) may occur after taking certain prescription medications. It usually resolves within 6 months after discontinuing the causative medication. This type of lupus rarely occurs, and only about 4% of individuals who develop DIL will have lupus antibodies. The most common medications that cause DIL include hydralazine, procainamide, quinidine, isoniazid, and phenytoin.

Neonatal Lupus

Neonatal lupus is a rare autoimmune disorder that affects infants at birth. It is developed from mothers who have lupus and/or anti-Ro/SSA antibodies. Infants will often develop a red rash, may develop liver problems and low blood platelets. Some infants also develop a serious heart condition known as congenital heart block. The symptoms associated with neonatal lupus, with the exception of congenital heart block, usually resolve themselves within the first several months of life.

There are many symptoms of lupus, and they vary between each patient. Lupus is often referred to as “the great imitator” because it mimics many other diseases including cancer, infections and other autoimmune diseases. It takes an average of 2 years for patients to be properly diagnosed with lupus, and symptoms may range from being mild to life-threatening. 

Symptoms

  • Fever and fatigue
  • Painful, achy or swollen joints
  • Loss of appetite or changes in weight
  • Butterfly shaped rash across the face
  • Other skin rashes
  • Hair loss
  • Sun or light sensitivity
  • Headaches or seizures
  • Chest pain
  • Muscle pain
  • Mouth or nose ulcers
  • Raynaud’s disease
  • Abnormal blood clotting
  • Swelling in feet or hands
  • Kidney issues

Lupus is a complex disease that is caused by a combination of genetic, environmental and hormonal factors. There is a growing interest in lupus research to develop new treatment options with the hope of finding a cure. It will be the continuing research that provides pivotal pieces of information to continue understanding this mysterious disease.

Hormones

Since lupus occurs more frequently in women than in men, researchers have looked at the relationship between estrogen and lupus. Many women have more lupus symptoms before menstrual periods and/or during pregnancy when estrogen production is high. However, studies have not yet proven that lupus is caused or enhanced by estrogen. It also has not been shown that women with lupus who take estrogen, in either birth control pills or as postmenopausal therapy, have any increase in disease activity.

Genetics

We know genetics plays a role in lupus because certain genes have been identified to contribute to its development. It appears to run in certain families but there are cases where twins who are raised in the same environment and have the same inherited features, yet only one developed lupus. Lupus can develop in families without any history of the disease, but it is likely other autoimmune disease exist within the family line. Certain ethnic groups (African, Asian, Hispanic/Latino, Native American, Native Hawaiian, or Pacific Island descent) have a greater risk of developing lupus, which may be related to the genes they have in common.

Environment

Most researchers today believe that an environmental agent, such as a virus or chemical, acts as a trigger to the disease when a genetically susceptible individual comes in contact with it. Researchers may have not identified a specific environmental agent as of yet but the hypothesis remains likely. While the environmental elements that can trigger lupus aren’t fully known, the most commonly cited are ultraviolet light (UVB in particular); infections (including the effects of the Epstein-Barr virus), and exposure to silica dust in agricultural or industrial settings. Other examples of environmental triggers include:

  • Exposure to medications that increase sun sensitivity
  • Infections such as a cold or viral illness
  • UV rays from sunlight or fluorescent lights
  • Exhaustion or injury
  • Emotional stress or other stressors

For a lupus diagnosis a physician or a rheumatologist will identify current symptoms, conduct blood tests, and review your medical or family history. A thorough medical examination is essential for an accurate diagnosis because there is no single laboratory test that can diagnose lupus. The gold standard used to diagnose lupus is the American College of Rheumatology (ACR) Criteria. If at least 4 of the 11 criteria develop at one time or individually over any period of observation then a patient may likely have SLE.

Once doctors have identified a proper diagnosis, it is important to learn how to manage your health and lifestyle. Physician’s recommendations will help you with the “next steps” in managing lupus. The good news is now you can start treating the disease and not just reacting to the symptoms.

Tests

  • Complete blood counts (CBC)
  • Blood tests for inflammatory markers
  • Urine samples to be analyzed for protein content
  • Other diagnostic tools

ACR Criteria

  • Malar rash
  • Discoid rash
  • Photosensitivity
  • Oral ulcers
  • Arthritis
  • Serositis
  • Renal disorder
  • Neurological disorder (seizures or psychosis)
  • Hematologic disorder
  • Immunologic disorder (autoantibodies such as anti-DNA)
  • Abnormal ANA titer

Although lupus can be well controlled, other medical conditions may be associated with the onset and duration of the disease. It is important to note that these conditions do not occur in everyone, but lupus patients have an increased risk of co-occurring conditions and other autoimmune diseases.

Autoimmune Diseases

  • Sjogren’s syndrome
  • Antiphospholipid syndrome
  • Scleroderma
  • Fibromyalgia
  • Raynaud’s disease
  • Thyroid problems
  • Celiac’s disease

Co-occuring Conditions

  • Lupus nephritis
  • Pericarditis
  • Myocarditis
  • Pleuritis
  • Vasculitis
  • Central Nervous System conditions
  • Osteonecrosis
  • Gastrointestinal issues

Being diagnosed with lupus can be challenging. If you have been diagnosed with lupus, you may feel a range of emotions from fear to frustration. Some things you can do for yourself if you are newly diagnosed is become educated about the disease. The more you know about lupus, the better you will feel about talking to healthcare providers regarding your care and treatment options. You can also gather support from friends and family. By teaching those around you about lupus they can know what to expect and learn to help out when you are not feeling well. It is beneficial to connect with others who have lupus. The support that can be offered from those who also have lupus reminds you that you are not alone, and that they are facing many of the same obstacles. You can connect with others with lupus through local support groups or online message boards. We offer support groups around Michigan, and always welcome new lupus patients or caregivers. Our support groups can be found here on our webpage, or you can call our office for more information about how we can help you or a loved one who has been diagnosed with lupus.

Who Gets Lupus?

More people have lupus than cerebral palsy, multiple sclerosis, sickle-cell anemia, and cystic fibrosis combined. It is estimated that 1.5 to 2 million people in the United States suffer from this disease. Lupus primarily affects young women between the ages of 15-45 years old, and it occurs more frequently in women of African American, Hispanic, or Asian descent. The cause of the disease is not well understood. Both genetics and environmental factors play a role in the development of the disease, and first-degree relatives of lupus patients have an increased risk of developing lupus within their lifetime.

References: Larry Silverman, M.D. F.A.C.P.; National Institute of Health; National Institute of Arthritis & Musculoskeletal & Skin Diseases; Johns Hopkins Lupus Center; Mayo Clinic; Lupus Research Institute