Systemic Lupus Erythematosus: The Great Imitator

butterfly or malar rash in SLE

Systemic Lupus Erythematosus (SLE) is an autoimmune disease that can affect any part of the body, hence the term systemic. Most commonly affected organs are the joints, skin, heart, lungs, kidneys, liver, blood vessels, and nervous system. In 2008, around 250.000 Americans have SLE. Women aged 20-30 years old are nine time more likely to be affected than men. African American and Asians are more commonly affected and the disease tend to be more severe in these ethnic groups.

Diagnosis

As in any autoimmune disorder, the immune system lost the ability to differentiate self and non self, producing auto antibody that attacks normal cells. Why our immune system turned against us is still unknown, but viral infection, sunlight and allergies may trigger the changes.

SLE is dubbed the great imitator because the symptoms can mimic other diseases, making the diagnosis a challenging one to make. The course of disease is completely unpredictable with flare-ups alternating with remissions. Severity ranges from mild to life threatening. Initially, most patients unspecific symptoms like fatigue, rashes, painful joints, and low grade fever.

Based on The American College of Rheumatology's criteria, a person is said to have SLE if he or she has at least four of the symptoms listed below.

the location of rashes in SLE

• Butterfly shaped rashes over the cheeks (malar rash), oval shaped rashes with raised borders (discoid rash), or rash over sun exposed skin
• Arthitis in two or more joints lasting more than a few weeks
• Chest pain when taking deep breaths, a sign of inflammation of the outer linings of the lung (pleuritis) or the heart (pericarditis)
• Kidney problems ranging from mild protein or blood in the urine to renal failure
• Nervous system disturbances like seizures, stroke, or delirium
• Sores in the mouth
• Abnormal lab results: 
• low red blood cells, low white blood cells, or low platelets
• positive auto antibodies like anti nuclear antibody (ANA) or anti double stranded DNA (anti-dsDNA), anti Smith (referred to as anti-Sm) or antiphospholipid antibodies, or a false positive test for syphilis

Treatment

Sadly there is no cure for SLE and its management can be rather complicated. The good news is that immunosuppresive therapy can greatly improve survival. Maintaining a good quality of life remains the greater challenge, however.

The medications commonly used for SLE includes:

• Nonsteroidal anti-inflammatory drugs (NSAIDs) to reduce joint pain and inflammation.
• Antimalarial drugs such as hydroxychloroquine help with fatigue, rashes, joint pain, and mouth sores.
• Immunosuppressants such as azathioprine, cyclophosphamide, cyclosporin are used in patients with more severe and wide-spread symptoms.
• Corticosteroids is also used in the more severe form of SLE.
• Biological agents such as rituximab and abatacept modulate the faulty immune system.

Rheumatoid Arthritis: More Than Just Aching Joints

Rheumatoid arthritis is more than just aching joints. Unlike osteoarthritis where the primary damage occurs in the cartilage, rheumatoid arthritis is an autoimmune disease where the main problem lies in faulty immune system. Chronic inflammation of the joints and its surrounding tissue are not the only problems because the disease can also cause problems with your lungs, heart, and eyes.

In 2007 about 1.5 million people in the United States had rheumatoid arthritis. It usually starts in the middle ages, between 25 to 55 years old. Incidence increases with age and women are more likely to get it than men.

As with other autoimmune diseases, the exact cause of rheumatoid arthritis is unknown. Genetic factor, infection, and hormones may play a role in the development of this condition. Unlike the wear-and-tear damage of osteoarthritis, rheumatoid arthritis causes inflammation on the synovium (the lining of your joints), causing damage to the cartilage that eventually result in bone erosion and joint deformity.

What are the symptoms?

Rheumatoid arthritis begins gradually, affecting small joints of the hands and feet at first, causing minor joint pain and stiffness that are accompanied by fatigue or low grade fever. Over time, symptoms often spread to the knees, ankles, elbows, hips and shoulders. Severity varies and symptoms may come and go. Periods of when the disease gets worse (flare-up), alternate with periods when the pain and swelling gets better (remission).

Joint symptoms may include:

• Joint pain that oftentimes affects the same joint on both sides of the body, accompanied with warmth and swelling.
• Morning stiffness that usually lasts for more than 1 hour. 
• Deformity of joints and the surrounding structure with limited range of motion

small joints of the hands and feet are usually affected first

Systemic symptoms may include:

• Fatigue
• Low grade fever
• Weight loss
• Anemia 
• Chest pain or shortness of breath 
• Dry eyes and mouth 
• Buring sensation and itchiness in both eyes
• Lumps under the skin, called rheumatic nodules.

What are the tests to support the diagnosis?

The two lab tests that often came up with abnormal results are rheumatoid factor test and Anti-CCP antibody test. Complete blood count any show anemia. C-reactive protein and erythrocyte sedimentatio rate may be elevated, a sign of chronic inflammation. Your physician may also preform joint imaging like x-ray, ultrasound, or MRI and synovial fluid analysis.

What are the complications?

Rheumatoid arthritis can affect almost every part of the body. Complications may include:

• Lung damage and scarring causing shortness of breath
• Inflammation of the heart muscle and the outer lining of the heart can progress to heart failure
• Hardening of the arteries which in turn leads to nerve, skin, and brain problems
• Spinal injury if the neck joints are affected
• Osteoporosis

What are the treatment options?

A treatment plan usually includes medications, physical therapy, exercise, and even surgery. Early and aggressive treatment can delay joint destruction, but the drugs can have significant side effects especially because they will be used for life-long.

The most important drugs that are used to treat rheumatoid arthritis are the disease modifying antireheumatic drugs (DMARDs) like methotrexate, leflunomide, and chloroquine. Anti-inflammatory drugs like aspirin, acetaminophen, nonsteroidal anti-inflammatory drugs (NSAIDs), and corticosteroids are used to reduce inflammation and joint pain. Biologic agents like rituximab, infliximab, etanercept, and tocilizumab may be used when the other drugs failed to give improvements. They work by suppressing the immune system and thus carry risks of infection.

The Basics of Autoimmune Diseases

The immune defense system is designed to defend our body from foreign substance and other harmful invaders. A healthy immune system has the ability to differentiate between self and non-self, that is knowing what to kill and what to leave alone. In autoimmune diseases, the immune system lost the ability to tell the difference and attacks normal cells by mistake.

There are more than 80 different types of autoimmune diseases affecting more than 23.5 million people in the United States, causing disability due to the chronic nature of these disorders. In general, women especially the ones of African American, Hispanics, and Native American racial background have higher risks.

What happens in autoimmune diseases?

An antigen is a substance that has the ability to trigger the immune system. It can be bacteria, virus, fungi, blood or tissue from another person or species, and toxin. When an antigen entered our body, white blood cells produces antibodies to destroy it. There is also a type of white blood cells called the T cells that keeps antibody production in check and make sure it doesn't attack what it's not supposed to. In autoimmune diseases, the antibody turned to attack our own cells and is called auto antibody.

What causes the changes in our immune system behavior resulting in autoimmune diseases is unknown. Some have proposed that the antigen itself triggers this conversion. A similarity between the bacteria or virus genes with our own may confuses our immune system.

An autoimmune disease can target any organ in our body. It can also affect more than one bodily systems at the same time. Blood vessels, red blood cells, glands, joints, muscles, connective tissues, and the skin are commonly affected. Some examples of autoimmune diseases are systemic lupus erythematosus, multiple sclerosis, type 1 diabetes, rheumatoid arthritis, and Graves' disease.

Symptoms

Diagnosing autoimmune diseases provides one the most difficult challenges for physician because the symptoms varies greatly, may come and go, and can mimic other diseases. For most people, the initial symptoms are quite vague: fatigue, muscle aches, low grade fever, and general unwell feeling. Over time, symptoms will develop depending on affected organs.

Management

It's difficult to cure autoimmune diseases because the exact cause is unknown in the first place, but symptoms can be reduced and complications prevented. Treatment plan depends on the specific diseases. Often times, if the symptoms gets worse, immunosuppressive drugs may be needed. These drugs weakens your immune system and infection can be a problem for people who uses them for long terms.