IVIG for Systemic Lupus Erythematosus

About 1.5 million Americans are living with lupus, with an estimated 16,000 new cases each year. It is a lifelong condition that can make your life challenging as it affects multiple body systems. Fortunately, advances in modern medicine have given us many treatment options, like corticosteroids or immunosuppressive drugs. However, if you do not respond to these medications, or in certain other cases, IVIG (intravenous immunoglobulin) can be beneficial to you. In this article, we’ll discuss everything you need to know about IVIG for systemic lupus erythematosus.

Systemic Lupus Erythematosus (SLE): Overview

Systemic lupus erythematosus (SLE) is the most common type of lupus. It is a long-term autoimmune disease where your body’s immune system mistakenly attacks its own tissues and organs. This can lead to damage to the skin, joints, kidneys, heart, lungs, brain, or blood cells [1].

Signs and symptoms of systemic lupus erythematosus (SLE) can vary widely from person to person and can include:

• Joint pain and swelling
• Extreme fatigue
• Swelling around the eyes and in the feet
• Skin rashes (commonly butterfly-shaped rashes on the cheeks/bridge of nose)
• Fever
• Sensitivity to sunlight or fluorescent light
• Chest pain
• Fingers/toes turning white or blue when exposed to cold or stress (Raynaud’s Syndrome)

Experts still do not fully understand the causes of SLE. However, many experts believe that a combination of factors is involved in causing SLE, including genetics, hormones (especially estrogen), environment (like sunlight or infection), and immune system dysfunction.

Unfortunately, there is no cure for SLE, but some treatment options are available to manage your condition. These include:

Corticosteroids: Corticosteroids are very powerful anti-inflammatory drugs that can quickly control your flare-ups (periods when the symptoms suddenly worsen or reappear after being stable). However, you can not use them long-term because of side effects.

Nonsteroidal anti-inflammatory drugs (NSAIDs): These drugs can help you with pain, inflammation, and fever.

Antimalarial drugs (like hydroxychloroquine): These drugs help manage skin rashes, joint pain, and fatigue in lupus by regulating immune system activity.

Immunosuppressants: Immunosuppressants can prevent organ damage in moderate to severe lupus cases by reducing immune system overactivity.

If these treatments are ineffective for you or cause significant side effects, your doctor may recommend intravenous immunoglobulin (IVIG).

How Does IVIG Help Patients With Systemic Lupus Erythematosus (SLE)?

IVIG (Intravenous Immunoglobulin) is a product containing human antibodies from thousands of healthy blood donors. It regulates and balances the immune system in various autoimmune diseases.

In systemic lupus erythematosus (SLE), your immune system mistakenly attacks your own tissues and organs. IVIG can help you with this condition by:

Blocking the activity of harmful autoantibodies: In SLE, autoantibodies attack the body’s own cells. IVIG contains normal antibodies that bind to these autoantibodies or their receptors. Then, IVIG blocks these autoantibodies from causing inflammation and harm.

Reducing inflammation: IVIG has anti-inflammatory effects. It can reduce inflammation caused by the overactive immune system. As a result, you can feel relief from pain, swelling, and organ stress.

Offering a rapid response to severe flares: In patients with severe or treatment-resistant SLE, IVIG can act quickly to control flare-ups (periods when the symptoms suddenly worsen or reappear after being stable). 

Neutralizing inflammatory molecules: Some molecules present in the body cause inflammation, such as cytokines. These molecules play a major role in SLE flare-ups and inflammation. IVIG binds to these molecules and neutralizes them.

Modifying immune cell function: In SLE, some immune cells, especially B cells and T cells, are overactive. IVIG alters the activity of these overactive immune cells. In this way, IVIG helps to calm the immune response.

Helping with platelet counts: In some lupus patients, IVIG improves low platelet counts (thrombocytopenia) by blocking the immune system from attacking them.

Another positive thing about IVIG is that it doesn’t broadly suppress the immune system like other standard treatments for SLE. Therefore, it can be a much safer option in certain cases, especially where infections are a concern.

Effectiveness of IVIG in Systemic Lupus Erythematosus (SLE) Found in Clinical Trials

IVIG is not considered a first-line treatment for systemic lupus erythematosus (SLE). However, in clinical trials, it has shown promise in severe cases and as an adjunct therapy in patients who do not respond to traditional medications. 

For instance, a 2006 study showed that IVIG is safe and beneficial for SLE patients who are resistant to conventional treatment [2]. Another 2005 study stated that high-dose IVIG is safe and beneficial [3]. A more recent study involving 31 patients also concluded that IVIG is effective in treating refractory SLE (not responding to standard treatment) [4].

In addition, IVIG can have certain other benefits for SLE patients who have the following conditions:

Severe Thrombocytopenia (Low Platelet Count): This is a serious condition in some SLE patients characterized by a very low level of platelets in the blood, which increases the risk of bleeding. Recent medical trials have shown that IVIG can rapidly increase platelet counts [5][6].

Neuropsychiatric Systemic Lupus Erythematosus (NPSLE): This is a condition where your SLE affects your brain and nervous system. It leads to symptoms that include seizures and psychosis. IVIG can benefit you by reducing inflammation in the brain. A study reported a 20-year-old woman with NPSLE who experienced complete resolution of symptoms (including depression, mania, and psychosis) within 48 hours of a 5-day IVIG treatment course [7].

Lupus Nephritis: Lupus nephritis is a serious condition where the kidneys become inflamed and damaged due to systemic lupus erythematosus (SLE). A 2022 systematic review of 2,328 articles found that IVIG has a 60-70% efficacy for lupus nephritis patients who didn’t respond well to first-line treatments [8].

FAQs

Here are some frequently asked questions about IVIG for systemic lupus erythematosus:

1. Is IVIG a long-term solution for systemic lupus erythematosus (SLE)?

Not really. IVIG is mainly used as a short-term therapy to manage flare-ups or when traditional medicines fail. Long-term use of IVIG is uncommon due to its cost and availability [9].

2. How quickly does IVIG work in systemic lupus erythematosus (SLE)?

IVIG usually works very quickly. You may experience improvements in your symptoms, like fatigue, joint pain, or platelet counts, within days. Achieving the full benefit may take a week or more. 

3. What is the cost of IVIG treatment for systemic lupus erythematosus?

IVIG is a very costly therapy. Your total cost will depend on factors such as weight, IVIG brand, country of treatment, healthcare facility, and insurance coverage. In the U.S., the price of IVIG ranges from $100 to over $350 or more per gram [10]. So, if you weigh 70 kg and receive a 2 g/kg dose (140g total), the cost of the drug alone may range between $14,000 to $49,000 per treatment cycle. 

4. What is the dosage of IVIG for systemic lupus erythematosus (SLE)?

The dosage of intravenous immunoglobulin (IVIG) for systemic lupus erythematosus (SLE) varies depending on several factors, including the patient’s overall health and the severity of the condition. 

5. Is IVIG Safe During Pregnancy and Breastfeeding?

Yes. You can take IVIG during pregnancy and breastfeeding because clinical trials have shown that it doesn’t harm you or your baby. Moreover, IVIG has been used safely in pregnant women with lupus to prevent complications like miscarriage, especially in those with antiphospholipid antibodies [12].