Lupus Nephritis

Last Updated: May 10, 2024

Medically reviewed by NKF Patient Education Team

Lupus nephritis is a disease that affects the kidneys, making it difficult for them to clean blood well.

About Lupus Nephritis

Lupus nephritis (LN) is an inflammation of the kidneys caused by systemic lupus erythematosus, often called lupus. When you have LN, your kidneys are not doing a good job removing waste from your blood or controlling the amount of fluids in your body.

Watch this short, animated video series, you will learn about:

  • Lupus nephritis 
  • How Lupus nephritis is diagnosed
  • Treatment options
  • How to manage Lupus nephritis

Signs and Symptoms

In its early stages, LN has very few signs of anything wrong. Some people have no specific symptoms. Kidney problems can start around the same time lupus symptoms appear and may include:

  • Swelling in the hands, face, feet, belly, or around the eyes
  • Weight changes – usually weight gain, but sometimes weight loss
  • Feeling very tired
  • Brown urine caused by blood
  • Foamy urine caused by protein
  • Urinating (peeing) less often than normal
  • High blood pressure

A key feature of ln is proteinuria, which is HIGH levels of protein in the urine.

Causes

No one knows why some people with lupus develop LN. Your family background and ancestry, medical conditions, and environmental factors such as exposure to chemicals or pollutants may all play a role in causing the disease.

In adults with systemic lupus erythematosus:

  • About 1 in 3 have LN at the time of diagnosis
  • Up to 5 out of 10 will develop LN over time

Complications

Without diagnosis and treatment, LN usually gets worse over time, which can lead to kidney failure. This serious condition occurs when almost all (over 90%) of your kidney function is lost. If your kidneys fail, you will need to have dialysis or a kidney transplant to survive.

Up to 3 out of 10 people with lupus nephritis will develop kidney failure within 15 years of diagnosis.

Diagnosis

Healthcare providers diagnose LN by looking at your symptoms and medical history to see if your kidneys have been affected. They then use certain tests to confirm this.

Lupus Nephritis

Looking for more info about lupus nephritis?

Join our lupus community to learn more about lupus nephritis, listen to patient-provider interactions and much more.

Tests

To accurately diagnose LN, your healthcare provider(s) must follow several steps, including a physical examination, getting your medical history, and doing special tests. These tests include:

  • Urine test to check for protein and blood. If protein is found, a urine albumin-creatinine ratio (uACR) test may also be needed
  • Blood tests are used to check:
    • eGFR (estimated glomerular filtration rate) to show how well your kidneys are filtering wastes
    • Anti-phospholipids, which are specific antibodies (proteins made by your immune system to help fight off infections) that can increase the risk of blood clots and are associated with a subset of lupus that can affect various organs, including the kidneys
    • Anti-nuclear antibodies (ANA)
      • Shows that a person’s immune system is producing antibodies against their own cells (a hallmark of autoimmune diseases like lupus)
      • Done at least once to confirm a diagnosis of lupus
  • Kidney biopsy to look at a tiny piece of the kidney under a microscope

Nephrologists (kidney doctors) confirm a diagnosis of LN with a kidney biopsy.

LN Classifications

Once a diagnosis of LN is confirmed, it is grouped into 5 classes based on the type, location, and amount of damage within the kidney cells, as seen from the kidney biopsy. Each class describes how the disease can affect the kidneys, the risk of getting worse, and a different long-term outlook.

While there are five different classes of LN, the numbers are not used to rank the severity of the kidney damage. For example, someone with class 5 LN may not have more kidney damage than another person with class 3 LN. Your nephrologist uses the class number to determine the most appropriate treatment for your type of LN.

Treatment

Most medications for LN work to prevent the overactive immune system from attacking the kidneys. Other medicines are used to keep your kidneys healthy. Treatments differ depending on the class of the disease.

The goals of treatment for lupus nephritis are to:

  • Reduce inflammation in your kidneys
  • Decrease immune system activity
  • Block your body’s immune cells from attacking the kidneys directly or making antibodies that attack the kidneys

No matter the class, LN is usually treated with Plaquenil® (hydroxychloroquine), which helps to keep your immune system activity well-balanced. In addition, blood pressure medications, such as angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs), are often recommended, even if you don’t have high blood pressure.

Depending on your class/type of LN, your nephrologist will likely recommend a treatment plan that is right for you and will help keep your kidneys working well. These treatments can range from simple monitoring (watching and testing) to stronger medicines that help manage any symptoms and prevent more harm to your kidneys.

Common Treatment Plans According to LN Class

Classes I & II

Initial (First) Phase of Treatment

  • Usually, no treatment is needed unless there is a large amount of protein in the urine (pee)
  • If protein is in your pee, medicines like prednisone (a type of steroid) may be used

Follow-up Treatments

  • Regularly check kidney function and the amount of protein in urine (pee)

Special Notes

  • Treatments may change based on individual symptoms and how well your kidneys are working

Active Classes III, IV & V

Initial (First) Phase of Treatment

  • Usually, the first phase (3 to 12 months) of treatment includes steroids and one or more medications like these:
    • Mycophenolate mofetil (MMF)
    • Mycophenolic acid (MPA)
    • Cyclophosphamide
    • Belimumab (Benlysta)
    • Calcineurin inhibitors, such as voclosporin (Lupkynis), tacrolimus, or cyclosporine
    • Rituximab (Class V only)

Follow-up Treatments

  • After the first phase, MMF or MPA is usually continued
  • The best follow-up treatment recommended for you will depend on what worked best during the first part of treatment.

Special Notes

  • Azathioprine is often recommended for people who may become pregnant because it is safer to use during pregnancy

Stable Classes III, IV, & V

Initial (First) Phase of Treatment

  • Usually, the first phase of treatment includes hydroxychloroquine and/or blood pressure medications
  • MMF or MPA may be recommended

Follow-up Treatments

  • Regularly check if LN is stable
  • More immune system-suppressing medications may be needed if symptoms return

Special Notes

  • Treatment times can vary depending on how well LN is controlled and whether symptoms increase

Relapse (Symptoms return or get worse)

Initial (First) Phase of Treatment

  • Treat with the same medications that worked before

Follow-up Treatments

  • Quickly address any signs that LN symptoms may be getting worse to prevent kidney damage.

Special Notes

  • Check-in with your healthcare provider may have your treatments changed as needed

Special Considerations

Living with LN means paying extra attention to your health. It’s important to know how to handle everyday issues, from what medicine to take to changes in your lifestyle. Here are some key things to remember to manage your disease best.

Lifestyle and Wellness

People with LN need to make smart lifestyle choices.  Remember, it’s never too late to make the changes to improve your overall health and wellbeing:

  • Don’t smoke: Smoking can make your condition worse, so it’s best to avoid it.
  • Keep active: Try to stay active with exercises that you enjoy and that are safe for you. Talk to your healthcare provider about how to exercise safely.
  • Eat well: A nutritious and healthy diet is key. Eat plenty of fruits, vegetables, and whole grains. And cut back on highly processed foods, salty snacks, and sugary drinks.
  • Be careful with over-the-counter (OTC) medicines and herbal/vitamin supplements: Talk to your doctor before you take any new medicines, especially OTC pain relievers like ibuprofen (Motrin or Advil) or naproxen (Aleve), also known as nonsteroidal anti-inflammatory drugs (NSAIDs) that can harm your kidneys.
  • Medicines like acetaminophen (Tylenol) may be safer at recommended doses, depending on your other health conditions. Also, some herbal and vitamin supplements may not be good for you. Always check with your healthcare provider before using any new medications or supplements.
  • Alcohol use: Drinking alcohol should be limited to moderate use only.
  • Stay up-to-date on vaccines: Protect yourself from infections by getting vaccinations when they are due, especially if your LN treatment has weakened your immune system.
  • Tests for bone health and anemia (low iron) recommended: Your doctor may suggest tests to check your bones and blood to catch any problems early.
  • Watch your sun exposure: Since people with LN are at a higher risk for skin cancer, you should wear sunscreen whenever you’re outside.
  • Consider using birth control: Pregnancy may be risky, so it’s important to talk to your healthcare provider when you are thinking about your reproductive plans.
  • Look after your mental health: Don’t hesitate to get help if you’re feeling stressed, depressed, or anxious. Talking to someone about your feelings can really help.

Take a quiz to test your knowledge!

Take this quick quiz to see what you know about LN

Preparing for Your Appointment

Getting ready for your doctor’s appointment is an important step in addressing how best to manage your LN. Here’s how you can prepare:

  1. List your symptoms: Write down any health changes or problems you’ve noticed, even if they seem small or unrelated to your kidneys.
  2. Medication list: Write down all your medicines, including OTC drugs, vitamins, herbal products, and supplements.
  3. Bring your medical history: Have information about your past health conditions and treatments ready.
  4. Questions for the doctor: Think about what you want to ask your health care provider and write these questions down.
  5. Bring someone: If possible, have a family member or friend come with you to your appointment. They can help you remember the information you hear and support you.

Questions to Ask

You may want to ask your healthcare provider some questions about LN. These questions help you understand more about your condition and what to expect:

  1. What stage of LN do I have, and what does that mean?
    • This helps you know how serious your condition is and what treatment might be like.
  2. What symptoms should I watch out for?
    • Knowing what symptoms to look for helps you take action early if your condition worsens.
  3. What are my treatment options, and what are the side effects?
    • Understanding your choices helps you decide which treatment to try and what side effects to be prepared for.
  4. How will this affect my daily life?
    • This question helps you plan for any changes you might need to make in your routine or work.
  5. What is the long-term outlook for my health?
    • Knowing what to expect in the future can help you plan and feel more prepared.
  6. How often should I come in for check-ups and tests?
    • Regular check-ups can help keep track of your condition and catch any changes early.
  7. Should I see any other doctors or specialists?
    • Sometimes, additional specialists can help you to manage your LN best.
  8. Are there changes I should make to my diet or lifestyle?
    • Small changes in your daily habits can greatly affect how you feel.
  9. Are there any signs I should watch for that mean I need to call you?
    • It’s important to know when you should get help right away.
  10. Can you recommend any resources for support or more information?
    • Getting more information and support can help you deal with your condition better.

How helpful was this content?

© 2024 National Kidney Foundation, Inc. This material does not constitute medical advice. It is intended for informational purposes only. Please consult a physician for specific treatment recommendations.