What is lupus nephritis:
Lupus nephritis or “lupus glomerulonephritis” is kidney inflammation caused by systemic lupus erythematosus (SLE). SLE is a chronic inflammatory autoimmune disease, where the body’s natural defense system (the immune system) attacks its own cells and organs by mistake. When this happens, inflammation and tissue damage can result in various parts of the body, including the kidneys. Some statistics vary, but it is believed that roughly 40-45% of people with SLE will develop lupus nephritis.
Your kidneys are the primary organs in the renal system, (system consisting of the kidney, ureters, and the urethra) which eliminates waste and controls fluids. The word “nephritis'' has its origins from late Latin and Greek words for nephros meaning "kidney" and itis meaning "inflammation." Lupus nephritis is a frequent complication in people who have systemic lupus erythematosus, making them unable to properly remove waste from their blood or control the amount of fluids in the body.
The kidneys, however, do more than just eliminate waste. The kidneys play an important role in regulating electrolytes, particularly calcium and potassium. They also help maintain normal blood pressure and secrete hormones that balance the body and the production of red blood cells. If the kidneys are not functioning properly, many parts of the body can be affected, causing an array of symptoms.
Common symptoms:
Lupus nephritis most often develops within five years after a lupus diagnosis. It is more common in women of color between the ages of 15-44, who tend to develop the disease earlier and experience more serious complications. LN can be a serious problem; however, the onset of symptoms may not always be obvious. This can be dangerous because people may not know they have the beginnings of lupus nephritis for a while. For many, the first noticeable symptom is edema - swelling of the legs, ankles, and feet. Sometimes even the face, eyelids, or hands can appear puffy.
Symptoms can also include blood in your urine, foamy urine, elevated blood pressure, and an increased need to urinate (especially at night). If you experience any of these symptoms, talk to your doctor as soon as possible. These could be signs that your lupus is active and attacking your kidneys. If lupus nephritis is not treated in an appropriate and timely manner, it can lead to permanent, irreversible kidney damage, including internal scarring of the kidneys.
Diagnostic Tests:
The first signs of lupus nephritis often appear not as symptoms but as abnormal urine lab tests. This may lead to further blood tests and even a kidney biopsy to assess disease progression. A kidney biopsy is when a nephrologist (kidney specialist) removes a very small sample of kidney tissue for analysis. It may sound scary, but this procedure is the most definitive test for diagnosing lupus nephritis and determining how severe (it is measured in class/stages) it is.
Some common tests are listed below:
After the tests are evaluated, the doctor will help find the right treatment protocol if there is a diagnosis of LN.
Treatment options:
The earlier appropriate treatment can be applied, the better the outcome. Patient compliance is also very important, because it could mean the difference between slowing down (even stopping) kidney disease and permanent damage.
The course of treatment will be determined by what stage/class kidney disease the LN has been classified as (see chart).
Common treatment options include medications that reduce kidney damage by lowering inflammation like corticosteroids, immunosuppressives (cyclosporine, tacrolimus, cyclophosphamide, azathioprine, mycophenolate, and rituximab), and blood thinners. In the last year, the FDA has approved two new therapies to treat LN, Benlysta and Lupkynis.
Benlysta (belimumab) is the first FDA approved drug in over 50 years that has been designed specifically for the treatment of systemic lupus and recently, lupus nephritis. It is a non-steroidal biologic therapy. That means it consists of genetically engineered proteins that use your own immune system to target specific parts that fuel inflammation, specifically BLys cells that can make your lupus active and cause lupus flares. It is given as a once-a-month infusion or as a weekly subcutaneous auto-injection. Benlysta has been proven to be the only drug to reduce the progression of renal damage over time.
Lupkynis™ is an immunosuppressant medication that was originally developed in British Columbia by the scientists at Aurinia Pharmaceuticals. This therapy was created to help with near and long-term outcomes in patients with lupus nephritis when added to standard medication protocols. It works (and can work fast) by inhibiting a specific protein that activates immune T-cells against the kidneys. By reducing the amount of chemical messengers traveling through an organ, voclosporin decreases inflammation in the kidneys and can halt further damage from occurring. It can also improve the filters of the kidneys (podocytes), allowing some patients to improve kidney function in as little as two weeks.
Besides medication, LN can be helped by a person controlling their blood pressure, cholesterol levels, diabetes, and keeping a healthy BMI.
If late-stage kidney disease or renal failure is present, a kidney transplant may be the best option. LN patients are faced with the decision to move forward with transplantation after dialysis (the process of an external machine doing the work of the kidney by filtering waste products and fluids from the blood body) has failed. Unfortunately, patients often deal with delays with finding a donor match. The wait on a donor list can be anywhere from months to years. A living donor (often a family member or friend) with a perfect match can shorten this waiting period considerably. Blood type can also influence how much time a person must wait for a match. The national kidney registry extends invitations to have people tested and added to their donor database. For more information on organ donation click here.
Many people find that after a kidney transplant, their symptoms improve for a long time and can even achieve remission with the aid of immunosuppressants and healthy lifestyle changes.
Here are some suggestions:
Eat less sodium (salt) to help control your blood pressure and reduce swelling
Eat smaller portions of foods with a lot of protein, like meat and dairy
Cut back on foods with saturated fats, like butter and fatty meats
If you drink alcohol, drink only in moderation
Work with your doctor to control your blood pressure, diabetes or high cholesterol (courtesy of the Lupus Foundation of America)
Patient stories:
We have now covered what LN is, the possible symptoms, diagnostic tests, and common treatment options. However, how does it actually feel living with it? How can it affect daily life, school, a job, and family planning?
Since MTL is a patient centered organization, we decided to ask a few of our community members to share some thoughts on the biggest challenges of living with lupus nephritis.
Here is what they said:
“I think the biggest challenge with living with lupus nephritis for me is planning to start a family. I am on CellCept (mycophenolate) and can’t take azathioprine (Imuran). I can’t be on CellCept if I want to try and start a family, but I am worried if I come off it and flare during a pregnancy.” - Danielle, SLE and LN Patient
“Being a teenager with LN means I have a LOT of doctor appointments, it's hard to talk to friends who don’t have issues like this, because it is a LOT. I have infusions, lab tests, and special diet protocols. When I am in the hospital, I am isolated a lot. I have to ask for special accommodations at school, which can be hard. But my mom, my sister, and my primary care physician were and are helpful.” - Micah, SLE and LN Patient
“Living with LN makes you a fighter. Many become one early in life (because the disease can start as a child) and it changes the way you see the world. You must live every day carefully, watching how much you do, what types of foods you eat and how rundown you get. If you don’t you can get caught in a terrible flare and suffer the consequences for months. I urge you, if you are living with this disease, please take it seriously. Change your lifestyle and food choices if necessary. Do everything you can to protect yourself and your kidneys.
As a 17-year lupus and LN survivor, I can tell you firsthand that careful management of your disease will greatly increase your life expectancy.” - Nicole, SLE and LN Patient
"So many challenges living with lupus nephritis: blood, brain, bone, and heart involvement; social challenges (missing school and other events); strict dietary challenges; weakness, swollen limbs, and gout. There are also career challenges, with lupus fog and blood toxicity. Not to mention financial hardships and medication side effects."
- Mehana, SLE and LN Patient
“Having kidney issues with lupus is like living with a dormant volcano inside your body. You never know when it may become active, so you become an expert at knowing what the signs and signals are. You stay vigilant, so you are ready to act, when it bubbles to the surface.” - Kelli
Written By:
Kelli (Casas) Roseta
Reviewed by: Donald Thomas, M.D., FACP. FACR, RhMSUS
Sources:
The Lupus Encyclopedia, Dr. Donald Thomas, Jr. MD., FACP, FACR, Johns Hopkins Press, 2014, pages 196-211; 62, 154, 340-41
**All resources provided by this blog are for informational purposes only, not to replace the advice of a medical professional. Kelli encourages you to always contact your medical provider with any specific questions or concerns regarding your illness. All intellectual property and content on this site and in this blog is owned bymorethanlupus.com. This includes materials protected by copyright, trademark, or patent laws. Copyright, More Than Lupus 2022.
In honor of Genesis BerlinIn honor of and all the lupus fighters that have fought the good fight against lupus and lupus nephritis.
December 2022
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