Systemic lupus erythematosis (SLE) is an autoimmune disease—a disorder in which the body’s immune system attacks the body’s own cells and organs. For this blog, we have enlisted and are honored to have the input of Dr.Donald Thomas, MD, the author of The Lupus Encyclopedia: A Comprehensive Guide for Patients and Families, for his expert knowledge on lupus.
Lupus can present itself with many different symptoms, and these can vary widely in frequency and severity between lupus patients. Lupus nephritis is kidney inflammation caused by systemic lupus erythematosis (SLE) and one of the most serious complications that can result from SLE. Evidence suggests that 35% of adults in the US show signs of nephritis at the time of a lupus diagnosis and approximately 40- 60% of people will develop kidney involvement during the course of the disease. That number jumps to 75% for those who are African American. If not diagnosed and treated early enough, kidney nephritis can lead to significant illness and sadly, even death.
“Before modern therapies became available to treat lupus (cortisone in 1950), lupus inflammation of the kidneys was the number one cause of death in people who had lupus causing up to 80% of lupus patients to not survive more than 2 years after their diagnosis. Today, I am happy to report that the majority of patients with inflammation of the kidneys from lupus respond well to treatment, most do not go on to dialysis or require a kidney transplant; but it is very important to abide by certain recommendations to ensure that you do well.” -Dr. Donald Thomas
What are the kidneys and what do they do?
The kidneys are two bean-shaped organs, each about the size of a fist. They are located near the middle of the back, just below the rib cage, with one located on either side of the spine. Every day, the two kidneys process about 200 quarts of blood to produce about 1 to 2 quarts of urine, which is made up of waste and extra water. The urine flows from the kidneys to the bladder through tubes called ureters. The bladder stores urine until releasing it through urination.
“They are truly amazing in their ability to remove waste products that our bodies do not need any more, while also constantly making sure that the perfect amount of elements (such as sodium, potassium, calcium) remain in the blood, maintain proper blood pressure, keep the pH acidity of the blood perfect at all times (so that the enzymes of our body function properly), and keep the actual amount of water in our bodies optimal. Many people do not realize that the kidneys even act as endocrine glands, secreting hormones that ensure that the bone marrow makes the red blood cells of the body, and also converts vitamin D produced by the skin into a more useful form of vitamin D.”- Dr. Thomas.
Kidney failure is a condition where the filtering units (glomeruli) inside of the kidneys become damaged and are unable to filter out the waste and toxins from the body. These toxins then remain in the bloodstream causing complications throughout the body. These complications can hinder the function of vital organs making a person become very ill.
What are the symptoms of lupus nephritis and how do I recognize it?
Kidney disease is often ‘silent’ with little or no visible symptoms. In addition, the symptoms of lupus nephritis can vary widely from person to person. Some of the symptoms may include:
- Foamy Urine- This is often one of the first signs of lupus nephritis
- Blood in the Urine- This is also often one of the first signs of lupus nephritis
- Pain in Your Mid-back or ‘Flank’
- Swelling- Usually in the legs, feet, or ankles and less often in the hands or face. This tends to worsen as the day progresses or after sitting (this is called edema)
- Dark Urine
- High Blood Pressure
- Changes in Frequency of Urination- Either much more or much less often.
- Vomiting- Toxins that build up because the kidney is unable to remove them from the body can result in abnormal function of the liver and the process of digestion.
- Water Retention/ Weight Gain- the entire body may seem bloated and firm, often affecting the legs and hands more than other parts of the body. The kidneys are not able to remove the extra water which is present in the blood of the person affected.
One may experience the symptoms of systemic lupus erythematosis in addition to the above symptoms for lupus nephritis.
It is important to know that not all kidney problems are due to lupus nephritis. Lupus patients are often prone to urinary tract infections. These can cause burning during urination and can be treated with antibiotics. There are medications that can also affect the kidneys, causing swelling and symptoms that may mimic lupus nephritis. Fortunately when the medications are stopped, those symptoms will typically disappear.
How does lupus nephritis get diagnosed?
The first thing a physician will consider when making a lupus nephritis diagnosis is your medical history, followed by a physical exam and an evaluation of symptoms.
“We always hope to catch lupus nephritis at an early stage where there may be some increased protein in the urine, yet the actual function on the kidney blood tests is still normal. This is when it can be treated most optimally.” -Dr. Thomas
The diagnosis will then be confirmed through imaging tests, urine and blood tests, and a kidney biopsy. Descriptions of these tests are as follows:
- Urinalysis- Urinalysis is testing of a urine sample. A high number of red blood cells or high levels of protein in the urine indicate kidney damage. “The urine test is the most important test to look for lupus nephritis.” –Dr. Thomas
- Iothalamate Clearance Testing- This test uses a dye to see if your kidneys are filtering correctly. Radioactive iothalamate is injected into your blood and then the doctors monitor to see how quickly it is evacuated into your urine. This is considered one of the most accurate tests to determine the filtration speed of the kidneys.
- Blood test- The blood test can show high levels of creatinine (a waste product of normal muscle breakdown excreted by the kidneys), which increases if the kidneys are not functioning well.
- Biopsy- A biopsy is a procedure that involves taking a small piece of kidney tissue for examination with a microscope. This test is used to confirm a diagnosis of lupus nephritis, determine how far the disease has progressed, and help to guide treatment options.
- Ultrasound- This test uses sound waves to create and accurate and detailed image of the kidneys, which helps determine if the size and shape of the organs is either normal or abnormal.
Note: The American College of Rheumatology recommends biopsies be taken for all people with evidence of active lupus nephritis that have not been previously treated.
Once diagnosed, how is lupus nephritis treated?
Treatment for lupus nephritis is based on the type and severity of nephritis that was determined by the biopsy. Treatments will be tailored to the individual’s medical needs and circumstances with the goal of improving kidney function and delay/prevent kidney failure.
The main types of treatment options are:
- Immunosuppressive Drugs- These are medications that suppress the immune system, so it stops attacking and damaging the kidneys. These are often similar to drugs used to treat cancer or drugs that help prevent the rejection of transplanted organs. These medications can include: cyclophosphamide (Cytoxan), azathioprine (Imuran) and mycophenolate (Cellcept).
- Corticosteroids- These are very strong anti-inflammatory drugs that decrease the inflammation in the kidneys. Prednisone is one of these corticosteroids. Because these types of drugs can cause various undesirable and potentially serious side effects, the physician will monitor the patient very closely and hopefully be able to taper down the prescribed dosage as the symptoms begin to improve.
- Medications that lower blood pressure- Such as angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) and prevent blood clots may also be prescribed if needed. These have proven effective in helping slow kidney disease progression.
- Hydroxychloroquine (Plaquenil)- This is a common medication for treating SLE. This should also be prescribed or continued for people with lupus nephritis.
- Diuretics- These medications, that help the kidneys remove fluid from the body, may also be prescribed.
“All of these medications have many potential side effects such as lowering the blood counts and increasing the risks for infections. They require very close monitoring by the doctor to ensure that side effects are dealt with if they occur and to see if they are helping with the kidney problem… The person with lupus nephritis truly requires an experienced physician to treat it, a large number of medications to ensure the best health for the patient, and a very compliant patient who is dedicated to following complex directions for taking the medications and doing all the proper tests regularly.”-Dr. Thomas
Even with treatment and close supervision by a medical provider, decreased kidney function can sometimes occur. If both of the kidneys fail, the patient may need dialysis. This is a procedure that filters blood through a machine in order to remove waste products from the body. In the most severe cases, a kidney transplant may eventually be necessary.
Can Lifestyle Changes Help Those With Lupus Nephritis?
As with most medical conditions including SLE and lupus nephritis, lifestyle decisions can play an important role in supporting the immune system and assist an individual in reac
- Stay Well Hydrated by Drinking Enough Fluids-According to the Mayo Clinic, The Institute of Medicine determined that an adequate intake (AI) for men is roughly 3 liters (about 13 cups) of total beverages a day. The AI for women is 2.2 liters (about 9 cups) of total beverages a day.hing their optimum level of health. The following lifestyle choices can help those who are suffering with lupus nephritis.
- Maintain a Healthy Blood Pressure (120/80 is generally considered to be in healthy range).
- Exercise regularly-30 minutes at least three times per week can make a positive impact on overall health. (Even moderate exercise, like walking, gentle stretching and yoga can help.)
- Limit Intake of Cholesterol- The American Heart Association recommends that you limit your average daily cholesterol intake to less than 300 milligrams. If you have heart disease, limit daily intake to less than 200 milligrams. People can significantly lower their dietary cholesterol intake by keeping their dietary intake of saturated fats low and by avoiding foods that are high in saturated fat and eliminating those that contain substantial amounts of dietary cholesterol.
- Avoid NSAIDs (non-steroidal anti-inflammatory drugs) as these can affect the kidneys, such as Ibuprofen, Aspirin, or Naproxen.
- Eat a Low Sodium Diet- Especially if you have high blood pressure.
- Wear sunscreen- This should be worn at all times as both outside and indoor lighting emit significant amounts of UV light.
- Get proper amounts of Vitamin D, essential for the proper functioning of the immune system. Check with your physician as to what amount is right for you.
- Take Medications as Directed by your physician(s).
How Research is Providing Hope
Researchers have learned much about kidney disease in recent years. The National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) and other components of the National Institutes of Health (NIH) are funding research studies that are aimed at understanding and treating lupus nephritis and finding treatments with the goal of stopping its progression. A number of new medications that may be effective in the treatment of lupus nephritis have been discovered through research, and they are now being tested in clinical trials.
“This past year we have seen two successful studies use much lower doses of steroids in the treatment of lupus nephritis with excellent results. One study from London actually used no oral steroids at all. The investigators used some steroids intravenously on only two occasions in their patients along with a medication called rituximab (Rituxan). A year after treatment, half of the patients were in complete remission while a third of the patients were in partial remission. Another study from Spain used starting doses of prednisone as low as 15 mg a day (usual doses are typically around 60 mg a day of methylprednisolone a day for nephritis) in nephritis patients with excellent results. We never change medical practice based upon small singular studies (as both of these were), so we await for the results of larger, better studies which are currently being done to see if these results can be reproduced. If so, then we may be looking at the use of much lower doses of steroids in lupus patients in the near future. The importance of this cannot be under emphasized since high doses of steroids are the cause of so many side effects in our patients.”- Dr. Thomas
Coping with a disease like lupus or lupus nephritis can leave you feeling powerless. One way to gain some control, or feel effective, could be to participate in a clinical trial. A few of the benefits of participation in a trial may be:
- Patients can play a more active role in their own health care
- Gain access to new research treatments before they are widely available
- Help others by contributing to medical research through participating in clinical trials
For information about current lupus clinical trials in your area, visit Alliance for Lupus Research or find other trials here: www.clinicaltrials.gov. Be sure to discuss participation in any clinical trial with your physician to help determine the pros and cons for your specific medical situation.
In Summary, by Dr. Donald Thomas
“I note that the present and the future are brighter when it comes to systemic lupus and nephritis. Previously, lupus nephritis was the primary cause of death in lupus patients. Today, we realize that it is not as common as thought even a few years ago, yet it still does affect around 35% of patients. Make sure to take your hydroxychloroquine (Plaquenil) every single day if your rheumatologist has you on it, take vitamin D regularly to keep your blood 25-OH vitamin D level 40 ng/mL or higher, and use your sunscreen religiously every day (even if you don’t go outside) in the hopes of decreasing your chances of getting nephritis. See your rheumatologist at least every few months to give a urine sample to look for lupus nephritis, even if you feel perfectly fine (remember that most people with lupus nephritis do not have any symptoms at all) in the hopes of catching lupus nephritis in the earliest stages possible. If you do develop nephritis, take your medications as directed and do all tests as your rheumatologist asks. In the near future, we will have newer and better therapies for lupus nephritis and will hopefully be able to use much lower doses of steroids in its treatment while we await the discovery of a cure for lupus.”-Dr. Thomas
*Donald Thomas, MD, is a rheumatologist in private practice in Greenbelt and Clinton, MD at Arthritis and Pain Associates. He has been in practice as a rheumatologist since graduating from his rheumatology fellowship program at Walter Reed Army Medical Center in Washington DC in 1995. He has had a special interest in lupus and therefore has a lot of experience with lupus in caring for a large lupus patient population. He currently serves as the Chair of the Medical and Scientific Advisory Council of the Lupus Foundation of America DC/MD/VA chapter, teaches as an Assistant Professor of Medicine at the Uniformed Services University of the Health Services in Bethesda, MD, and is the author of the upcoming book The Lupus Encyclopedia: Book about SLE, fibromyalgia, Sjögren’s, arthritis etc. Educating and helping patients is his passion, and he has found Facebook a fantastic vehicle to help people all over the world stricken by this disorder.You can find Dr. Thomas on Facebook, http://www.facebook.com/LupusEncyclopedia, or by email at [email protected]
To read the link to Dr. Thomas’s complete article on Lupus Nephritis, including sources, please click the following link:
*All resources provided by Molly’s Fund are for informational purposes only and should be used as a guide or for supplemental information, not to replace the advice of a medical professional. The personal views do not necessarily encompass the views of the organization, but the information has been vetted as a relevant resource. We encourage you to be your strongest advocate and always contact your medical provider with any specific questions or concerns.