What is Pediatric Lupus Nephritis: Causes, Symptoms, Types, Treatment, Diagnosis

Lupus nephritis is a condition that causes inflammation of the kidneys. It is caused by systemic lupus erythematosus (SLE). SLE is also commonly known as lupus and is a common type of autoimmune disease. In a person suffering from lupus, the body’s immune system starts attacking its own body tissues. When lupus involves the kidneys, the condition is known as lupus nephritis. Nearly 60% of all lupus patients develop lupus nephritis. Children suffering from lupus nephritis often end up in a critical condition. Lupus nephritis in children also impacts the overall outcome of their systemic lupus erythematosus. When children come down with lupus, it becomes a severe case of multisystem autoimmune disease. Lupus nephritis is often fatal in pediatric patients and conducting a renal biopsy becomes a critical investigation in managing this disease.

The treatment of lupus nephritis in children using steroids is also known to have excessive side effects and an overall poor outcome. While the inclusion of cyclophosphamide to the treatment of pediatric lupus nephritis has helped in improving and controlling the disease, newer agents still need to be tried and tested to improve the chances for pediatric lupus nephritis.

Lupus nephritis is a condition in which the kidneys become inflamed due to systemic lupus erythematosus (SLE), which is an autoimmune disease. Lupus nephritis occurring in children is known as pediatric lupus nephritis. Pediatric lupus nephritis causes the glomeruli to become inflamed and therefore is known as a type of glomerulonephritis. During lupus, the body’s immune system starts targeting its own body tissues. Nearly 60% of patients who suffer from lupus are likely to develop lupus nephritis. Due to inflammation of the kidneys in lupus nephritis, they cease to function properly and can leak protein. If left untreated, lupus nephritis can lead to kidney failure. Pediatric lupus nephritis causes swelling or even scarring of the small blood vessels (these are known as glomeruli) that are responsible for filtering out wastes from your kidney. Lupus is more common in women than in men but often occurs in children as well. In women as well, lupus generally strikes during child-bearing years. About 5 out of 10 adults, who have lupus, end up having kidney disease. In children with lupus, about 8 out of 10 will end up having kidney disease.

As with most autoimmune diseases, there is no known cause of pediatric lupus nephritis. Genetics is thought to play a role in it along with environmental factors such as viruses, infections, pollutants or toxic chemicals. Pollutants such as factory smoke or car fumes are likely to aggravate an underlying condition of pediatric lupus nephritis.

Pediatric Lupus Nephritis is a critical disease if it is not treated on time. However, the symptoms of pediatric lupus nephritis are not usually serious. The first noticeable symptom that occurs is the swelling of ankles, feet, and legs. There can also be swelling in the hands or face, though this is less common. Other symptoms of pediatric lupus nephritis vary from patient to patient and from day to day and include:

• Blood in the urine, known as hematuria, glomerular disease causes leakage of blood into the urine, causing the urine to look pink or dark brown in color.
• Weight gain happens due to the fluid that the kidneys is not able to get rid of.
• High blood pressure.
• Frothy, foamy urine, known as proteinuria, happens because the glomeruli leaks protein into the urine.
• Pressing need to urinate in the night.
• Joint pain.
• Fever.
• Edema.
• Muscle pain.
• A red rash, usually on the face, also known as a butterfly rash.

However, do note that not all kidney or urinary problems in lupus sufferers arise due to pediatric lupus nephritis. People with lupus are more prone to getting urinary tract infections and this causes a burning sensation during urination and frequently requires treatment with antibiotics. Some lupus medications also affect the kidneys and lead to swelling and several other symptoms that are similar to pediatric lupus nephritis. Once these medications are stopped, these problems also disappear.

To diagnose pediatric lupus nephritis, your doctor will begin by taking a medical history, conducting a physical examination, and evaluate the symptoms of the child. Tests will be prescribed to either confirm the diagnosis or determine what the underlying problem is. Patients of pediatric lupus nephritis have symptoms that vary in severity and thus the treatment also has to be customized according to individual requirements.

Generally doctors suspect a case of pediatric lupus nephritis if the child is showing the following symptoms:

• Protein is detected in your urine test.
• Urine has a foamy appearance.
• Your blood test reveals high levels of creatinine.
• You have high blood pressure.
• There is swelling in your ankles, feet or hands.

The two most common tests done for diagnosing Pediatric Lupus Nephritis include:

Renal Function Test: A blood test and a 24-hour urine culture test are done to measure the kidney function. Sometimes, many patients are also given an iothalamate clearance test that makes use of a special contrast due to indicate the filtering function of your kidneys.

Kidney Biopsy: This test is the ultimate testing done to diagnose pediatric lupus nephritis. In this, doctors remove a small section of the patient’s kidney tissue to analyze it microscopically. It helps doctors understand what stage the Pediatric Lupus Nephritis is at.

The primary goal of treatment in pediatric lupus nephritis is to bring the kidney function back to normal or to at least try and prevent the progression of total loss of kidney function. There are five different types of lupus nephritis and treatment for each type is determined by conducting a biopsy first. As symptoms and their severity vary from patient to patient, treatments are also tailored to meet the individual conditions.

Commonly used medications for the treatment of Pediatric Lupus Nephritis include:

Corticosteroids: These are known to be anti-inflammatory in nature and work to lower inflammation of the kidneys. These strong drugs are prescribed by the doctors till the condition of Pediatric Lupus Nephritis shows some improvement. Corticosteroids also have known serious side effects and their dosage needs to be monitored carefully. Patients are weaned off corticosteroids slowly, once the symptoms of Pediatric Lupus Nephritis begin to improve.

Medications that lower blood pressure or prevent blood clots if necessary.

Immunosuppressant Drugs: Immunosuppressive drugs are used to suppress the activity of the immune system that causes damage to the kidneys. Some of these drugs used in Pediatric Lupus Nephritis include azathioprine (Imuran), cyclophosphamide (Cytoxan), and mycophenolate (Cellcept).

Decreased Salt Intake: On a general basis, doctors also recommend that patients suffering from Pediatric Lupus Nephritis follow a specialized diet to improve their kidney function. The diet includes a restriction on salt intake.

Many times, even with timely treatment, the loss of renal function progresses and in the case of failure of both the kidneys, children with Pediatric Lupus Nephritis need to be put on dialysis. Sometimes, a kidney transplant might also be necessary.

There is no permanent cure for Pediatric Lupus Nephritis and the treatment revolves around inducing remission of the active disease and preventing the disease from worsening. Management of symptoms and avoiding the stage of dialysis or kidney failure are also the goals of the treatment.

Systemic lupus erythematosus (SLE) strikes children in a more severe form than adults. As kidney disease is a common aspect of SLE, children are thus equally susceptible to getting lupus nephritis. As the severity of the disease is much more in children, if not treated properly, it may prove to be fatal for the child. In children, the stages of Pediatric Lupus Nephritis also progress much more rapidly to kidney failure as compared to adults. However, the issue of subjecting pediatric patients to aggressive treatments for Pediatric Lupus Nephritis has severe side effects as well. For example, aggressive treatment with corticosteroids has been known to carry the risk of growth retardation, severe infections, as well as accelerate atherosclerosis.

In children, Pediatric Lupus Nephritis is classified as being of six types, depending on how severe the histological lesions are. Treatment protocol also depends on the type of Pediatric Lupus Nephritis a child is.

Class I & II Pediatric Lupus Nephritis: For class 1 or Class II Pediatric Lupus Nephritis, in which mild lesions occur, treatment is limited to only careful observation and follow-up to ensure that the disease is not progressing.

Class III Pediatric Lupus Nephritis: Patients having class III nephropathy have segmental and focal glomerulonephritis. They present with mild clinical symptoms and no specific therapy is required. If symptoms become more severe, or high blood pressure and moderate renal damage are detected, then the doctor will start the child off on the appropriate medications that class IV patients are given.

Class IV Pediatric Lupus Nephritis: Class IV patients suffering from Pediatric Lupus Nephritis usually present with diffuse proliferative glomerulonephritis. The treatment protocol for class IV patients includes medications such as methylprednisolone, prednisone, and cyclophosphamide, for a cycle of six months.

Following the second round of kidney biopsy, the child is slowly tapered off some of the medications and continued only on azathioprine.

Class V Pediatric Lupus Nephritis: Pediatric patients presenting with class V Pediatric Lupus Nephritis display membranous glomerulonephritis and require aggressive treatment if they also present with advanced kidney damage. If only membranous nephropathy is present, then no specific treatment protocol is started.

Class VI Pediatric Lupus Nephritis: Pediatric patients with last-stage kidney failure are put on the transplant list. A kidney transplant is the only solution in these cases, as recurrence of Pediatric Lupus Nephritis in the transplanted kidney is known to be very rare.

A child suffering from Pediatric Lupus Nephritis needs to introduce certain lifestyle changes. This can help them protect their kidneys from further damage. Some of these habits include:

• Drinking sufficient fluids, especially water, to remain well hydrated.
• Avoid smoking and alcohol consumption.
• Follow a regular exercise schedule.
• Consume a diet that is low in sodium, particularly if you are suffering from high blood pressure as well.
• Keep a watch on your blood pressure. Try to maintain it within the normal levels.
• Avoid medications that can have a damaging impact on your kidneys, such as nonsteroidal anti-inflammatory drugs (NSAIDs).
• Limit foods that are high in cholesterol.