Lupus nephritis (patient information)

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 * Jinhui Wu, MD.; Associate Editor-In-Chief: Mohammed A. Sbeih, M.D. [mailto:msbeih@perfuse.org]

Overview
Lupus nephritis is an inflammation of the kidney caused by systemic lupus erythematosus (SLE), which is a disease of the immune system. The underlying cause of this autoimmune disease is not clear. Usual symptoms of lupus nephritis include blood in the urine, foamy appearance to urine, hypertension, swelling and weight gain. Positive anti-nuclear antibody (ANA) titer and damaged renal function suggest the diagnosis of lupus nephritis. Treatment opinion includes medications such as corticosteroids, immunosuppressants and cytotoxic drugs. For end stage renal failure, dialysis and kidney transplant may be considered. Prognosis varies from person to person, mainly depending on the class of lupus nephritis.

What are the symptoms of Lupus nephritis?
Some patients may show no signs and symptoms of lupus nephritis. Usual symptoms include:
 * Blood in the urine
 * Foamy appearance to urine
 * Hypertension
 * Swelling of any area of the body, especially around the eyes.
 * Weight gain

Diseases with similar symptoms are
 * Chronic glomerulonephritis
 * Polyarteritis nodosa
 * Wegener granulomatosis

What causes Lupus nephritis?
Systemic lupus erythematosus (SLE, or lupus) is an autoimmune disease. This means there is a problem with the body's immune system.

Normally, the immune system helps protect the body from harmful substances. But in patients with an autoimmune disease, the immune system cannot tell the difference between harmful substances and healthy ones. As a result, the immune system attacks otherwise healthy cells and tissue.

SLE may damage different parts of the kidney, leading to interstitial nephritis, nephrotic syndrome, andmembranous GN. It may reapidly worsen to kidney failure.

Lupus nephritis affects approximately 3 out of every 10,000 people. In children with SLE, about half will have some form or degree of kidney involvement.

More than half of patients have not had other symptoms of SLE when they are diagnosed with lupus nephritis.

SLE is most common in women ages 20 - 40.

Who is at highest risk?
The underlying cause of this autoimmune disease is not clear.

When to seek urgent medical care?
Call your doctor if your symptoms of lupus nephritis develop. If you experience the following symptoms, seeking urgent medical care as soon as possible:
 * Urine output decreases even anuria.

Diagnosis
A physical exam shows signs of decreased kidney functioning with edema. Blood pressure may be high. Abnormal sounds may be heard when the doctor listens to the heart and lungs, indicating fluid overload.

Tests that may be done include:

A kidney biopsy is not used to diagnose lupus nephritis, but to determine what treatment is appropriate.
 * ANA titer.
 * BUN and creatinine.
 * Lupus test.
 * Urinalysis.
 * Urine immunoglobulin light chain

This disease may also affect the results of the following tests:


 * Complement component 3.
 * Complement.
 * Syphilis test.

Treatment options
Your physician will run the following test to come to a conclusion
 * Urinalysis: Patients with lupus nephritis may demonstrate blood or protein in the urine which is a sign of kidney damage.
 * Anti-nuclear antibody (ANA) titer: Anti-nuclear antibody titer was the first indication that processes affecting the cell nucleus were responsible for systemic lupus erythematosus (SLE) and is used to measure the pattern and amount of autoantibody. In patients with lupus nephritis, it is higher than normal.
 * Blood tests: Patients with lupus nephritis may demonstrate elevated BUN and creatinine. These are damages of kidney function.
 * Kidney images such as x-ray, ultrasound and CT: These images can show the shape and size of the surfaces of the kidney, and tell the stucture of the kidney. They may help doctor exclude other diseases in the kidney.
 * Kidney biopsy: This biopsy can confirm the diagnosis of lupus nephritis and determine how far the damages have developed. During this procedure, the doctor insert a long needle through the skin to obtain a kidney tissue sample for examination with a microscope. And more important, the result of biopsy can help select appropriate treatment for patients.

Treatment of lupus nephritis depends on the symptoms and test results. The purpose of treatment is to improve kidney function.
 * General treatments
 * Take a kidney disease diet, limiting protein, sodium, and potassium in your diet.
 * Maintain your weight
 * Have a bedrest
 * Avoid nephrotoxicity drugs
 * Control blood pressure


 * Medicines
 * Corticosteroids: Corticosteroids can decrease swelling and inflammation by suppressing the immune system. Side effects of corticosteroids may include a decreased ability against infection, worse healing in the wound and osteoporosis.
 * Immunosuppressants: Immunosuppressive drugs, such as cyclophosphamide, mycophenolate mofetil, or azathioprine, may be used if the patient has an inadequate response or excessive sensitivity to corticosteroids.
 * Cytotoxic drugs: These kind of drugs may interfere with growth of normal and neoplastic cells by cross-linking of DNA or RNA or proteins and are typically given to people who have lupus nephritis to prevent kidney damage. Usual drugs include cyclophosphamide and azathioprine. Side effects include marrow suppression, liver damage, nausea and vomiting.


 * Dialysis: When you develop end stage renal failure, the doctor may recommend dialysis. There are two types of dialysis, hemodialysis and peritoneal dialysis.
 * Kidney transplant: This is an operation that places a healthy kidney to take over the work of the two kidneys that failed. Some transplanted kidneys come from donors who have died and, while others come from a living family member. Patients who have transplants need to take immunosuppressants to keep their body from rejecting the new kidney for the rest of their lives.

Where to find medical care for Lupus nephritis?
Directions to Hospitals Treating Lupus nephritis

What to expect (Outlook/Prognosis)?
The outcome varies, depending on:
 * The class of lupus nephritis: Focal lupus nephritis, minimal mesangial lupus nephritis and mesangial proliferative lupus nephritis have better prognosis than other classes. And, advanced sclerosis lupus nephritis carries a poor prognosis.
 * Nephrotic syndrome carries worse prognosis.
 * Creatinine level: Patients with elevated creatinine (>3 mg/dL) at presentation have worse outcomes.
 * Patients with persistently elevated anti-dsDNA and low C3 and C4 levels have poorer outcomes.
 * Renal biopsy findings showing diffuse lupus nephritis or high chronicity index suggest worse prognosis.
 * Age onset of lupus nephritis: Young age onset carries worse prognosis.
 * Male gender have poorer outcomes than female.
 * Black race have worse outcomes than other races.

Possible complications

 * Acute renal failure.
 * Chronic renal failure.
 * End-stage renal disease.
 * Nephrotic syndrome.

Prevention
Prevention of lupus nephritis is unknown.