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A.D.A.M. Medical Encyclopedia. Atlanta (GA): A.D.A.M.; 2011.

A.D.A.M. Medical Encyclopedia.

Lupus nephritis

Nephritis - lupus; Lupus glomerular disease

Last reviewed: September 19, 2011.

Lupus nephritis is a kidney disorder that is a complication of systemic lupus erythematosus.

Causes, incidence, and risk factors

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 infection or 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, and membranous GN. It may rapidly 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. For more information, see: systemic lupus erythematosus.

Symptoms

Symptoms of lupus nephritis include:

For general lupus symptoms, see the article on SLE.

Signs and tests

A physical exam shows signs of decreased kidney functioning with body swelling (edema). Blood pressure may be high. Abnormal sounds may be heard when the doctor listens to your heart and lungs.

Tests that may be done include:

This list may not be all-inclusive.

A kidney biopsy is not used to diagnose lupus nephritis, but to determine what treatment is appropriate.

Treatment

The goal of treatment is to improve kidney function and to delay kidney failure.

Medicines may include corticosteroids or other medications that suppress the immune system, such as cyclophosphamide, mycophenolate mofetil, or azathioprine.

You may need dialysis to control symptoms of kidney failure, sometimes for only a while. A kidney transplant may be recommended. People with active lupus should not have a transplant because the condition can occur in the transplanted kidney.

Expectations (prognosis)

How well you do depends on the specific form of lupus nephritis. You may have flare-ups, and then times when you do not have any symptoms.

Some people with this condition develop chronic kidney failure.

Although lupus nephritis may return in a transplanted kidney, it rarely leads to end-stage kidney disease.

Calling your health care provider

Call your health care provider if you have blood in the urine or swelling of your body.

If you have lupus nephritis, call your health care provider if you notice decreased urine output.

Prevention

There is no known prevention for lupus nephritis.

References

  1. Appel GB. Glomerular disorders and nephrotic syndromes. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 122.
  2. Appel GB, Radhakrishnan J, D’Agti V. Secondary glomerular disease. In: Brenner BM, ed. Brenner and Rector's the Kidney. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 31.

Review Date: 9/19/2011.

Reviewed by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Herbert Y. Lin, MD, PHD, Nephrologist, Massachusetts General Hospital; Associate Professor of Medicine, Harvard Medical School. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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Drugs of interest

  • Cyclophosphamide
    Cyclophosphamide is used alone or in combination with other medications to treat Hodgkin's lymphoma (Hodgkin's disease) and non-Hodgkin's lymphoma (types of cancer that begin in a type of white blood cells that normally fights infection); cutaneous T-cell lymphoma (CTCL, a group of cancers of the immune system that first appear as skin rashes); multiple myeloma (a type of cancer of the bone marrow); and certain types of leukemia (cancer of the white blood cells), including chronic lymphocytic leukemia (CLL), chronic myelogenous leukemia (CML), acute myeloid leukemia (AML, ANLL), and acute lymphoblastic leukemia (ALL). It is also used to treat retinoblastoma (cancer in the eye), neuroblastoma (a cancer that begins in nerve cells and occurs mainly in children), ovarian cancer (cancer that begins in the female reproductive organs where eggs are formed), and breast cancer. Cyclophosphamide is also used to treat nephrotic syndrome (a disease that is caused by damage to the kidneys) in children whose disease has not improved, has gotten worse, or has come back after taking other medications or in children who experienced intolerable side effects with other medications. Cyclophosphamide is in a class of medications called alkylating agents. When cyclophosphamide is used to treat cancer, it works by slowing or stopping the growth of cancer cells in your body. When cyclophosphamide is used to treat nephrotic syndrome, it works by suppressing your body's immune system.
  • Mycophenolate
    Mycophenolate (CellCept) is used with other medications to help prevent transplant organ rejection (attack of the transplanted organ by the immune system of the person receiving the organ) in people who have received kidney, heart, and liver transplants. Mycophenolate (Myfortic) is used with other medications to help prevent the body from rejecting kidney transplants. Mycophenolate is in a class of medications called immunosuppressive agents. It works by weakening the body's immune system so it will not attack and reject the transplanted organ.
  • Azathioprine
    Azathioprine is used with other medications to prevent transplant rejection (attack of the transplanted organ by the immune system) in people who received kidney transplants. It is also used to treat severe rheumatoid arthritis (a condition in which the body attacks its own joints, causing pain, swelling, and loss of function) when other medications and treatments have not helped. Azathioprine is in a class of medications called immunosuppressants. It works by decreasing the activity of the body's immune system so it will not attack the transplanted organ or the joints.
  • Cyclophosphamide Injection
    Cyclophosphamide is used alone or in combination with other medications to treat Hodgkin's lymphoma (Hodgkin's disease) and non-Hodgkin's lymphoma (types of cancer that begin in a type of white blood cells that normally fights infection); cutaneous T-cell lymphoma (CTCL, a group of cancers of the immune system that first appear as skin rashes); multiple myeloma (a type of cancer of the bone marrow); and certain types of leukemia (cancer of the white blood cells), including chronic lymphocytic leukemia (CLL), chronic myelogenous leukemia (CML), acute myeloid leukemia (AML, ANLL), and acute lymphoblastic leukemia (ALL). It is also used to treat retinoblastoma (cancer in the eye), neuroblastoma (a cancer that begins in nerve cells and occurs mainly in children), ovarian cancer (cancer that begins in the female reproductive organs where eggs are formed), and breast cancer. Cyclophosphamide is also used to treat nephrotic syndrome (a disease that is caused by damage to the kidneys) in children whose disease has not improved, has gotten worse, or has come back after taking other medications or in children who experienced intolerable side effects with other medications. Cyclophosphamide is in a class of medications called alkylating agents. When cyclophosphamide is used to treat cancer, it works by slowing or stopping the growth of cancer cells in your body. When cyclophosphamide is used to treat nephrotic syndrome, it works by suppressing your body's immune system.
  • Rituximab Injection
    Rituximab is used alone or with other medications to treat certain types of non-Hodgkin's lymphoma (NHL; a type of cancer that begins in a type of white blood cells that normally fights infection). Rituximab is also used with another medication to treat the symptoms of rheumatoid arthritis (RA; a condition in which the body attacks its own joints, causing pain, swelling, and loss of function) in people who have already been treated with a certain type of medication called a tumor necrosis factor (TNF) inhibitor. Rituximab is in a class of medications called biologic antineoplastic agents. It treats NHL by causing the death of blood cells that have multiplied abnormally. It treats rheumatoid arthritis by causing the death of certain blood cells that may cause the immune system to attack the joints.
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