Neurological Complications of Lupus
Terry D. Heiman-Patterson, MD
LEARNING ABOUT LUPUS: A USER FRIENDLY GUIDEFrom the Lupus Foundation of Delaware Valley, Inc.Edited by: Mary E. Moore, Ph.D., M.D., Carolyn H. McGrory, MS, RN, Robert S. Rosenthal, M.D.
Systemic lupus etythematosus (SLE) is a multisystem disorder that frequently affects the nervous system. In fact, of all the connective tissue disorders, lupus has the highest incidence of nervous system complications. These neurologic complications may occur in 30-75 percent of patients. In order to understand the variety of complications, one must have some notion of what makes up the nervous system.
The nervous system contains the brain, brain stem, spinal cord, nerves outside the brain and spinal column (peripheral nerves), and muscle. The brain has centers for speech, movement, sensation, and also more complex functions such as thinking, memory, and personality. The brain relays commands and messages to other areas of the nervous system and receives information from these areas, as well. The brain blends together all this information so that persons can interact with their environment. Between the brain and the spinal cord is the brain stem. The brain stem contains nerve cells that control the muscles of eye movement, facial expression, speaking, swallowing, hearing and balance. The spinal cord contains nerve cells that supply impulses to and from the muscles (i.e., innervate the muscles), and that receive sensory information from the skin and joints.
Muscles attach to bones and when they contract they enable a person to move. Information travels from the brain to the brain stem and spinal cord where contact is made with the nerve cells, (i.e. the cranial and peripheral nerves) innervating muscles of the head and arms and legs. Sensory information is also relayed from these areas through contacts in the spinal cord and brain stem to the sensory centers of the brain. There are several possible ways that lupus can damage the nervous system. First, the blood vessels of the brain may themselves be damaged, leading to poor blood flow, to death of brain tissue (strokes), or to bleeding (hemorrhage). There may be an immune system attack on the nervous system because of antibodies that are produced. These can cause problems directly by attacking nerve cells (antineuronal antibodies) or indirectly by causing blood clots in the brain (lupus anticoagulants or anticardiolipin antibodies).
When lupus affects the nervous system, the type of symptom and the distribution of complaints will depend on what area of the nervous system is involved and on what sensory and motor pathways are damaged. When there is more general involvement of the brain, a patient may demonstrate personality changes and psychiatric disorders. Actual psychosis (severe mental illness) can occur, or mood changes such as depression, or even an unnatural feeling of well-being (euphoria) can occur. Other patients may experience anxiety that is out of proportion to the problems they are experiencing. in all these psychiatric disorders, thinking and memory are normal. However, some patients may actually have loss of intellectual skills (i.e., dementia), or experience confusion and altered consciousness, (sleepiness and even coma). Although confusion and altered consciousness may occur because of direct involvement of the brain by lupus, it can also occur because of drugs used in the treatment of lupus (i.e. steroids) or because of other complications from lupus (i.e. infection, kidney failure).
In these instances, withdrawal of the drug, treatment of the infection, or management of the other systemic complications will help to clear the confusion and improve alertness. Another complication due to brain involvement is having seizures or convulsions. These can occur in up to 20% of patients. In patients with kidney failure due to lupus, the rate of seizures rises to almost 50 percent. Lupus can also cause strokes in which there is a sudden onset of neurologic symptoms. Strokes can be due to bleeding into the brain or to death of brain tissue because of lack of blood flow. These problems occur if there is inflammation of blood vessels, clotting of blood within vessels, or not enough clotting of blood causing patients to hemorrage more readily. Patients with strokes may have a variety of symptoms, including loss of sensation on one side of the body, loss of movement (paralysis) on one side of the body, problems with verbal expression, or problems with vision.
The exact symptoms will depend on what area of the brain is damaged. Headaches can occur in 20-25 percent of lupus patients. These are most like migraine headaches that are pounding, associated with nausea and vomiting, and cause photophobia (light hurting the eyes). Other much rarer complications due to brain damage relate to problems with coordinated movements. Brain stem involvement with lupus also causes a variety of symptoms. These are primarily due to involvement of the nerves to the muscles of the head and of the sensory pathways from the face. Problems with movement of the eyes can cause double vision. Inflammation of the nerve for vision can cause blurring of vision.
Sensation or movement of the face can be altered, causing numbness or tingling or weakness of the face. In addition, other symptoms of brain stem involvement include hearing problems, slurred speech, and trouble swallowing. Since the longer nerve pathways that carry motor and sensory information between the brain and spinal cord may also be damaged in the brain stem, there may be sensory and motor symptoms, not only of the head and face, but also in the arms and legs. Spinal cord involvement is very rare. When it does occur, it is called "myelopathy" and patients may not be able to move their arms and legs depending on what section of the spinal cord is involved. In addition, sensation is also changed below the area of the spinal cord that is involved. The patient may not feel pain or temperature properly. Other sensations may also be affected such as the ability to feel a vibration or to sense the body's position.
Lupus can imitate inflammation of the spinal cord that is usually due to other diseases such as multiple sclerosis. These symptoms require careful examination to determine their cause. The peripheral nervous system includes the rest of the motor and sensory nerves and the muscles. It includes the nerves that come from the spinal cord to all parts of the body. The peripheral motor nerves carry motor information (originating in the brain) from the spinal cord to the muscle. The muscle will then contract so movement can occur. The sensory fibers carry sensory information from the skin, joints, and muscle to the spinal cord, where it can then be relayed back to the brain. Damage to the peripheral nerves occurs in 10-15 percent of patients with lupus and can result in numbness (pins and needles sensation) of the feet and hands, an inability to feel a pin prick, temperature change, vibration, or change in position, and weakness. If this peripheral nerve damage is severe, patients may inadvertently hurt themselves without knowing it.
If this becomes a frequent occurrence, it can lead to infections, ulcerations, and joint problems. Sometimes individual peripheral nerves to the arm or leg can be damaged. This results in more local sensation and movement problems. Finally, some types of sensation may be more affected than others. If position sense is altered, difficulties with walking may result. This is due to an inability to sense where the feet are being placed. In addition to peripheral nerve involvement, the muscle can also be involved with inflammation or other nonspecific damage. This occurs in 5-30 percent of patients. Patients with muscle problems will experience weakness of the thighs and shoulders. They may have trouble arising from chairs or going up stairs. There will be complaints related to combing the hair and putting things up on a shelf. There may also be some muscle tenderness.
When patients with lupus are clearly weak, other factors should also be considered, i.e. steroid-induced weakness and weakness due to other problems from lupus. Kidney disease, poor nutrition, or immobility can all lead to muscle weakness. When neurologic complications begin, the first step is to identify lupus as the causative factor and make sure all other possible causes are considered (i.e., infection, anemia, kidney failure, drug toxicities). If they are present, these other causes should then be treated appropriately. On the other hand, if the complication is primarily due to lupus, then treatment of the lupus itself is necessary.
=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
Sunday, March 05, 2006
Lupus and the Kidney
Lupus and the Kidney
Alan G. Wasserstein, M.D.
LEARNING ABOUT LUPUS: A USER FRIENDLY GUIDEFrom the Lupus Foundation of Delaware Valley, Inc.Edited by: Mary E. Moore, Ph.D., M.D., Carolyn H. McGrory, MS, RN, Robert S. Rosenthal, M.D.
Lupus does not always cause kidney disease, but when it does, kidney involvement can be one of its most significant problems. In all of these cases, the kidney problems are due to the production of abnormal autoantibodies. These antibodies are directed against the patient's own tissue, for example, against DNA, the material of which the genes are made. The formation of immune complexes (combinations of these antibodies joining with normal body substances) appears to set up an inflammatory reaction in the kidney.
Several serious clinical disorders can result. The kidney may leak large amounts of protein, a condition called the nepbrotic syndrome. In the nephrotic syndrome, the ability of the kidneys to remove salt and water from the body is impaired. Excess fluid accumulates in the legs and abdomen and around the eyes, causing discomfort and inconvenience. Patients can not fit into their clothes or shoes. Diuretics are usually prescribed to help eliminate the excess fluid by increasing urination. The most feared complication is kidney failure. Kidney failure may or may not be preceded by nephrotic syndrome. Minor abnormalities in the results of urine or blood tests often provide the clue that damage to the kidney exists. Such abnormalities include excessive amounts of protein in the urine, red blood cells in the urine, or a slight loss of kidney function indicated by a rise in the serum creatinine (a blood study).
Sometimes kidney damage is more severe than the clinical symptoms reveal. A kidney biopsy should be done early if such problems are suspected. If kidney damage caused by lupus is diagnosed and treated early, the treatment is usually more effective. There is no doubt that the biopsy is the best way to determine the extent of disease and the need for treatment. After the kidney has been located with ultrasound or a CAT scan, a biopsy is done with the patient lying on his or her abdomen. Only a local anesthetic is required. A tiny cylinder of kidney tissue is withdrawn with a special needle. This tissue is examined under a microscope. Since there is a risk of bleeding from the kidney, the patient must stay in the hospital for observation for 12 hours or overnight following the biopsy.
A treatment plan is developed on the basis of the biopsy. Mild abnormalities are present in practically all lupus patients, even those with no other clinical or laboratory evidence of kidney involvement. These do not require treatment. Corticosteroids, such as prednisone, have been the basis of treatment for a long time. Their use may result in some improvement in lupus kidney disease, but the improvement is not as long lasting as we would like. (They are very effective, however, for other lupus problems such as arthritis, rash, fever, etc.) A significant advance in the treatment of lupus-related kidney disease has been the use of immunosuppressive drugs, specifically Cytoxan (cyclophosphamide).
Immunosuppressive drugs reduce the production of antibodies which cause inflammation. Cytoxan is usually given in combination with prednisone. In many cases it has led to clear improvement in lupus kidney disease. This has been demonstrated by observing improvement in biopsy specimens, reduction in the amount of protein in the urine, and increase in kidney function. Cytoxan is a toxic drug, however, which has many side effects. These include a decrease in the white blood cell count which makes a patient more susceptible to bacterial and other infections. It can also cause bleeding from the bladder (hemorrhagic cystitis), hair loss, sterility, and, years later, some patients may even develop cancer from its use. Sometimes kidney failure occurs very rapidly in patients with lupus (acute renal failure). in these cases, extremely large doses of corticosteroids and/or Cytoxan given intravenously (pulse therapy) may be helpful.
Patients who do not respond to treatment may progress to kidney failure, requiring dialysis or a kidney transplant. Although these are unfortunate outcomes, both dialysis and kidney transplants may have favorable results. Patients who have kidney failure which is advanced enough to require dialysis develop a natural immunosuppression and their lupus improves. As a result, dialysis patients with lupus do as well as other dialysis patients without systemic disease. Similarly, there has been success with kidney transplants in lupus patients. For the best outcome, a patient's lupus should be in remission at the time of the transplant. Kidneys for transplantation may come from either relatives or organ donors. In order to minimize the chance of rejection, the tissue type of the donated kidney should match the tissue type of the patient as closely as possible.
Cyclosporine, a new immunosuppressive drug given to kidney transplant patients, has significantly improved the success rate of transplants. Once the new kidney has been accepted by the body, recurrence of lupus in the kidney is rare.
=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
Alan G. Wasserstein, M.D.
LEARNING ABOUT LUPUS: A USER FRIENDLY GUIDEFrom the Lupus Foundation of Delaware Valley, Inc.Edited by: Mary E. Moore, Ph.D., M.D., Carolyn H. McGrory, MS, RN, Robert S. Rosenthal, M.D.
Lupus does not always cause kidney disease, but when it does, kidney involvement can be one of its most significant problems. In all of these cases, the kidney problems are due to the production of abnormal autoantibodies. These antibodies are directed against the patient's own tissue, for example, against DNA, the material of which the genes are made. The formation of immune complexes (combinations of these antibodies joining with normal body substances) appears to set up an inflammatory reaction in the kidney.
Several serious clinical disorders can result. The kidney may leak large amounts of protein, a condition called the nepbrotic syndrome. In the nephrotic syndrome, the ability of the kidneys to remove salt and water from the body is impaired. Excess fluid accumulates in the legs and abdomen and around the eyes, causing discomfort and inconvenience. Patients can not fit into their clothes or shoes. Diuretics are usually prescribed to help eliminate the excess fluid by increasing urination. The most feared complication is kidney failure. Kidney failure may or may not be preceded by nephrotic syndrome. Minor abnormalities in the results of urine or blood tests often provide the clue that damage to the kidney exists. Such abnormalities include excessive amounts of protein in the urine, red blood cells in the urine, or a slight loss of kidney function indicated by a rise in the serum creatinine (a blood study).
Sometimes kidney damage is more severe than the clinical symptoms reveal. A kidney biopsy should be done early if such problems are suspected. If kidney damage caused by lupus is diagnosed and treated early, the treatment is usually more effective. There is no doubt that the biopsy is the best way to determine the extent of disease and the need for treatment. After the kidney has been located with ultrasound or a CAT scan, a biopsy is done with the patient lying on his or her abdomen. Only a local anesthetic is required. A tiny cylinder of kidney tissue is withdrawn with a special needle. This tissue is examined under a microscope. Since there is a risk of bleeding from the kidney, the patient must stay in the hospital for observation for 12 hours or overnight following the biopsy.
A treatment plan is developed on the basis of the biopsy. Mild abnormalities are present in practically all lupus patients, even those with no other clinical or laboratory evidence of kidney involvement. These do not require treatment. Corticosteroids, such as prednisone, have been the basis of treatment for a long time. Their use may result in some improvement in lupus kidney disease, but the improvement is not as long lasting as we would like. (They are very effective, however, for other lupus problems such as arthritis, rash, fever, etc.) A significant advance in the treatment of lupus-related kidney disease has been the use of immunosuppressive drugs, specifically Cytoxan (cyclophosphamide).
Immunosuppressive drugs reduce the production of antibodies which cause inflammation. Cytoxan is usually given in combination with prednisone. In many cases it has led to clear improvement in lupus kidney disease. This has been demonstrated by observing improvement in biopsy specimens, reduction in the amount of protein in the urine, and increase in kidney function. Cytoxan is a toxic drug, however, which has many side effects. These include a decrease in the white blood cell count which makes a patient more susceptible to bacterial and other infections. It can also cause bleeding from the bladder (hemorrhagic cystitis), hair loss, sterility, and, years later, some patients may even develop cancer from its use. Sometimes kidney failure occurs very rapidly in patients with lupus (acute renal failure). in these cases, extremely large doses of corticosteroids and/or Cytoxan given intravenously (pulse therapy) may be helpful.
Patients who do not respond to treatment may progress to kidney failure, requiring dialysis or a kidney transplant. Although these are unfortunate outcomes, both dialysis and kidney transplants may have favorable results. Patients who have kidney failure which is advanced enough to require dialysis develop a natural immunosuppression and their lupus improves. As a result, dialysis patients with lupus do as well as other dialysis patients without systemic disease. Similarly, there has been success with kidney transplants in lupus patients. For the best outcome, a patient's lupus should be in remission at the time of the transplant. Kidneys for transplantation may come from either relatives or organ donors. In order to minimize the chance of rejection, the tissue type of the donated kidney should match the tissue type of the patient as closely as possible.
Cyclosporine, a new immunosuppressive drug given to kidney transplant patients, has significantly improved the success rate of transplants. Once the new kidney has been accepted by the body, recurrence of lupus in the kidney is rare.
=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
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