Jose G. Veliz, M.D. M.S.A.
Palomar Pain Management Center
Posted: December 13, 2008
PAIN ASSOCIATED WITH LUPUS
In people with lupus, the immune system attacks healthy tissues and cells by mistake. The areas of the body affected include the following:
Common symptoms may include some or all of the following:
Swollen or Painful Joints
Loss of Hair
Swelling of the Eyes or Legs
Chest Pain with Deep Breathing (Pleuritic Chest Pain)
Purple Fingers or Toes
Periods of illness (flares) alternate with symptom free periods (remission). Currently, there is no cure for lupus.
There are different types of lupus and they include the following:
Systemic Lupus Erythematosus (SLE)
Discoid Lupus Erythematosus
Subacute Cutaneous Lupus Erythematosus
Drug Induced Lupus
Many systems in the body may be affected. They include the following:
Central Nervous System
This article will focus on pain associated with lupus.
During the course of their illness, approximately 90% of people with SLE (systemic lupus erythematosus) experience joint pain, muscle pain or nerve pain. Approximately 50% of the SLE patients present with painful joints when they are first diagnosed. We will review the different types of pain associated with this.
Inflammation of the joints is the major cause of joint pain in SLE. The following symptoms may occur in the affected joint:
Puffiness of the Hands
Multiple joints are usually involved at one time. These include:
Both sides of the body are usually affected (symmetrical).
Approximately 33% of patients with lupus experience recurrent attacks of arthritis. Fortunately, lupus arthritis is not as disabling as rheumatoid arthritis. There is less destruction of the joint with lupus arthritis. Approximately 5% of people with lupus arthritis develop deformities of the feet or hands (Jaccoud type deformities).
The best way to diagnose lupus arthritis is to study the pattern and distribution of the affected joints. In lupus arthritis, x-rays of the affected joints are usually normal. In addition, fluid removal (arthrocentesis) of the affected joint will show a low grade inflammation.
If lupus arthritis is the only symptom present, making a diagnosis may be difficult. Anti-nuclear antibodies (ANA) or rheumatoid factor (RF) may be present in the blood. A more specific blood test is the presence of anti-DNA and/or anti–SM in the blood.
Lupus arthritis may be treated with non-steroidal anti-inflammatory drugs (NSAIDs). These include:
These medications should be avoided in people with gastritis, peptic ulcer disease or kidney disease. If you have lupus, do not take NSAIDs without first consulting with your doctor.
Anti-malarial agents, such as hydroxy chloroquine (Plaquenil), are added when NSAIDs are not effective at controlling lupus arthritis.
If swelling and pain continue, corticosteroids (Prednisone) may be used.
Agents which suppress the immune system (immunosuppressant medications) may be effective in the treatment of lupus arthritis. These include:
Occupational therapy and physical therapy are integral in the treatment of lupus arthritis.
Avascular Necrosis of the Bone (AVN)
Although not directly associated with lupus, prolonged use of high doses of corticosteroids, in the treatment of lupus, can result in AVN. Microscopic breaks leads to weakening of the bone and, eventually, collapse of the bone surface. Knees, hips and shoulders are usually affected.
There is pain in the affected joints when running, walking or lifting.
Eventually, decreased range of motion and stiffness of the affected joint occurs.
X-ray or MRI of the affected joint can confirm the diagnosis of AVN. Although there are other causes of AVN, prolonged use of high doses of corticosteroids explains the connection between lupus and AVN.
Unfortunately, effective medical treatment is not available.
The following may decrease symptoms:
NSAIDs or Acetaminophen. These should be avoided in persons with kidney disease.
Low dose narcotics
Avoid prolonged walking or prolonged weightbearing
In moderate to severe cases, canes or crutches to decrease weightbearing of the affected joint, may decrease pain.
Every opportunity should be taken to decrease the dose of steroids in between flare-ups. This will decrease the probability of developing AVN.
In severe cases of AVN, surgery may decrease pain and improve function. Joint replacement is an example.
Carpal Tunnel Syndrome:
SLE is one of many medical conditions which can lead to carpal tunnel syndrome.
Symptoms include pain, numbness and tingling in certain fingers and are the result of pressure on the median nerve at the level of the wrist. Please refer to the article titled: "Carpal Tunnel Syndrome."
Approximately 50% of patients with SLE develop inflammation of the skeletal muscles (myositis) during flare-ups. Symptoms include muscle pain, muscle tenderness and muscle weakness. The muscles most commonly involved are the neck, shoulder girdle, upper arms, pelvic girdle and thighs. Initially, the symptoms are subtle and may include difficulty climbing stairs or getting out of a chair. Later, more simple activities of daily living may be affected such as combing one’s hair, raising one’s head and turning over in bed.
Tests used to diagnose lupus myositis include:
- Elevated blood level of specific enzymes (CPK, SGPT, SGOT and aldolase)
- Abnormal electromyogram (EMG) test which is a measurement of electrical activity of muscles.
- Inflammation or destruction of muscle fibers revealed by biopsy of the arm or thigh muscle.
The drug of choice, for the treatment of lupus myositis, is corticosteroids (Prednisone). Muscle inflammation decreases resulting in an increase of muscle strength. The blood levels of the enzyme described above usually return to normal after corticosteroids are given. Once symptoms are improved, the dose of corticosteroids is slowly decreased.
For patients who do not improve with corticosteroids, immunosuppressive agents that suppress the immune system are used. These include azathioprine or methotrexate.
Occupational therapy or physical therapy is vital to increase muscle strength and improve functionality.
Muscle Weakness Secondary to Medication Use:
Muscle weakness may occur secondary to the use of certain medications and not directly related to SLE itself. Examples of medication which may result in muscle weakness include:
Long term or high dose corticosteroids (Prednisone)
Drugs which lower cholesterol.
Discontinuing or decreasing the dose of these medications usually results in increased muscle strength. Do not discontinue or decrease your dose without consulting with your doctor.
Lupus can also affect the nervous system.
The nervous system includes the brain, spinal cord, muscle and peripheral nerves (nerves of the arms, legs and feet). Because the immune system creates antibodies which attack the brain, spinal cord muscles and peripheral nerves, neurological and psychological problems may occur.
Indirect Neurological and Psychological Problems:
Neurological problems may occur secondary to medications used to treat lupus. For example, immunosuppressants may increase the risk of infection such as meningitis. Meningitis, itself, can cause neurological problems.
As was discussed earlier, corticosteroids (Prednisone) are sometimes used to treat lupus. Prolonged use and high dose use of corticosteroids can cause anxiety, insomnia, and depression. Atrophy of the muscles, secondary to prolonged and high dose corticosteroid use, can lead to weakness.
Kidney and liver failure, secondary to lupus, can cause confusion.
Finally, anxiety and depression may occur as a result of psychological reactions to having a long term, chronic illness such as lupus.
The immune system attacking one’s own nerve cells is an example of a direct or primary effect on the nervous system. When the brain is affected, confusion, disorientation, agitation, psychosis and seizures may occur.
If psychosis develops, it is important to determine whether or not it is secondary or primary. Is it directly or indirectly related to the lupus? MRI scans and spinal fluid analysis can assist in differentiating primary from secondary causes of psychosis. For example, a flare up of lupus affecting the central nervous system may improve with corticosteroids, while anti-psychotic medications may be needed when symptoms are not related to a lupus flare.
Anti-phospholipid antibody is an antibody which can attack the brain in some people with lupus. Anti-phospholipid antibodies may increase the risk of the following:
Chorea – which is an involuntary twitching movement of the arms and legs.
Myelopathy – which is a dysfunction of the spinal cord leading to jerky type movements.
Treatment for lupus patients with anti-coagulant antibody may include blood thinners. This is different from treating lupus flare-ups in patients without anti-phospholipid antibody.
Peripheral Nerve Problems
Electromyograms (EMGs), which are electrical tests of muscles and nerve conduction studies which are electrical tests of nerves may reveal abnormalities in approximately 25% of patients with lupus. Half of those patients may develop symptoms which include weakness, numbness, tingling or burning of the hands or legs. This is called polyneuropathy or peripheral neuropathy. Treatment includes amitriptyline (Elavil), nortriptyline (Pamelor) gabapentin (Neurontin), pregabalin (Lyrica) or duloxetine (Cymbalta).
Similar to lupus arthritis, a polyneuropathy or peripheral neuropathy is symmetrical (affecting both sides of the body). On rare occasions, the neuropathy may not be symmetrical but, rather, patchy. This is called mononeuritis multiplex. This is the type which occurs when the immune system attacks the blood vessels of the body (vasculitis). The treatment of mononeuritis multiplex may require more aggressive treatment with corticosteroids and/or immunosuppressant drugs.
Schaefer J. How Lupus Can Affect the Nervous System. Adapted from A Talk at the SLE Workshop of the Hospital for Special Surgery.