Symptoms Of Lupus

Lupus is a type of immune system disorder known as an autoimmune disease. In autoimmune diseases, the body harms its own healthy cells and tissues. This leads to inflammation and damage of various body tissues. Lupus can affect many parts of the body, including the joints, skin, kidneys, heart, lungs, blood vessels, and brain. Although people with the disease may have many different symptoms, some of the most common ones include extreme fatigue, painful or swollen joints (arthritis), unexplained fever, skin rashes, and kidney problems. Lupus is also known as a rheumatic disease. The rheumatic diseases are a group of disorders that cause aches, pain, and stiffness in the joints, muscles, and bones.

At present, there is no cure for lupus. However, the symptoms of lupus can be controlled with appropriate treatment, and most people with the disease can lead active, healthy lives. Lupus is characterized by periods of illness, called flares, and periods of wellness, or remission. Understanding how to prevent flares and how to treat them when they do occur helps people with lupus maintain better health. Intense research is underway and scientists funded by the NIH are continuing to make great strides in understanding the disease, which ultimately may lead to a cure.

Although "lupus" is used as a broad term, there actually are several kinds of lupus. Systemic lupus erythematosus (SLE) is the form of the disease that most people are referring to when they say "lupus." The word "systemic" means the disease can affect many parts of the body. The symptoms of SLE may be mild or serious. Although SLE usually first affects pe ople between the ages of 15 and 45 years, it can occur in childhood or later in life as well.

Two of the questions researchers are studying are who gets lupus and why. We know that many more women than men have lupus. Lupus is three times more common in black women than in white women and is also more common in women of Hispanic, Asian, and Native American descent. In addition, lupus can run in families, but the risk that a child or a brother or sister of a patient also will have lupus is still quite low. It is difficult to estimate how many people in the United States have the disease because its symptoms vary widely and its onset is often hard to pinpoint.

Lupus is a complex disease whose cause is unknown. It is likely that there is no single cause but rather a combination of genetic, environmental, and possibly hormonal factors that work together to cause the disease. The exact cause may differ from one person to another. Scientists are making progress in understanding the causes of lupus, as described here and in the Current Research section of this booklet. Research suggests that genetics plays an important role; however, no specific "lupus gene" has been identified. Instead, it appears that several genes may increase a person's susceptibility to the disease.

Some of the factors that scientists are studying include sunlight, stress, certain drugs, and infectious agents such as viruses. Even though a virus might trigger the disease in susceptible individuals, a person cannot "catch" lupus from someone else.

Fatigue is a nearly universal complaint of patients with SLE even when no other manifestations of the disease are present. The cause of this debilitating fatigue is not known. The patient should be evaluated for factors that may exacerbate fatigue, such as overexertion, insomnia, depression, stress, anemia, and other inflammatory diseases. Fatigue in SLE patients may be lessened by adequate rest, healthful diet, exercise, and attention to psychosocial factors.

Many patients with SLE experience changes in weight. At least one-half of patients report eight loss before being diagnosed with SLE. Weight loss in SLE patients may be attributed to a decreased appetite, side effects of medications, gastrointestinal problems, or fever. Weight gain can occur in some patients and may be due in part to prescribed medications, especially corticosteroids, or fluid retention from kidney disease.

Episodic fever is experienced by more than 80% of SLE patients, and there is no particular fever pattern. Although high fevers can occur during a lupus flare, low-grade fevers are more frequently seen. A complicating infection is often the cause of an elevated temperature in a patient with SLE. The patient's WBC count may be normal to elevated with an infection, but low with SLE alone. However, certain medications, such as immunosuppressives, will suppress the WBC even in the presence of fever. Therefore, it is important to rule out other causes of a fever, including an infection or a drug reaction. Urinary and respiratory infections are common in SLE patients. Psychological and emotional effects, such as grief, depression, and anger, are commonly experienced by lupus patients. These can be related to the outward changes, such as skin alterations, caused by the disease as well as by other aspects of the disease and its treatment. It is important for health professionals to be alert to potential psychological repercussions and to assist in alleviating them.

Approximately 80% of patients with SLE have skin manifestations and often suffer from itching, pain, and disfigurement. The classic sign of SLE is the "butterfly" rash extending over the cheeks (malar area) and bridge of the nose. This rash ranges from a faint blush to a severe eruption with scaling. It is photosensitive, and it may be transitory or fixed. Between 55 and 85% of patients develop this rash at some time in the course of the disease.

Other rashes may occur elsewhere on the face and ears, upper arms, shoulders, chest, and hands. Discoid lupus erythematosus (DLE) is seen in 15-30% of patients with SLE. Subacute cutaneous LE, seen in about 10% of SLE patients, produces highly photosensitive papules that itch and burn. Skin changes, especially the butterfly rash and subacute cutaneous LE, can be precipitated by sunlight.

Some patients may develop mouth, vaginal, or nasal ulcers. Hair loss (alopecia) occurs in about one-half of SLE patients. Most hair loss is diffuse, but it may be patchy. It can be scarring or nonscarring. Alopecia may also be caused by corticosteroids, infection, or immunosuppressive drugs.

Raynaud's phenomenon (paroxysmal vasospasm of the fingers and toes) frequently occurs in patients with SLE. For most patients, Raynaud?s phenomenon is mild. However, some SLE patients with severe Raynaud's phenomenon may develop painful skin ulcers or gangrene on the fingers or toes.

Arthralgia or arthritis is experienced by 95% of SLE patients at some time during the course of the disease. Articular pain is the initial symptom in about one-half of patients eventually diagnosed with SLE. Morning stiffness and joint and muscle aching can also occur. Joint pain may be migratory; it is typically symmetric but is asymmetric in many patients. Joints may become warm and swollen. X rays of the joints usually do not show erosion or destruction of bone.

Unlike rheumatoid arthritis, the arthritis of SLE tends to be transitory. Proliferation of the synovium is more limited, and joint destruction is rare. The joints most commonly involved are those of the fingers, wrists, and knees; less commonly involved are the elbows, ankles, and shoulders.

Abnormal blood conditions are common in patients with SLE. Problems include anemia, thrombocytopenia, and other clotting disorders. Blood vessels may become inflamed (vasculitis), affecting the way blood circulates through the body. The inflammation may be mild, and may not require treatment

Anemia, which is common in SLE patients, reflects insufficient bone marrow activity, shortened red blood cell life span, or poor iron uptake. Aspirin, NSAIDs, and prednisone can cause stomach bleeding and exacerbate the condition. There is no specific therapy for this type of anemia. Immune-mediated anemia (or hemolytic anemia), which is due to antibodies directed at RBCs, is treated with corticosteroids.

The following systems in the body also can be affected by lupus.

Kidneys:
Inflammation of the kidneys (nephritis) can impair their ability to effectively get rid of waste products and other toxins from the body. Because the kidneys are so important to overall health, lupus in the kidneys generally requires intensive drug treatment to prevent permanent damage. There is usually no pain associated with kidney involvement, although some patients may notice that their ankles swell. Most often the only indication of kidney disease is an abnormal urine test.

Central nervous system:
In some patients, lupus affects the brain or central nervous system. This can cause headaches, dizziness, memory disturbances, vision problems, stroke, or changes in behavior. Some of these symptoms, however, also can be caused by some treatments of lupus or by the emotional stress of dealing with the disease.

Lungs:
Some people with lupus develop pleuritis, an inflammation of the lining of the chest cavity that causes chest pain, particularly with breathing. Patients with lupus also may get pneumonia.

Heart:
In some people with lupus, inflammation can occur in the arteries that supply blood to the heart (coronary vasculitis), the heart itself (myocarditis and endocarditis), or the membrane that surrounds it (pericarditis), causing chest pains or other symptoms.

Diagnosing and treating lupus is often a team effort between the patient and several types of health care professionals. A person can go to his or her family doctor or internist, or can visit a rheumatologist. A rheumatologist is a doctor who specializes in arthritis and other diseases of the joints, bones, and muscles. Clinical immunologists (doctors specializing in immune system disorders) may also treat people with lupus. As treatment progresses, other professionals often help. These may include nurses, psychologists, social workers, and specialists such as nephrologists (doctors who treat kidney disease), hematologists (doctors specializing in blood disorders), dermatologists (doctors who treat skin disease), and neurologists (doctors specializing in disorders of the nervous system).

Several types of drugs are used to treat lupus. The treatment the doctor chooses is based on the patient's individual symptoms and needs. For people with joint pain, fever, and swelling, drugs that decrease inflammation, referred to as nonsteroidal anti-inflammatory drugs (NSAIDs), are often used. While some NSAIDs are available over the counter, a doctor's prescription is necessary for others. NSAIDs may be used alone or in combination with other types of drugs to control pain, swelling, and fever.

The mainstay of lupus treatment involves the use of corticosteroid hormones, such as prednisone (Deltasone), hydrocortisone, methylprednisolone (Medrol), and dexamethasone (Decadron, Hexadrol). Corticosteroids are related to cortisol, which is a natural anti-inflammatory hormone. They work by rapidly suppressing inflammation.

Antimalarials are another type of drug commonly used to treat lupus. These drugs were originally used to treat the symptoms of malaria, but doctors have found that they also are useful treatments for lupus. Exactly how antimalarials work in lupus is unclear, but scientists think that they may work by suppressing parts of the immune response. Specific antimalarials used to treat lupus include hydrochloroquine (Plaquenil), chloroquine (Aralen), and quinacrine (Atabrine).

People with lupus should receive regular preventive health care, such as gynecological and breast examinations. Regular dental care will help avoid potentially dangerous infections. If a person is taking corticosteroids or antimalarial medications, a yearly eye exam should be done to screen for and treat eye problems.

Staying healthy requires extra effort and care for people with lupus, so it becomes especially important to develop strategies for maintaining wellness. Wellness involves close attention to the body, mind, and spirit. One of the primary goals of wellness for people with lupus is coping with the stress of having a chronic disorder. Effective stress management varies from person to person. Some approaches that may help include exercise, relaxation techniques such as meditation, and setting priorities for spending time and energy.

The patient with lupus often has special nutritional needs related to medical conditions that may arise during the course of the disease. These conditions include steroid-induced osteoporosis or diabetes, cardiovascular disease, and kidney disease. For the SLE patient to maintain optimal health, the nurse must work closely with the patient, dietitian, and physician to develop a nutritional plan specific to the patient?s disease and manifestations.

With research advances and a better understanding of lupus, the prognosis for people with lupus today is far brighter than it was even 20 years ago. It is possible to have lupus and remain active and involved with life, family, and work. As current research efforts unfold, there is continued hope for new treatments; improvements in quality of life; and ultimately, a way to prevent or cure the disease. The research efforts of today may yield the answers of tomorrow, as scientists continue to unravel the mysteries of lupus.

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