General
Manifestations of SLE
Fatigue
is a nearly universal complaint of persons with Systemic Lupus Erythematosus
(SLE), even when no other manifestations of the disease are present. The
cause of this debilitating fatigue is not known. The person should be
evaluated for factors that may exacerbate fatigue, such as overexertion,
insomnia, depression, stress, anemia, and other inflammatory diseases.
Fatigue in SLE people may be lessened by adequate rest, healthful diet,
exercise, and attention to psychosocial factors.
Many people with SLE experience changes in weight. At least one-half of people report weight loss before being diagnosed with SLE. Weight loss in SLE persons may be attributed to a decreased appetite, side effects of medications, gastrointestinal problems, or fever. Weight gain can occur in some people 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 percent 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 person with SLE. The person's white blood count (WBC) 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 people.
Psychological and emotional effects, such as grief, depression, and anger, are commonly experienced by Lupus people. These can be related to the outward changes caused by the disease, such as skin alterations, 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.
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Dermatologic
Manifestations
Approximately 80 percent of people 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 (molar 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 percent of people 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 percent of people with
SLE. Subacute cutaneous LE, seen in about 10 percent of SLE people, 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 people. 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 vasopasm of the fingers and toes) frequently occurs in patients with SLE. For most people, Raynaud's phenomenon is mild. However, some SLE people with severe Raynaud's phenomenon may develop painful skin ulcers or gangrene on the fingers or toes.
Varying levels of pain and discomfort due to skin alterations may occur. Pruritus accompanies many types of skin lesions. Attacks of Raynaud's phenomenon can cause a deep tingling feeling in the hands and feet that can be very uncomfortable. Both pain and itching may affect a person's ability to carry out activities of daily living (ADL).
Skin alterations in the Lupus person, particularly those of DLE, can be disfiguring. As a result, people may experience fear of rejection by others, negative feelings about their body, and depression. Changes in lifestyle and social involvement may occur.
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Musculoskeletal
Manifestations
Anthralgia or arthritis is experienced by 95 percent of SLE people at
some time during the course of the disease. Articular pain is the initial
symptom in about one-half of people 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 people.
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.
Several joint complications may occur in SLE people, including Jaccoud's anthropathy and osteonocrosis. Subcutaneous nodules, especially in the small joints of the hands, are seen in about 5 percent of people. Tendinitis, tendon rupture, and carpal tunnel syndrome are seen occasionally.
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Hematologic
Manifestations
Abnormal blood conditions are common in people with SLE. Problems include
anemia, thrombocytopenia, and other clotting disorders.
Anemia, which is common in SLE people, reflects insufficient bone marrow activity, shortened Red Blood Count (RBC) 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.
Thrombocytopenia may occur and may respond to low-dose corticosteroids. Mild forms may not need to be treated, but a severe form requires high-dose corticosteroid or cytotoxic drugs. The major clinical features of Antiphospholipid Anitbodies (APLs) and APL syndrome are venous thrombosis, and thrombocytopenia with a history of positive anticardiolipin antibody (ACL) tests.
Abnormal laboratory tests may include a false-positive VDRL test for syphilis. Fluorescent treponemal antibody absorption (FTA-ABS) and microhemagglutination-Treponema pallidum (MHA-TP) tests, which are more specific tests for syphilis, are almost always negative if the person does not have syphilis. An elevated erythrocyte sedimentation rate (ESR) is a common finding in active SLE, but it does not always mirror disease activity.
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Cardiopulmonary
Manifestations
Cardiac abnormalities contribute significantly to morbidity and mortality
in SLE and are one of the most important clinical manifestations of the
disease. In addition, involvement of the lungs and pleurae is common.
Pericarditis, an inflammation of the pericardium, is the most common cardiac
abnormality in SLE. Myocarditis, an inflammation of the heart muscle,
may also occur, but is rare. Myocardial infarction, caused by artherosclerosis,
has been reported in SLE people below the age of 35 years.
Vasculitis (inflammation of the blood vessels) and serositis (inflammation of serous membranes) are frequently part of the autoimmune pathology of SLE. These conditions report well to corticosteroids. Vasculitis may cause many different symptoms, depending on the system(s) most affected. Serositis most commonly presents as pleurisy or pericarditis. Pleuritic chest pain is common. Pleurisy is the most common respiratory manifestation in SLE. Attacks of pleuritic pain can also be associated with pleural effusions. Many patients complain of chest pain, but pericardial changes are not often demonstrated on clinical evaluation.
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Renal
Manifestations
Renal damage is one of the most serious complications of SLE. The majority
of Lupus people have some degree of asymptomatic microscopic kidney damage.
Less than 50 percent have clinical renal disease, and most of those with
renal disease have one of the milder forms. Kidney damage may necessitate
treatment with corticosteroids, cytotoxic agents, dialysis, or renal transplantation.
Renal biopsy can be helpful in making decisions about drug treatments and determining prognosis by assessing the presence of active renal disease versus scarring.
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Central
Nervous System Manifestations
Neurologic manifestations of SLE are common and vary from mild to severe.
They can be difficult to diagnose and distinguish from other diseases.
All portions of the nervous system may be affected, including the CNS.
Definite diagnosis of CNS Lupus may be difficult, as symptoms may be related
to medications, other medical conditions, or to individual reactions to
chronic illness.
Cranial or peripheral neuropathy occurs in 10-15 percent of people; it is probably secondary to vasculitis in small arteries supplying nerves. Cerebrovascular accidents (strokes) are reported in approximately 15 percent of people. Between 10 and 20 percent of people experience seizures. Although cognitive impairment is believed to be very common, there are few measurements to document it.
Serious CNS involvement ranks behind only kidney disease and infection as a leading cause of death in Lupus. However, the majority of SLE people with CNS complications do not develop a life-threatening disease.
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Gastrointestinal
Manifestations
Gastrointestinal (GI) problems are common and range from vague complaints
of anorexia to life-threatening bowel perforation secondary to mesenteric
arteritis. Anorexia, nausea, vomiting, and diarrhea may be related to
the use of salicylates, NSAIDs, antimalarials, corticosteroids, and cytotoxic
drugs.
SLE people who present with acute abdominal pain and tenderness need immediate, aggressive, and comprehensive evaluation to rule out an intra-abdominal crisis. Ascites, an abnormal accumulation of fluid in the peritoneal cavity, is found in about 10 percent of SLE people. Pancreatitis is a serious complication occurring in approximately 5 percent of SLE people and is usually secondary to vasculitis.
Mesenteric or intestinal vasculitis is a life-threatening condition that may have complications of obstruction, perforation, or infarction. They are seen in more than 5 percent of people with SLE. Abnormal liver enzyme levels are also found in about one-half of SLE people (usually secondary to medications). Active liver disease is rarely found.
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Ophthalmologic
Manifestations
Visual impairment may be due to SLE or to drug treatment (corticosteroids
or antimalarials) or it may be a separate problem (glaucoma or retinal
detachment). Blindness due to SLE occurs, but is rare. Other visual problems
may occur:
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A Lupus rash may develop on the eyelids | |
Conjunctivitis occurs in 10 percent of SLE people and is usually infectious. Kerato-conjunctivitis is usually mild | ||
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Cytoid bodies are the most common retinal change in SLE. They reflect microangiopathy of the retinal capillaries and localized microinfarction of the superficial nerve fiber layers of the retina | |
Sjogren's syndrome is an autoimmune condition manifest as excessive dryness of mucous membranes. Lupus persons with these symptoms require artificial tears to relieve dry eyes | ||
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Glaucoma and cataracts may be caused by corticosteroids | |
Antimalarials can damage the retina, which can impair vision (particularly color vision) or, in rare instances, cause blindness | ||
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Pregnancy
Twenty years ago, women with Lupus were counseled not to become pregnant
because of the risk of a flare of the disease and an increased risk of
miscarriage. Research and careful treatment have made it possible for
more and more women with Lupus to have successful pregnancies. Although
a Lupus pregnancy is still considered high risk, most women with Lupus
are able to carry their babies safely to term. Experts disagree on the
exact numbers, but approximately 20-25 percent of Lupus pregnancies end
in miscarriage, compared with 10-15 percent of pregnancies in women without
the disease. Pregnancy counseling and planning before pregnancy is important.
Optimally, a woman should have no signs or symptoms of Lupus before she
becomes pregnant.
Researchers have now identified two closely related Lupus autoantibodies, anticardiolipin antibody and Lupus anticoagulant, that are associated with risk of miscarriage. One-third to one-half of women with Lupus have these autoantibodies, which can be detected by blood test. Identifying women with the autoantibodies early in the pregnancy may help physicians take steps to reduce the risk of miscarriage. Pregnant women who test positive for these autoantibodies and who have had previous miscarriages are generally treated with baby aspirin or heparin throughout their pregnancy.
Some women may experience a mild to moderate flare during or after their pregnancy; others may not. Pregnant women with Lupus, especially those taking corticosteroids, are also likely to develop pregnancy-induced hypertension, diabetes, hyperglycemia, and kidney complications. About 25 percent of babies of women with Lupus are born prematurely, but do not suffer from birth defects.
About 3 percent of babies born to mothers with SLE will have neonatal Lupus, or specific antibodies called anti-Ro(SSA) and anti-La(SSB). This is not the same as SLE and is almost always temporary. The syndrome is thought to be caused by passive transfer of anti-Ro antibodies from the mother to the fetus. About one-third of women with SLE have this antibody. By 3-6 months of age, the rash and blood abnormalities associated with neonatal Lupus disappear. Very rarely, babies with neonatal Lupus will have a congenital complete heart block. This problem is permanent, but can be treated with a pacemaker.
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Infection
SLE affects the immune system, thus reducing the body's ability to prevent
and fight infection. In addition, many of the drugs used to treat SLE
also suppress the function of the immune system, thereby further depressing
the ability to fight infection. The risk of infection parallels medication
dosages and duration of treatment.
People with SLE who show signs and symptoms of infection need prompt therapy to prevent it from becoming life threatening. The most common infections involve the respiratory tract, urinary tract, and skin and do not require hospitalization if they are treated promptly. Other opportunistic infections, particularly Salmonella, Herpes Zoster, and Candida infections, are more common in people with SLE because of altered immune status.
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Nutrition
The person 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 person to maintain optimal health, they
must work closely with the dietitian and physician to develop a nutritional
plan specific to the person's disease and manifestations.
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