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Medical Disability Advisor  >  Lupus Erythematosus Systemic

Lupus Erythematosus, Systemic


Differential Diagnoses


Specialists


  • Cardiovascular Internist
  • Clinical Psychologist
  • Dermatologist
  • Nephrologist
  • Neurologist
  • Psychiatrist
  • Rheumatologist

Comorbid Conditions


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Factors Influencing Duration


Length of disability depends on the severity of symptoms and the organs involved. Treatment, particularly prolonged corticosteroid therapy, may produce side effects that can also cause disability.

Medical Codes


ICD-9-CM:
710.0 - Diffuse Diseases of Connective Tissue, Lupus Erythematosus, Systemic; Disseminated Lupus Erythematosus; Libman-Sacks Disease
710.9 - Diffuse Diseases of Connective Tissue, Unspecified

Definition


Systemic lupus erythematosus (SLE, or lupus) is an ongoing (chronic) inflammatory connective tissue disease in which the body's immune system malfunctions and attacks healthy tissue. SLE is a serious and potentially fatal condition that can inflame and damage the skin, joints, kidneys, heart, lungs, brain, nervous system, and mucous membranes and cause various associated symptoms. It is characterized by periods when symptoms lessen or disappear without a known cause (spontaneous remissions) and periods when symptoms return or flare (relapses).

The number and variety of antibodies that appear in lupus determine what symptoms will develop, although the level of antibodies is not always proportional to an individual's symptoms.

Individuals using certain medications may develop a lupus-like syndrome. The drugs most frequently responsible are those used to treat high blood pressure (antihypertensives), irregular heart rhythms (cardiac depressants), and tuberculosis (tuberculostatics). Symptoms of drug-induced lupus are generally milder and fade when the drug is discontinued.

The cause of lupus is not known.

Risk: Risk factors include sex, race, heredity, and exposure to certain medications or viruses. Sex hormones appear to play some role because most cases develop during the childbearing years. In the US, black women appear to be at increased risk. Family members of affected individuals may also be at increased risk. Approximately 90% of individuals with SLE are young women in their late teens to 30s, but the disease can also affect children (primarily girls), older women, and men (Schumacher 378). Drug-induced lupus affects men and women equally.

Incidence and Prevalence: On average, about 1 in 10,000 people in the US get lupus every year (Hildebrand). More people have lupus than AIDS, multiple sclerosis, cerebral palsy, sickle cell anemia, and cystic fibrosis combined (Batiste). The condition is considered more common in China, in Southeast Asia, and among blacks in the Caribbean.

Source: Medical Disability Advisor



History


History: The severity of symptoms varies in different individuals and within the same individual over time. Early symptoms may include joint or muscle pain, fever, or migraine-type headaches. Individuals may also report loss of appetite (anorexia); abdominal pain; swollen glands; sensitivity to cold in the fingers, toes, nose, and ears (Raynaud's phenomenon); feelings of stress and anxiety; weight loss; or a general feeling of ill health (malaise). Visual disturbances may occur, including over-sensitivity to light (photophobia) or blurred vision. The person may feel pain when taking deep breaths. Most individuals will complain of a skin rash at some time, particularly after exposure to sunlight.

Physical exam: The hallmark symptom of SLE is the characteristic red, blotchy, butterfly-shaped rash over the cheeks and bridge of the nose. Individuals with SLE may also present with ulcers in the mouth, hair loss, and red, swollen, painful joints. If more systems are involved, symptoms are related to inflammation in the involved organs. Common findings include inflammation of the lining of the lungs (pleurisy) and of the membrane surrounding the heart (pericarditis). Scattered abdominal tenderness may be due to inflammation of the membrane lining the abdominal cavity that is not related to infection (sterile peritonitis). High blood pressure (hypertension) and ankle swelling (peripheral edema) are the most common indications of kidney involvement (renal disease).

Tests: No one test can determine lupus, but various laboratory tests can help confirm the diagnosis. Anemia detected on a complete blood cell count (CBC) may be caused by chronic inflammation, renal disease, or destruction of red blood cells (hemolysis). Increased urea nitrogen or creatinine on a blood chemistry or an abnormal urinary sediment indicates kidney involvement. Blood tests used to detect specific antibodies include ANA (antinuclear antibody test), the anti-DNA antibody test, the anti-Sm antibody test, and complement levels. Removal of tissue for microscopic examination (skin biopsy) can also detect antibodies that occur when the disease is active. X-rays of painful joints, muscle testing through electromyography (EMG), and microscopic examination of fluid withdrawn from the joints may be done to rule out other disease processes. Chest x-rays may be taken to detect lung damage, and an electrocardiogram (ECG) can detect heart problems. CT or MRI can be used to evaluate CNS involvement.

Source: Medical Disability Advisor



Treatment


There is no cure for SLE. Treatment depends on which organs are affected and whether lupus is mild or severe and is aimed at reducing inflammation and relieving symptoms. Mild lupus may not require treatment. Any drug that may have triggered lupus should be withdrawn, if possible. Individuals whose symptoms are made worse by sunlight should avoid exposure to sunlight and use sunscreen. The skin rash can often be effectively treated with antimalarial drugs, which are also indicated for disease-related fever or pleurisy. Inflammation of the joints (lupus arthritis) is treated with nonsteroidal anti-inflammatory (NSAID) drugs or antimalarial drugs. Disease-caused muscle inflammation (lupus myositis) can be treated with corticosteroids and an effective exercise program. Corticosteroids may also be prescribed for treatment of neurological symptoms, kidney disease, or severe cases of lupus. Anticancer (cytotoxic) drugs are reserved for serious systemic involvement, especially when corticosteroids have been tried but are ineffective. If the kidneys fail, mechanical support (dialysis) may be required. Psychological needs must be addressed through counseling or other support.

Source: Medical Disability Advisor



Prognosis


The prognosis for individuals with SLE has improved over the past two decades. Females with disease onset after age 60 have the most favorable prognosis. Children with SLE have the least favorable prognosis. However, because the course of lupus is unpredictable, the prognosis may vary. About half of all individuals with lupus experience kidney involvement, which can lead to life-threatening conditions. The 10-year survival rate is now 70% to 90% (Greenspun). Factors aiding this improved prognosis include a more accurate diagnosis, faster and more effective treatment, availability of dialysis for treatment of kidney failure, and more availability of antibiotics effective in treating infectious complications. In most individuals, the illness pursues a mild, ongoing (chronic) course, occasionally interrupted by relapses of disease activity. For many, the disease may affect only a few organs.

Source: Medical Disability Advisor



Complications


Possible complications include serious impairment of vital organs such as the lungs, heart, brain, or kidneys. Infections that occur due to a compromised immune system (opportunistic infections) are a leading cause of death. Treatment with antimalarial drugs can damage the back inner portion of the eye where focusing occurs (retina). Corticosteroid treatment may cause facial swelling or high blood pressure (hypertension). Other infections such as colds or influenza may worsen symptoms.

Source: Medical Disability Advisor



Return to Work (Restrictions / Accommodations)


Restrictions and accommodations are determined by the severity of symptoms and the particular organ or system involved. Flexibility and adaptability are necessary because cycles of remission and relapse may hamper the individual's ability to continue long-term tasks. Individuals with lupus may require protection from sunlight provided by sunscreens and protective clothing such as hats.

Source: Medical Disability Advisor



Failure to Recover


If an individual fails to recover within the expected maximum duration period, the reader may wish to consider the following questions to better understand the specifics of an individual's medical case.

Regarding diagnosis:

  • Has diagnosis of systemic lupus erythematosus (SLE) been confirmed?
  • If diagnosis is uncertain, have other conditions with similar symptoms been ruled out?
  • Is there a family history of SLE or other connective tissue disorders?
  • Does individual report spontaneous remissions and relapses?
  • Has individual experienced complications such as impairment of vital organs (lungs, heart, brain, or kidneys), opportunistic infections, retinal damage, or hypertension that may affect recovery?

Regarding treatment:

  • Because there is no cure, has treatment been aimed at reducing inflammation and relieving symptoms?
  • If symptoms are made worse by sunlight, is individual avoiding exposure to sunlight? Does individual use sunscreen?
  • If condition was triggered by a drug, has that drug been withdrawn? Can another drug be substituted?
  • Have antimalarial drugs been effective against skin rash?
  • Was an appropriate exercise program recommended? Is individual complying with exercise plan?
  • If corticosteroids were ineffective against a serious systemic involvement, is use of cytotoxic drugs indicated?
  • Has individual experienced kidney failure? If dialysis was necessary, are kidneys functional again?
  • Are individual's psychological needs being met through counseling or other support?

Regarding prognosis:

  • If symptoms persist despite treatment, does diagnosis need to be revisited?
  • Does individual have access to factors indicating an improved prognosis, such as faster and more effective treatment, availability of dialysis for treatment of kidney failure, and more availability of antibiotics effective in treating infectious complications?
  • Does individual have a comorbid condition such as kidney disease, hepatitis, or multiple sclerosis that may complicate treatment and affect recovery?

Source: Medical Disability Advisor



Cited References


Batiste, Linda Carter, and Beth Loy. "Accommodating People with Lupus." U.S. Department of Labor. U.S. Department of Labor. 12 Dec. 2004 <http://www.jan.wvu.edu/media/Lupus.html>.

Greenspun, Bertram. "Systemic Lupus Erythematosus." eMedicine. Eds. Martin K. Childers, et al. 5 Oct. 2004. Medscape. 12 Dec. 2004 <http://emedicine.com/pmr/topic135.htm>.

Hildebrand, Julie, and Daniel Muller. "Systemic Lupus Erythematosus." eMedicine. Eds. Carlos J. Lozada, et al. 1 Oct. 2004. Medscape. 12 Dec. 2004 <http://emedicine.com/med/topic2228.htm>.

Schumacher, Ralph H. "Systemic Lupus Erythematosus." The Merck Manual of Home Health Care. Ed. Mark H. Beers. 2nd ed. Whitehouse Station, NJ: Merck Research Laboratories, 2003. 378-380.

Source: Medical Disability Advisor






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