DRG Category: 546
Mean LOS: 4.7 days
Description MEDICAL: Connective Tissue Disorders With CC
DRG Category: 595
Mean LOS: 7.6 days
Description MEDICAL: Major Skin Disorders With Major CC
Nursing Type Primary: acute care
Nursing Type Secondary: gender health
System Primary: immune
System Secondary: not applicable
Lupus erythematosus is an autoimmune disease that affects the connective tissue of the body as well as the kidney, blood cells, and nervous system. The Lupus Foundation of America reports that 1.5 million people have lupus in the United States, with more than 16,000 new cases reported each year. The course of disease is variable and unpredictable, with episodes of remission and relapse. Only a small percentage of patients (< 10%) have long-lasting remissions.
Lupus takes two forms. Systemic lupus erythematosus (SLE) is a multisystem inflammatory disease that affects any body system but primarily the musculoskeletal, cutaneous, renal, nervous, and cardiovascular systems. People with SLE develop an autoantibody response to proteins (antigens) in the nucleus and cytoplasm of body cells leading to inflammation and the formation of circulating immune complexes in the capillaries. These complexes are deposited in the basement membranes of the skin and kidneys, disrupting the function of these organs. Discoid lupus erythematosus (DLE) is a less serious form of the disease that primarily affects the skin. DLE is characterized by skin lesions of the face, scalp, and ears. Longstanding lesions can cause scarring, hypopigmentation, and redness. Only 5% to 10% of patients with DLE develop SLE. The multisystem nature of SLE places the patient at risk for multiple complications, and the disease is ultimately fatal. The survival rates of patients with SLE are about 90% at 5 years and 80% at 10 years. The most common causes of death are renal failure and infections, followed by neurological and cardiovascular disorders. In the last decades, therapy has reduced mortality from lupus, with 5-year survival rates of more than 90% and 20-year survival rates of 70%.
The cause of lupus erythematosus is not known. A familial association has been noted that suggests a genetic predisposition, but a genetic link has not been identified. Approximately 8% of patients with SLE have at least one first-degree family member (parent, sibling, child) with the disease. Environmental factors, susceptibility to certain viruses, and an immune system dysfunction with production of autoantibodies are possible causes. Hormonal abnormality and ultraviolet radiation are considered possible risk factors for the development of SLE. Some drugs have been implicated as initiating the onset of lupus-like symptoms and aggravating existing disease; they include hydralazine hydrochloride, procainamide hydrochloride, penicillin, isonicotinic acid hydrazide, chlorpromazine, phenytoin, and quinidine. Possible childhood risk factors include low birth weight, preterm birth, and exposure to farming pesticides.
While familial clustering of lupus erythematosus is relatively rare, twin studies have shown concordance in 24% to 59% of identical twins and in only 2% to 5% of nonidentical twins, which suggests a significant genetic predisposition. Heritability is estimated at 43%, and there are now over 80 loci that are associated with lupus erythematosus. Genes encoding proteins of the complement system appear to have the strongest association with lupus erythematosus. Polymorphisms in the toll-like receptor-5 (TLR5) gene are also associated with SLE.
Gender, Ancestry, and Life Span Considerations
SLE occurs most frequently in females between the ages of 15 and 45, with the average age of onset at 30 years. Approximately 90% of people with SLE are women; it is 15 times more likely to occur in women during childbearing years than in men. It is also more prominent in people with Asian, Latino/Hispanic, and African American ancestry than in other populations. DLE is more common in women than in men, and approximately 60% of cases are female patients in their late 20s or older.
Global Health Considerations
Global data on prevalence vary widely. Prevalence is higher in white people living in Western Europe and in people from the Caribbean living in Europe, and it is lower in Africa and China.
Initial symptoms may involve one organ only or multiple systems. Symptoms vary from mild and infrequent to persistent and life-threatening. Take a careful history with a focus on both systemic and single-organ symptoms. Systemic symptoms include fatigue, malaise, weight loss, anorexia, and fever.
The patient may report musculoskeletal and cutaneous symptoms, including joint and muscle pain, puffiness of hands and feet, joint swelling and tenderness, hand deformities, and skin lesions such as the characteristic "butterfly rash" (fixed reddish and flat rash that extends over both cheeks and the bridge of the nose). Other symptoms may include maculopapular rash (small, colored area with raised red pimples), sensitivity to the sun, photophobia, vascular skin lesions, leg ulcers, oral ulcers, and hair loss.
Other symptoms originate in the genitourinary tract (menstrual abnormalities, amenorrhea, spontaneous abortion) or central nervous system (visual problems, memory loss, mild confusion, headache, seizures, psychoses, loss of balance, depression). Establish a history of symptoms related to the hematologic system (venous or arterial clotting, bleeding tendencies), cardiopulmonary system (chest pain, shortness of breath, lung congestion), or gastrointestinal system (nausea, vomiting, difficulty swallowing, diarrhea, and bloody stools).
Ask if there is a family history of SLE. Establish any immune system dysfunction or recent viral infections. Ask if the patient has a history of hormonal abnormality or ultraviolet radiation. Ask the patient if he or she is taking or has taken any of the medications implicated as initiating lupus-like symptoms.
Common symptoms include fever, joint pain, and rash. Inspect the integumentary system thoroughly, including the mucous membranes, to determine the site of skin rashes and lesions. Check for lesions and necrosis on the fingertips, toes, and elbows; these may be caused by inflammation of terminal arterioles. Examine the hairline for any signs of hair loss. Assess the patient's extremities and joints for signs of arthritis, lymphadenopathy, and peripheral neuropathy. Determine the extent of range of motion and movement of extremities and level of joint discomfort. Auscultate the lungs and heart to determine the presence of a pleural or pericardial friction rub. Palpate the spleen and liver to determine the presence of tenderness, splenomegaly, or hepatomegaly. Examine the patient's urine for hematuria, proteinuria, and casts.
Assess for fever, pallor, and signs of bleeding, including petechiae and bruising. Check the patient's blood pressure because increased blood pressure might indicate kidney involvement.
A patient is facing a chronic and often debilitating disease that can be fatal. The patient may have problems maintaining professional and family roles and may experience loss over a deteriorating health status. The loss of childbearing potential is another loss experience for some women. Lupus is associated with an increased incidence of spontaneous abortion, fetal death, and prematurity. Assess the patient's and family's ability to cope with the illness. Determine the level of anxiety, fear, and depression.
Tests: Complete blood count, erythrocyte sedimentation rate, antinuclear antibody, urinalysis, anti-DNA antibody, complement levels, anti–double-stranded DNA antibody assay, blood urea nitrogen, creatinine, creatinine clearance, creatine kinase assay, liver function tests. To make the diagnosis of lupus, the American College of Rheumatology developed the following list. The patient needs to have at least four of the criteria (including one clinical and one immunological) using the mnemonic SOAP BRAIN MD:
|Serositis||Pericarditis, pleurisy on electrocardiogram or imaging scan|
|Oral ulcers||Sores, usually painless, on the lips and in the mouth|
|Arthritis||Tenderness or swelling of two or more peripheral joints|
|Photosensitivity||Unusual skin reaction (skin rash) to sun exposure|
|Blood disorder||Leukopenia, lymphopenia, thrombocytopenia, hemolytic anemia|
|Renal involvement||Proteinuria, cellular casts|
|Antinuclear antibodies||Elevated titers|
|Immune phenomenon||Presence of antibodies or lupus erythematosus cells|
|Neurological disorder||Seizures or psychosis in absence of other causes|
|Malar rash||Fixed erythema over cheeks and nose|
|Discoid rash||Raised, red lesions with scaling and follicular plugging|
Primary Nursing Diagnosis
Diagnosis: Acute pain related to joint or peripheral nerve inflammation or dysfunction as evidenced by self-reports of pain, facial grimacing, and/or protective behavior
Outcomes: Comfort level; Pain control behavior; Pain level; Symptom severity
Interventions: Analgesic administration; Anxiety reduction; Environmental management: Comfort; Pain management; Medication management; Teaching: Individual; Prescribed activity/exercise
Much of the therapy is pharmacologic. General supportive therapy includes adequate sleep and avoidance of fatigue because mild disease exacerbations may subside after several days of bedrest. A physical therapy program is important to maintain mobility and range of motion without allowing the patient to get overtired. If the kidneys are involved, renal dialysis or transplantation may be required.
|Medication or Drug Class||Dosage||Description||Rationale|
|Hydroxychloroquine (Plaquenil)||400–600 mg PO daily for 5–10 days, gradually increasing dose until effective; maintenance is usually 200–400 mg/day PO||Antimalarial||Reduces rash, photosensitivity, arthralgias, arthritis, alopecia, and malaise|
|Corticosteroids||Varies with drug||Prednisone, 1–2 mg/kg PO qd; methylprednisolone 500 mg IV||Control SLE in most severe or life-threatening cases (glomerulonephritis, debilitation from symptoms)|
|Disease-modifying antirheumatic drugs (DMARDs)||Varies with drug||Nonbiologic DMARDs: Cyclophosphamide, methotrexate, azathioprine, mycophenolate, cyclosporine; Biolgic DMARDs: belimumab||Suppress immune system and reduce consequences of disease|
|NSAIDs||Varies with drug||Diclofenac, ibuprofen, naproxen||Treat the joint pain and swelling; should be avoided in patients with active nephritis|
Other Drugs: Monoclonal antibody (rituximab [Rituxan]). Topical steroids are often used to treat skin rashes. Anticonvulsants may be necessary if seizures occur.
The pain and discomfort of SLE can be physically and mentally debilitating. Encourage the patient to maintain activity when the symptoms are mild or in remission. Encourage patients to pace all activity and to allow for adequate rest. Hot packs may relieve joint pain and stiffness. If the patient has Raynaud phenomenon (abnormal vasoconstriction of the extremities), use warmth to relieve symptoms and protect the patient's hands from injury.
Support the patient's self-image by encouraging good grooming. Suggest hypoallergenic cosmetics, shaving products, and hair products. Encourage the patient to use a hair stylist or barber who specializes in caring for people with scalp disorders and to protect all body surfaces from direct sunlight. The patient should use sunscreen with a protective factor of at least 20 and wear a hat and long sleeves while in the sun. Note that certain drugs (tetracycline) and foods (figs, parsley, celery) augment the effects of ultraviolet light and therefore should be avoided.
Fatigue and stress can lead to exacerbations of the illness. Explore ways for the patient to get adequate rest. Because the patient's immune system may have a diminished capacity, encourage the patient to avoid exposure to illness.
Explore the meaning of the chronic illness and coping strategies with the patient. Allow adequate time to discuss fears and concerns. A referral to a support group or counselor may also be necessary.
Evidence Based Practice Health Policy
Morgan, C., Bland, A.R., Maker, C., Dunnage, J., & Bruce, I.N. (2018). Individuals living with lupus: Findings from the LUPUS UK members survey 2014. Lupus, 27, 681–687. The authors mailed an anonymous survey to 5,660 people with SLE who are members of a charity that supports people with SLE in the United Kingdom. A total of 2,527 surveys were returned; 94% of the surveys were returned by females. The most common self-reported symptoms that interfered with daily life were fatigue/weakness (91%) and joint/pain swelling (77.4%). Seventy-three per cent acknowledged having problems from their symptoms as they carried out their usual daily activities. Thirty-two per cent of the respondents were using non-traditional therapies such as acupuncture and massage to manage their symptoms.
- Physical changes: Vital signs, particularly blood pressure and temperature; daily weight, intake and output; signs of bleeding or tarry stools, petechiae, bruising, pallor
- Physical changes: Location site and description of any skin lesions or rashes and overall condition of the skin
- Physical changes: Presence of any seizure activity, visual disturbances, headaches, personality changes, or memory deficits
- Tolerance to activity, level of pain and fatigue, patient's ability to perform activities of daily living and range of motion of extremities; note the extent of joint involvement and the presence of tingling, numbness, or weakness
Discharge and Home Healthcare Guidelines
Teach the patient the purpose, dosage, and possible side effects of all medications. Explain to the patient the disease process, the purpose of treatment regimens, and the importance of compliance. Teach the patient when to seek medical attention. Teach the patient to wear a Medic Alert bracelet noting the disease and medications so appropriate action can be taken in an emergency. Recommend smoking cessation for patients who use tobacco. Encourage the patient to keep all vaccinations current such as the meningococcal vaccine, pneumococcal vaccine, and routine flu vaccines.
Teach the female patient the importance of planning pregnancies with medical supervision because pregnancy is likely to cause an exacerbation of the disease.
Discuss all precipitating factors that need to be avoided, including fatigue, vaccination, infections, stress, surgery, certain drugs, and exposure to ultraviolet light. Teach the patient how to minimize ultraviolet exposure. Teach the patient to avoid strenuous exercise, instead striving for a balance. Describe pain management strategies. Stress the importance of adequate nutrition. Small, frequent meals may be better tolerated. Any cosmetics should be approved by the physician and should be hypoallergenic. Encourage the patient to contact the Arthritis Foundation, the Lupus Foundation, and other appropriate support groups that are available in the area.
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