DRG Category: 546
Mean LOS: 4.4 days
Description MEDICAL Connective Tissue Disorders With Complication or Comorbidity
DRG Category: 595
Mean LOS: 7.5 days
Description MEDICAL Major Skin Disorders With Major Complication or Comorbidity
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 (2021) 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 10 years, and 80% at 15 years. Because of recent advances in treatment, many people are living 25 or 30 years after diagnosis. The most common causes of death are renal failure and infections, followed by neurological and cardiovascular disorders.
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.
SLE occurs most frequently in females between the ages of 15 and 44 years, with the average age of onset at 30 years. Approximately 90% of people with SLE are women. DLE is more common in women than in men, and approximately 60% of cases are female patients in their late 20s or older.
Lupus erythematosus is more prominent in Black, Native American, Asian, and Hispanic persons as compared to White persons. Native American and Black persons with SLE have the highest mortality rates. People living in poverty have poorer disease outcomes and higher mortality rates than people with low, middle, and high incomes. Low educational attainment is also associated with increased mortality (Peschken, 2020). Lupus nephritis, one of the most serious complications of SLE, has the highest frequency among Black and Hispanic persons. Native American children are three times more likely than children of other groups to develop SLE. People from minority groups and people who are on Medicare have difficulties with accessing primary care and speciality care for SLE and are more likely to require hospitalization than other groups (Brown et al., 2020). Sexual and gender minority status has no known effect on the risk for SLE and DLE.
The Lupus Foundation of America (2021) estimated that at least 5 million people have a form of lupus. 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 hematological 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 if the patient 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. Assess for neurological changes that may include headache, changes in mental status, and seizure activity.
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 patients. 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 a lifelong chronic illness that may affect many organ systems. Determine the level of anxiety, fear, and depression.
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 status; Knowledge: Pain management; Pain level; Pain control; Symptom severity; Knowledge: Medication; Medication response
Interventions: Analgesic administration; Anxiety reduction; Pain management: Acute; Medication management; Medication administration; Teaching: Prescribed medication
Much of the therapy is pharmacologic and guided by the patient’s symptoms. 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||Nonbiological DMARDs: Cyclophosphamide, methotrexate, azathioprine, mycophenolate, cyclosporine; biological 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 hairstylist 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.
Castellano-Rioja, E., Giménez-Espert, M., & Soto-Rubio, A. (2020). Lupus erythematosus quality of life questionnaire (LEQoL): Development and psychometric properties. International Journal of Environmental Research and Public Health. Advance online publication. https://doi.org/10.3390/ijerph17228642 [PMID:33233805]
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 of America, and other appropriate support groups that are available in the area.