Systemic Lupus Erythematosus - Diffuse Connective Tissue Diseases

SYSTEMIC LUPUS ERYTHEMATOSUS

The overall prevalence of SLE is estimated to be 100 per 100,000 persons. It occurs 10 times more frequently in women than in men and approximately three times more frequently in the African-American population than in Caucasians (Ruddy et al., 2001).

Pathophysiology

SLE is a result of disturbed immune regulation that causes an exaggerated production of autoantibodies. This immuno-regulatory disturbance is brought about by some combination of genetic, hormonal (as evidenced by the usual onset during the childbearing years), and environmental factors (sunlight, thermal burns). Certain medications, such as hydralazine (Apresoline), procainamide (Pronestyl), isoniazid (INH), chlorpromazine (Thorazine), and some antiseizure medications, have been impli-cated in chemical or drug-induced SLE.

In SLE, the increase in autoantibody production is thought to result from abnormal suppressor T-cell function, leading to im-mune complex deposition and tissue damage. Inflammation stim-ulates antigens, which in turn stimulate additional antibodies, and the cycle repeats.

Clinical Manifestations

The onset of SLE may be insidious or acute. For this reason, SLE may remain undiagnosed for many years. Clinical features of SLE involve multiple body systems.

SYSTEMIC MANIFESTATIONS

SLE is an autoimmune systemic disease that can affect any body system. Involvement of the musculoskeletal system, with arthral-gias and arthritis (synovitis), is a common presenting feature of SLE. Joint swelling, tenderness, and pain on movement are also common. Frequently, these are accompanied by morning stiffness.

Several different types of skin manifestations may occur in pa-tients with SLE, including subacute cutaneous lupus erythe-matosus, which involves papulosquamous or annular polycyclic lesions, and discoid lupus erythematosus, which is a chronic rash that has erythematous papules or plaques and scaling and can cause scarring and pigmentation changes. The most familiar skin manifestation (but occurring in fewer than half of patients with SLE) is an acute cutaneous lesion consisting of a butterfly-shaped rash across the bridge of the nose and cheeks (Fig. 54-5). In some cases of discoid lupus erythematosus, only skin involvement may occur. In some SLE patients, the initial skin involvement may be the precursor to more systemic involvement. 

The lesions often worsen during exacerbations (flares) of the systemic disease and possibly are provoked by sunlight or artificial ultraviolet light. Oral ulcers, which may accompany skin lesions, may involve the buccal mucosa or the hard palate. The ulcers occur in crops and are often associated with exacerbations.

Pericarditis is the most common cardiac manifestation. Women who have SLE are also at risk for early atherosclerosis.

Serum creatinine levels and urinalysis are used in screening for renal involvement. Early detection allows for prompt treatment so that renal damage can be prevented. Renal involvement may lead to hypertension, which also requires careful monitoring and management.

Central nervous system involvement is widespread, encom-passing the entire range of neurologic disease. The varied and fre-quent neuropsychiatric presentations of SLE are now widely recognized. These are generally demonstrated by subtle changes in behavior patterns or cognitive ability. Depression and psychosis are common (Klippel, 2001; Petri, 2000; Ruddy et al., 2001).

Assessment and Diagnostic Findings

Diagnosis of SLE is based on a complete history, physical exam-ination, and blood tests. In addition to the general assessment performed for any patient with a rheumatic disease, assessment for known or suspected SLE has special features. The skin is inspected for erythematous rashes. Cutaneous erythematous plaques with an adherent scale may be observed on the scalp, face, or neck. Areas of hyperpigmentation or depigmentation may be noted, depending on the phase and type of the disease. The pa-tient should be questioned about skin changes (because these may be transitory) and specifically about sensitivity to sunlight or arti-ficial ultraviolet light. The scalp should be inspected for alopecia and the mouth and throat for ulcerations reflecting gastrointesti-nal involvement.

Cardiovascular assessment includes auscultation for peri-cardial friction rub, possibly associated with myocarditis and accompanying pleural effusions. The pleural effusions and infil-trations, which reflect respiratory insufficiency, are demonstrated by abnormal lung sounds. Papular, erythematous, and purpuric lesions developing on the fingertips, elbows, toes, and extensor surfaces of the forearms or lateral sides of the hand that may be-come necrotic suggest vascular involvement.

Joint swelling, tenderness, warmth, pain on movement, stiff-ness, and edema may be detected on physical examination. The joint involvement is often symmetric and similar to that found in RA.

Typically, assessment reveals classic symptoms, including fever, fatigue, and weight loss and possibly arthritis, pleurisy, and peri-carditis. Interactions with the patient and family may provide further evidence of systemic involvement. The neurologic as-sessment is directed at identifying and describing any central nervous system changes. The patient and family members are asked about any behavioral changes, including manifestations of neuroses or psychosis. Signs of depression are noted, as are reports of seizures, chorea, or other central nervous system manifestations.

No single laboratory test confirms SLE; rather, blood testing reveals moderate to severe anemia, thrombocytopenia, leukocy-tosis, or leukopenia and positive antinuclear antibodies. Other diagnostic immunologic tests support but do not confirm the diagnosis. Hematuria may be found on urinalysis.

Medical Management

Treatment of SLE includes management of acute and chronic disease. Although SLE can be life-threatening, advances in its treatment have led to improved survival and reduced morbidity. Acute disease requires interventions directed at controlling in-creased disease activity or exacerbations that may involve any organ system. Disease activity is a composite of clinical and lab-oratory features that reflect active inflammation secondary to SLE. Management of the more chronic condition involves peri-odic monitoring and recognition of meaningful clinical changes requiring adjustments in therapy (Ruddy et al., 2001).

The goals of treatment include preventing progressive loss of organ function, reducing the likelihood of acute disease, mini-mizing disease-related disabilities, and preventing complications from therapy. Management of SLE involves regular monitoring to assess disease activity and therapeutic effectiveness.

PHARMACOLOGIC THERAPY

Medication therapy for SLE is based on the concept that local tis-sue inflammation is mediated by exaggerated or heightened im-mune responses, which can vary widely in intensity and require different therapies at different times. The NSAIDs used for minor clinical manifestations are often used along with cortico-steroids in an effort to minimize corticosteroid requirements.

Corticosteroids are the single most important medication available for treatment. They are used topically for cutaneous manifestations, in low oral doses for minor disease activity, and in high doses for major disease activity. Intravenous administra-tion of corticosteroids is an alternative to traditional high-dose oral use. Antimalarial medications are effective for managing cu-taneous, musculoskeletal, and mild systemic features of SLE. Immunosuppressive agents (alkylating agents and purine analogs) are used because of their effect on immune function. These med-ications are generally reserved for patients who have serious forms of SLE and who have not responded to conservative therapies (Kimberly, 2001; National Institutes of Health, 1998; Ruddy et al., 2001).

Nursing Management

The nursing care of the patient with SLE is based on the basic plan presented earlier. The most common prob-lems include fatigue, impaired skin integrity, body image distur-bance, and lack of knowledge for self-management decisions. The disease or its treatment may produce dramatic changes in ap-pearance and considerable distress for the patient. The changes and the unpredictable course of SLE necessitate expert assessment skills and nursing care and sensitivity to the psychological re-actions of the patient. Patients may benefit from participation in support groups by receiving disease information, daily manage-ment tips, and social support. Because sun and ultraviolet light exposure can increase disease activity or cause an exacerbation, patients should be taught to avoid exposure or to protect them-selves with sunscreen and clothing.

Because of the increased risk for involvement of multiple organ systems, patients should understand the need for routine periodic screenings as well as health promotion activities. A di-etary consultation may be indicated to ensure that the patient is knowledgeable about dietary recommendations, given the in-creased risk for cardiovascular disease, including hypertension and atherosclerosis. The nurse instructs the patient about the im-portance of continuing prescribed medications and addresses the changes and side effects that are likely with their use. The patient is reminded of the importance of monitoring because of the in-creased risk for systemic involvement, including renal and cardio-vascular effects.