Scleroderma - Diffuse Connective Tissue Diseases

SCLERODERMA

Scleroderma (“hard skin”) is a relatively rare disease that is poorly understood; the cause is unknown. Its incidence is 18 to 20 per-sons per million per year (Ruddy et al., 2001).

Pathophysiology

Like other diffuse connective tissue diseases, scleroderma (also known as systemic sclerosis) has a variable course with remissions and exacerbations. Its prognosis is not as optimistic as that of SLE. The disease commonly begins with skin involvement. Mononuclear cells cluster on the skin and stimulate lymphokines to stimulate procollagen. Insoluble collagen is formed and accu-mulates excessively in the tissues. Initially, the inflammatory re-sponse causes edema formation, with a resulting taut, smooth, and shiny skin appearance. The skin then undergoes fibrotic changes, leading to loss of elasticity and movement. Eventually, the tissue degenerates and becomes nonfunctional. This chain of events, from inflammation to degeneration, also occurs in blood vessels, major organs, and body systems (Klippel, 2001).

Clinical Manifestations

Scleroderma starts insidiously with Raynaud’s phenomenon and swelling in the hands. The skin and the subcutaneous tissues be-come increasingly hard and rigid and cannot be pinched up from the underlying structures. Wrinkles and lines are obliterated. The skin is dry because sweat secretion over the involved region is sup-pressed. The extremities stiffen and lose mobility. The condition spreads slowly; for years, these changes may remain localized in the hands and the feet. The face appears masklike, immobile, and expressionless, and the mouth becomes rigid.

The changes within the body, although not visible directly, are vastly more important than the visible changes. The left ventricle of the heart is involved, resulting in heart failure. The esophagus hardens, interfering with swallowing. The lungs become scarred, impeding respiration. Digestive disturbances occur because of hardening (sclerosing) of the intestinal mucosa. Progressive renal failure may occur.

The patient may manifest a variety of symptoms referred to as the CREST syndrome. CREST stands for calcinosis (calcium de-posits in the tissues), Raynaud’s phenomenon, esophageal harden-ing and dysfunctioning, sclerodactyly (scleroderma of the digits), and telangiectasis (capillary dilation that forms a vascular lesion).

Assessment and Diagnostic Findings

Assessment focuses on the sclerotic changes in the skin, contrac-tures in the fingers, and color changes or lesions in the fingertips. Assessment of systemic involvement requires a systems review with special attention to gastrointestinal, pulmonary, renal, and cardiac symptoms. Limitations in mobility and self-care activities should be assessed, along with the impact the disease has had (or will have) on body image.

There is no one conclusive test to diagnose scleroderma. A skin biopsy is performed to identify cellular changes specific to sclero-derma. Pulmonary studies show ventilation-perfusion abnormal-ities. Echocardiography identifies pericardial effusion (often present with cardiac involvement). Esophageal studies demonstrate de-creased motility in 75% of patients with scleroderma. Blood tests may detect antinuclear antibodies, indicating a connective tissue disorder and possibly distinguishing the subgroup of scleroderma. A positive antinuclear antibody test result is common in patients with scleroderma.

Medical Management

Treatment of scleroderma depends on the clinical manifestations. All patients require counseling, during which realistic individual goals may be determined. Support measures include strategies to decrease pain and limit disability. A moderate exercise program is encouraged to prevent joint contractures. Patients are advised to avoid extreme temperatures and to use lotion to minimize skin dryness.

PHARMACOLOGIC THERAPY

No medication regimen has proved effective in modifying the dis-ease process in scleroderma, but various medications are used to treat organ system involvement. Calcium channel blockers and other antihypertensive agents may provide improvement in symp-toms of Raynaud’s phenomenon. Anti-inflammatory medications can be used to control arthralgia, stiffness, and general musculo-skeletal discomfort (Klippel, 2001; Ramsey-Goldman, 2001).

Nursing Management

The nursing care of the patient with scleroderma is based on the basic plan of nursing care presented earlier. The most common nursing diagnoses of the patient with scleroderma in-clude impaired skin integrity; self-care deficits; imbalanced nu-trition, less than body requirements; and disturbed body image. The patient with advanced disease may also have problems with impaired gas exchange, decreased cardiac output, impaired swal-lowing, and constipation.

Providing meticulous skin care and preventing the effects of Raynaud’s phenomenon are major nursing challenges. Patient teaching must include the importance of avoiding cold and pro-tecting the fingers with mittens in cold weather and when shop-ping in the frozen-food section of the grocery store. Warm socks and properly fitting shoes are helpful in preventing ulcers. Care-ful, frequent inspection for early ulcers is important. Smoking cessation is critical.