Scleroderma, a systemic sclerosis that’s also known as progressive systemic sclerosis, is a diffuse connective tissue disease characterized by fibrotic, degenerative, and occasionally inflammatory changes in skin, blood vessels, synovial membranes, skeletal muscles, and internal organs (especially the esophagus, intestinal tract, thyroid, heart, lungs, and kidneys).
This disease occurs in distinctive forms:
❑ CREST syndrome — a benign form characterized by calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia
❑ diffuse systemic sclerosis — characterized by generalized skin thickening and invasion of internal organ systems
❑ localized scleroderma — characterized by patchy skin changes with a droplike appearance known as morphea
❑ linear scleroderma — characterized by a band of thickened skin on the face or extremities that severely damages underlying tissues, causing atrophy and deformity (most common in childhood).
Other forms include chemically induced localized scleroderma, eosinophilia myalgia syndrome (recently associated with ingestion of l-tryptophan), toxic oil syndrome (associated with contaminated oil), and graft-versus-host disease.
The cause of scleroderma is unknown. Known risk factors include exposure to silica dust and polyvinyl chloride.
Scleroderma affects more females than men, especially between ages 30 and 50. Approximately 30% of patients with scleroderma die within 5 years of onset.
Scleroderma typically begins with Raynaud’s phenomenon — blanching, cyanosis, and erythema of the fingers and toes in response to stress or exposure to cold. Progressive phalangeal resorption may shorten the fingers.
Compromised circulation, which results from abnormal thickening of the arterial intima, may cause slowly healing ulcerations on the tips of the fingers or toes that may lead to gangrene. Raynaud’s phenomenon may precede scleroderma by months or years.
Later symptoms include pain, stiffness, and finger and joint swelling. Skin thickening produces taut, shiny skin over the entire hand and forearm. Facial skin also becomes tight and inelastic, causing a masklike appearance and “pinching” of the mouth. As tightening progresses, contractures may develop.
GI dysfunction causes frequent reflux, heartburn, dysphagia, and bloating after meals. These symptoms may cause the patient to decrease food intake and lose weight. Other GI effects include abdominal distention, diarrhea, constipation, and malodorous floating stools.
In advanced disease, cardiac and pulmonary fibrosis produce arrhythmias and dyspnea. Renal involvement is usually accompanied by malignant hypertension, the main cause of death.
Typical cutaneous changes provide the first clue to diagnosis. Results of diagnostic tests include:
❑ blood studies — slightly elevated erythrocyte sedimentation rate, positive rheumatoid factor in 25% to 35% of patients, and positive antinuclear antibody test
❑ chest X-rays — bilateral basilar pulmonary fibrosis
❑ electrocardiogram — possible nonspecific abnormalities related to myocardial fibrosis
❑ GI X-rays — distal esophageal hypomotility and stricture, duodenal loop dilation, small-bowel malabsorption pattern, and large diverticula
❑ hand X-rays — terminal phalangeal tuft resorption, subcutaneous calcification, and joint space narrowing and erosion
❑ pulmonary function studies — decreased diffusion and vital capacity and restrictive lung disease
❑ skin biopsy — may show changes consistent with the disease’s progress, such as marked thickening of the dermis and occlusive vessel changes
❑ urinalysis — proteinuria, microscopic hematuria, and casts (with renal involvement).
Currently, no cure exists for scleroderma. Treatment aims to preserve normal body functions and minimize complications. Use of an immunosuppressant such as chlorambucil is a common palliative measure. Corticosteroids and colchicine seem to stabilize symptoms; d-penicillamine may be helpful. Blood platelet levels need to be monitored throughout drug therapy.
Other treatments vary according to symptoms:
❑ chronic digital ulcerations — a digital plaster cast to immobilize the area, minimize trauma, and maintain cleanliness; possibly surgical debridement
❑ esophagitis with stricture — antacids, cimetidine, periodic esophageal dilation, and a soft, bland diet
❑ hand debilitation — physical therapy to maintain function and promote muscle strength, heat therapy to relieve joint stiffness, and patient teaching to make performance of daily activities easier
❑ Raynaud’s phenomenon — various vasodilators and antihypertensive agents (such as methyldopa or calcium channel blockers), intermittent cervical sympathetic blockade or, rarely, thoracic sympathectomy
❑ scleroderma kidney (with malignant hypertension and impending renal failure) — dialysis, antihypertensives, and calcium channel blockers
❑ small-bowel involvement (diarrhea, pain, malabsorption, and weight loss) — broad-spectrum antibiotics, such as erythromycin or tetracycline, to counteract bacterial overgrowth in the duodenum and jejunum related to hypomotility.
❑ Assess the patient’s motion restrictions, pain, vital signs, intake and output, respiratory function, and daily weight.
❑ Because of compromised circulation, warn against finger-stick blood tests.
❑ Remember that air conditioning may aggravate Raynaud’s phenomenon.
❑ Help the patient and his family adjust to the patient’s new body image and to the limitations and dependence that these changes cause.
❑ Teach the patient to avoid fatigue by pacing activities and organizing schedules to include necessary rest.
❑ Stress to the patient and his family the need to accept the fact that this condition is incurable. Encourage them to express their feelings and help them cope with their fears and frustrations by offering information about the disease, its treatment, and relevant diagnostic tests.
❑ Whenever possible, let the patient participate in treatment by measuring his own intake and output, planning his own diet, assisting in dialysis, giving himself heat therapy, and doing prescribed exercises.
❑ Direct the patient to seek out support groups, which can be found in every state. Instruct the patient to call 1-800-722-HOPE or go to www.scleroderma.org (if possible) to determine the closest location.
Review other book chapters online related to Scleroderma:
Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: Professional Guide to Diseases (Eighth Edition) Authors: Springhouse Publisher: Lippincott Williams & Wilkins Copyright: 2005 ISBN: 1-58255-370-X 
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