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In This Article

Abstract and Case Study

Introduction

Pathogenesis

Epidemiology

Classification

Clinical Manifestations

Overview of Treatment

Indications for Rapid Referral and Treatment

Patient Education and Support

Symptomatic Management

Summary

Patient and Clinician Resources

Figures

References

From Topics in Advanced Practice Nursing eJournal

Scleroderma: A Fascinating, Troubling Disease

Posted 05/03/2004

Elaine A. Furst, RN, MA

Abstract and Case Study

AbstractScleroderma is classified as a rare, autoimmune, collagen-vascular

disease of unknown etiology that can be difficult to diagnose and treat. Its

onset can be insidious or rapid; the symptoms range from challenging and painful

to life threatening. Individuals may experience a range of symptoms different

from the classic description of the disease, resulting in confused health

professionals and frightened patients. The toll on lives, careers, and disrupted

family and social roles can be overwhelming as well as destructive both to the

patient and to loved ones. Because it is rare (the incidence rate for the

systemic form is estimated to be 19 new cases per year per 1 million adults),

many

nurses and physicians have only read about scleroderma.[1] This article seeks

to familiarize advanced practice nurses (APNs) and other clinicians with the

major manifestations of scleroderma and to provide the information necessary

for its recognition and symptom management. A list of resources is provided to

assist with care planning.

Case Study: Sara Has Unusual Symptoms but No Clear DiagnosisSara is a

40-year-old woman who is ordinarily very active and busy with her family and her

community, but her busy life is at a standstill. She has severe fatigue,

arthralgias, and tight, itchy skin on her face, arms, hands, and trunk. She

spends most

of her days sitting up in the lounger in the living room because she is both

fatigued and has constant heartburn. She has had frequent dental problems

because of excessive dryness of her mouth. She describes a dwindling appetite

and

thus has had very poor nutritional intake for months.

Sara is always cold, and her fingertips and nails are often numb, even in the

summertime. There are 2 small, painful, digital ulcers on the knuckles of her

right hand and 1 under the thumbnail on her left. Her fingers exhibit the

beginnings of sclerodactyly, making it difficult for her to perform daily

activities and household duties.

Her internist has prescribed an antihistamine for the itching and a protein

pump inhibitor for dyspepsia and told her to rest and " reduce the stress in

your life. " She has been to see quite a few specialists about her complaints,

including a psychologist, and none of them were definitive about the diagnosis.

Sara is finally referred to a rheumatologist at a nearby university medical

department who diagnoses systemic sclerosis. She and her husband are

frightened, especially when she is prescribed methotrexate, which they know is a

drug

used in cancer. She types scleroderma into her computer search engine and

discovers advertisements for herbal remedies for scleroderma, the site for the

Scleroderma Foundation, and articles about scleroderma in women's magazines.

This

only adds to her confusion and worry.

Section 1 of 12

Next Page: Introduction

Elaine A. Furst, RN, MA, Clinical Educator, Cedars-Sinai Hospital, Los

Angeles, California; Chair of Board of Directors, Scleroderma Foundation

Disclosure: Elaine A. Furst, RN, MA, has no significant financial interests

or relationships to disclose.

Topics in Advanced Practice Nursing eJournal 4(2), 2004. © 2004 Medscape