Scleroderma

This multisystem disease can cause fatigue, disfigurement and depression.

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Learning Scope #308
1.5 contact hours
Expires April 6, 2014

The goal of this continuing education offering is to educate nurses about the principal manifestations of systemic sclerosis. After reading this article, you should be able to: 

1. Define and discuss scleroderma in terms of its diagnosis, treatment, acuity and progression.
2. Discuss the pathogenesis of scleroderma.
3. Plan the nursing care for a patient with scleroderma according to the patient's symptoms.

You can earn 1.5 contact hours of continuing education credit in three ways: 1) Grade and certificate are available immediately after taking the online test. 2) Send the answer sheet (or a photocopy) to ADVANCE for Nurses, Learning Scope, 2900 Horizon Dr., King of Prussia, PA 19406. 3) Fax the answer sheet to 610-278-1426. If faxing or mailing, allow 30 days to receive certificate or notice of failure. A certificate of credit will be awarded to participants who achieve a passing grade of 70 percent or better.

Merion Matters is an approved provider of continuing nursing education by the Pennsylvania State Nurses Association (No. 008-0-07), an accredited approver by the American Nurses Credentialing Center's Commission on Accreditation. 

Merion Matters is also approved as a provider by the California Board of Registered Nursing (No. 13230) and by the Florida Board of Nursing (No. 3298).

The author has completed a disclosure form and reports no relationships relevant to the content of this article.

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There are an estimated 300,000 people with various forms of scleroderma in the U.S., 75 percent of whom are females and in the 40-55 age range. Scleroderma is rare in children (less than 1 percent of cases) and usually manifests itself as morphea. People with diffuse forms of scleroderma can have moderate-to-severe symptoms that affect every aspect of their lives.

Scleroderma is a multisystem disease of unknown etiology that can affect the GI tract, muscles, joints, skin and underlying structures and tissues, kidneys, lungs, nerves and heart. Fatigue, disfigurement and depression are common along with psychosocial consequences such as loss of job, dysfunctional coping, financial difficulties and fear of death and disfigurement.

This article will familiarize you with the principal manifestations of systemic sclerosis so you will know when and where to refer these complicated patients and care for them when in your clinic or hospital.

Case Study

Sara is a 40-year-old woman whose busy life is at a standstill. She has severe fatigue 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 to help with the fatigue and gastroesophageal reflux disease (GERD). She describes a dwindling appetite with worsening of her nutritional status.

Sara is always cold and her fingertips and nails often are severely hyperemic. There are two small, painful digital ulcers on her right hand. Her fingers are beginning to curl, making it difficult for her to perform her ADLs and household duties.

She has seen quite a few specialists about her complaints, including a psychologist, and none of them could be definitive about the diagnosis. (Patients often report a period of 1-3 years from first signs and symptoms to actual diagnosis.)

Sara finally is 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 to treat cancer. She types scleroderma into her computer search engine and discovers ads for herbal remedies, the Scleroderma Foundation site and scary articles in various magazines.

Pathogenesis

Scleroderma is an autoimmune connective tissue disease whose hallmark is excessive collagen deposited around capillaries and in affected tissues, such as the skin, lungs, kidney and esophagus. The etiology is unknown, but its pathogenesis involves an activated immune system, abnormal vascular endothelium and an exaggerated production of fibroblasts that results in abnormal collagen buildup.

Collagen is the major component of connective tissue found in skin, tendons, joints, ligaments and around major organs. Collagen is the basic substance that forms scar tissue, an indispensable part of the repair process of the body. However, an overproduction of collagen interrupts vital systems by replacing functioning cells with "scars."

When this process goes on in the lungs, for example, it results in pulmonary fibrosis, leading to the disruption of the oxygen/carbon dioxide exchange cycle with subsequent changes in pulmonary function. It also can affect the pulmonary vasculature, resulting in pulmonary hypertension -- or it can affect both components of the lung more or less simultaneously, the alveoli (fibrosis) and the vessels (pulmonary hypertension).1

There is an interrelationship among immune, vascular, endothelial, fibroblast and genetic processes that results in the manifestations of scleroderma.2 If there is a genetic background and an external stimulus, the immune system is activated, resulting in vascular injury, fibroblast proliferation and collagen deposition in skin and, often, internal organs.3

Examples of external stimuli include cold (in the case of Raynaud's phenomenon), toxic substances such as organic solvents and silica dust, and various kinds of organ transplantation. Although these have been implicated in cases of scleroderma and scleroderma-like disease, none has been shown to be the singular cause of the disease.

More than 90 percent of people with scleroderma have Raynaud's phenomenon. The vasospasm manifested in Raynaud's can stimulate the immune system, resulting in a mini-feedback loop in which immune activation results in vascular damage (or vice versa).1 The vasospasm observed in the fingers of people with Raynaud's also has been found in the lungs and kidneys of scleroderma patients by radiographic examination.1

There is generally no specific genetic predisposition to scleroderma, although there is an human leukocyte antigen-associated increase in prevalence.4 The presence of other autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosis and Graves' disease in relatives of the scleroderma patient has been noted.1,5

Epidemiology

A disease variable in presentation and unusual in occurrence like scleroderma makes epidemiological surveys complex and difficult. As more research is being done on more patients, more reliable data has emerged, including the following:

• Women outnumber men by approximately 5 to 1, (range 3:1 to 8:1).1,6

• Mean age range is 40-50.1

• With treatment, the 5-year survival rate is over 80 percent, although morbidity is considerable. In patients with the most severe symptoms, 10-15 percent, the 5-year mortality is 50 percent.

• The most recent study reveals that the overall prevalence in adults is 240/million or about 300,000.6

• Blacks have more severe disease more frequently than whites, Asians and Hispanics.5

• Twin studies have indicated that the likelihood of twins having scleroderma is 6 percent. This is higher than the general population but not high enough (100 percent) to indicate a pure genetic etiology.5

Classification

The word scleroderma means, literally, "hard skin." French physicians named it in the late 1700s. There are two main forms and a third group of scleroderma-like disorders that share some of the symptoms of the other two.5

Localized scleroderma (most often found in children):

• Morphea (patches of thickened skin)

• Linear (thickened skin tethered to underlying muscle and bone)

• Scleroderma en coup de sabre ("cut of the saber," linear scleroderma of the head and face only).

Systemic scleroderma:

• Limited scleroderma (was also called CREST, an acronym for Calcinosis, Raynaud's, Esophageal dysfunction, Sclerodactyly and Telangiectasias; this acronym should be discarded as inaccurate)

• Diffuse scleroderma (extensive skin thickening proximal to the elbows and knees along with internal organ involvement)

• Scleroderma sine sclerosis (Raynaud's phenomenon and internal involvement without skin thickening). 

Clinical Manifestations

Many patients go from physician to physician looking for a definitive diagnosis because it is difficult to diagnose early. Following is a description of manifestations of systemic scleroderma (SSc) by body system in rough order of frequency.

Skin: Non-edematous, thickening and tightening of the skin over the fingers can progress through the body over weeks to months. The skin is shiny, swollen, sometimes severely itchy and dysesthetic to the touch. This is usually followed by a tight hidebound phase in which the skin is hard and may even feel like plastic. Later, the skin becomes atrophic and feels less tight, although it is still bound down to the subcutaneous tissue. Not all patients have all phases or progress through all stages.1,5

Vascular: Raynaud's phenomenon of the hand(s), earlobes, nose and/or toes occurs early in 95 percent of patients, with coldness, numbness, and cyanotic and hyperemic color changes.5,7

Constitutional symptoms: Severe fatigue, malaise and some weight loss are very common early in the disease. Depression and non-specific anxiety can occur pre- or post-diagnosis.5

GI: Estimates of GI involvement in SSc patients is 75-90 percent of all patients. There may be symptoms of GERD with and progression down the GI tract, with diarrhea and/or constipation and even pseudo-obstruction. Extra-esophageal symptoms can include cough, hoarseness and aspiration. Dry mouth is a frequent symptom. It often results in severe dental problems, xerostomia and oral candidiasis with painful mouth and decreased taste.1

Musculoskeletal: Joint pain, often without heat, swelling or redness, occurs often and may reflect the tightening of the skin over the joints or actual arthritis. These symptoms occur in up to 97 percent of patients and can include tendon friction rubs (TFR). TFRs often indicate active generalized disease.1,5

Pulmonary: Alveolitis (in 25-90 percent of patients) leading to pulmonary fibrosis (PF) is a common complication of scleroderma that can lead to restrictive lung disease and death.1 The onset of PF is usually within the first 3 years of disease in most patients.4 The 9-year survival rate of these patients is approximately 30 percent. Symptoms range from cough and shortness of breath (SOB) on exertion to SOB at rest with need for continuous oxygen. Baseline pulmonary function tests and echocardiograms should be done on the first workup and then yearly.5

In pulmonary arterial hypertension (PAH), pulmonary arteries that carry blood from the heart to the lungs constrict abnormally, forcing the heart to work faster and causing blood pressure within the lungs to rise to abnormally high levels, putting unusual strain on the heart. Symptoms include SOB, ankle swelling, chest pain and cyanosis of the lips. Patients should be tested with an ECG, electrocardiogram, right heart catheterization and a 6-minute walk test. Scleroderma patients can have both PAH and PF.

Cardiac: Cardiac disease can be manifested as myocardial or pericardial disease, conduction system disease or arrhythmias. Dyspnea on exertion and palpitations are among the most common symptoms.1

Renal: Until the advent of the angiotensin-converting enzyme (ACE) inhibitors, scleroderma renal crisis (SRC) was the most severe complication of scleroderma and the most frequent cause of death. SRC is defined as a new onset of extremely high arterial blood pressure and "rapidly progressive oliguric renal failure during the course of SSc."1 It tends to occur early and in patients with diffuse disease. It can be characterized by onset of seizures, severe headache, blurred vision or a blood pressure greater than 150/90.

Neurological: Obvious neurological problems are rare in SSc. Entrapment neuropathies, (e.g., carpal tunnel syndrome) are most common, although they occur in a small percentage of patients and are usually seen early in the disease. Occasionally, Bell's palsy or trigeminal neuralgia ("tic douloureux") are seen.1,5

Serological changes: The following tests are useful in describing the prognosis of the two main forms of SSc, diffuse and localized, based on their cutaneous and/or internal involvement:

• Serum anti-topoisomerase, also known as SCL-70 (positive in 30 percent of diffuse SSc patients), is specific to SSc.1

• Anticentromere antibody, also known as ACA (positive in 30 percent of SSc patients), is present in patients with localized SSc; up to 30 percent of this subgroup may have the serious complication of PAH.1

Psychosocial: Patients with scleroderma experience not only pain, disfigurement and fatigue, but also fear and depression. Their family members or significant others also are fearful. The unpredictability of SSc and the lack of control this engenders is one of the most difficult aspects of this disease.1,5

Patients often experience financial troubles because they can't work, especially while they are being diagnosed. This puts added strain on the family and the couple, and can lead to familial dysfunction.1Studies of other chronic diseases, such as rheumatoid arthritis, demonstrate interventions aimed at the spouse or significant other benefit the patient as well.

Finally, unlike patients with more well-known diseases, the newly diagnosed SSc patient often has never heard of the disease and feels isolated and alone. She or her friends and family surf the Internet and discover information that can be difficult to evaluate for accuracy and helpfulness. The patient often tries alternative medicine routes that at best don't bring relief and at worst have negative side effects. 

Overview of Treatment

Overall, treatment of the scleroderma patient is focused on the rapid reduction of inflammation, prevention and treatment of fibrosis, and organ-specific therapy.

Immunosuppressive medications such as methotrexate and cyclophosphamide are used in relatively high doses to quickly reduce the inflammatory process in the early stages of diffuse SSc. Despite the usual side effects of these medications early in treatment, the disease can be quieted and patients gain significant relief from debilitating signs and symptoms.

Corticosteroids in doses higher than 10-15 mg per day have been found to be counterproductive in patients with diffuse disease, sometimes precipitating acute SRC. They sometimes are used for joint or tendon symptoms.1

The calcium channel blockers, such as nifedipine, are useful in opening blood vessels in the skin and heart. ACE inhibitors have been used to good effect in the kidney, and bosentan or epoprostenol improve blood flow in the lungs. Antiplatelet therapy (low-dose aspirin) can help prevent clotting, allowing better blood flow through partly occluded vessels.1 These medications are augmented by warm clothing including gloves, even in the hot months, small packets of hand warmers and immediate smoking cessation.

GERD can be treated with protein pump inhibitors, raising the head of the bed with books or bricks and teaching the patient how to take medications to reduce gastric irritation. A small number of patients will need total parenteral nutrition to maintain nutrition. Finally, antibiotics are used to treat the bacterial overgrowth and malabsorption that results from lower bowel gastroparesis.

In general, patients with scleroderma should be treated by rheumatologists as their primary specialist, although other specialists clearly are needed as contributors to overall care. Nurses are important members of the care team. In clinics where large numbers of scleroderma patients are seen, the nurse may be a resource for patient and family education and emotional support.

Indications for Rapid Referral & Treatment

Early diffuse disease with rapidly advancing skin involvement, acute onset high blood pressure, seizures, new proteinuria and an early decrease in pulmonary function are all signals the patient may be in trouble.1,5

These serious symptoms of scleroderma may be seen within the first 3-5 years after diagnosis, and any one of them signals the patient should be seen for rapid workup and treatment. For SRC, hospitalization is the rule.1 Early intervention in SRC in the form of rapid reduction of sudden hypertension is crucial to reduce morbidity and mortality in this potentially catastrophic complication of SSc.

Patient Education & Support

"The role of the nurse, regardless of the setting, is a multifaceted one: caregiver, health educator and referral liaison for both medical and ancillary care, and a critical link between physician, patient and family."1

Nursing care of patients with Raynaud's phenomenon (95 percent of SSc patients) centers around education regarding keeping warm, medications and modifications in care when pain, infection and ulcerations occur. It is important for patients to understand when there is an ischemic episode in the extremities there is sometimes demonstrable ischemia in the lungs, heart and kidneys; therefore, keeping warm, even in the summertime, maintaining their medications and stopping smoking will protect not only the skin but also the internal organs.

The Skin

Since SSc's most prominent sign is hardening of the skin, patients have great difficulties with this area. Not only is it painful, it also is disfiguring and debilitating, causing the typical "mouse face" as well as telangiectasias, clawed fingers, joint pain and itching from the entrapment of superficial pain nerves. Skin breakdown can occur from excessive dryness.

It must be emphasized that only time and immunosuppressives have been found to actually soften the skin, not specific moisturizers or massage, though they can feel good and may be protective.

When digital ulcers occur, wound care specialists can be consulted for treatment options. It is important to protect the hands from harsh chemicals, bumps and abrasions as much as possible.

Telangiectasias, visible as small red or purple spots on the fingers, palms, face and lips can be covered with special non-scented, waterproof cosmetics. 

Musculoskeletal

Joint pain, stiffness and swelling are results of tight skin and inflammation. NSAIDs can be helpful in their management, although the patient must be educated to take these with food and anti-ulcer meds to minimize GI distress.

Loss of hand function is usual and referral to an occupational therapist who can provide hand exercises and splints is useful. Range of motion, paraffin baths, massage and splints occasionally preserve hand function.

Patients benefit from a general exercise regimen as soon as pain and fatigue has been controlled. This may include water aerobics and should be supervised by a physician and physical therapist.

Gastrointestinal

Most patients with SSc have GI involvement of one kind or another.

Upper GI: Preventive dental care using flossing appliances and pediatric toothbrushes is important to maintaining dental health in the face of microstomia and decreased saliva production. Regular dental visits are also important, and dentists with experience in treating scleroderma patients should be sought.

GERD can often be controlled through use of proton pump inhibitors and avoidance of alcohol and greasy, fatty or spicy foods, tobacco and coffee. Elevating the head of the bed is critical, as well as not eating after the evening meal. Given GERD's role in aspiration lung disease, it is important to control this symptom.

SSc can cause abnormally slow movement of food through a narrowed esophagus and thus cause swallowing difficulties. These conditions may require food that is more liquid or softer. Patients should be encouraged to eat slowly and chew thoroughly, and avoid dry foods that may stick in the throat. Remaining upright after meals uses gravity to keep food and gastric acids in the stomach rather than backing up into the esophageal sphincter.

Lower GI: The dysmotility and scarring seen in the upper GI tract also can occur in the lower tract, resulting in constipation and/or diarrhea, pseudoobstruction and bacterial overgrowth. Significant weight loss may occur and total parenteral nutrition through a central venous catheter can be successful. Education regarding the details of using any of the above modalities should be introduced early, given the large percentage of patients experiencing these difficulties.

Pulmonary: Once the particular lung condition has been diagnosed, nurses can help patients understand the medications prescribed, identify the side effects and adverse effects, learn how the medications should be administered, and realize the importance of continuous infusion and subcutaneous or IV medications. The cost of these life-prolonging therapies can be quite high. Nurses can help the patients benefit from social services or patient access programs through pharmaceutical companies.

It is also important, furthermore, to encourage the patient to keep follow-up pulmonary appointments, including echocardiograms, pulmonary function tests and 6-minute walk testing, since changes in these tests may indicate advancing lung disease; quick treatment may forestall further lung degradation.

Renal Involvement

Scleroderma renal crisis is a potentially catastrophic event, and for this reason, patients and families should be carefully taught to take blood pressures at least three times per week and keep a log so as to determine when the blood pressure is going up. The nurse should test the patient carefully for technique and should train the people with whom she lives as well. They should be taught to call the nurse or the physician when the BP is beginning to rise as it is better to have a false negative than to ignore the signs.

Symptoms of SRC indicate an immediate need (hours, not days) for hospitalization and treatment. The family will need reassurance and education about what is happening and what to expect. If dialysis is necessary, the patient and family need help in adapting to this all-consuming treatment. Kidney transplants, if necessary, have been successfully performed on scleroderma patients.

Psychosocial Adjustment

People affected by scleroderma need education about their disease and help in adjusting to the new challenges they will encounter living with their disease. While they're learning and adjusting, they will exhibit signs and symptoms of anxiety and depression at various times during the various diagnostic phases as well as over the months and years of their disease. Strides in new therapies for the symptoms of the disease have been made over the years so that patients can be given encouragement that there is hope and help.

The basis of good psychosocial nursing care is the trusting relationship that the nurse can set up with the patient and family. Coupled with a thorough grounding in assessment and technical nursing skills, nurses can help patients over the obstacles of this serious disease.

Nursing Care Pivotal

Scleroderma is an uncommon, multisystem and often fatal disease that affects an estimated 300,000 people and, indirectly but clearly, their loved ones.

Early diagnosis and treatment for people with scleroderma is essential for good quality of life and a reduction in morbidity and mortality. The nurse is a pivotal member of the team and has a unique opportunity to provide support and education to patients and their significant others.

Elaine A. Furst has conducted workshops for healthcare professionals, patients and families affected by scleroderma, both in the U.S. and Europe, since joining the Scleroderma Foundation in 1994. She currently is volunteer outreach coordinator for the foundation's Southern California chapter.

References
1. Clements, P.J., & Furst, D.E. (Eds.). (2003). Systemic sclerosis (second edition). Baltimore: Williams & Wilkins.
2. Clements, P.J., & Furst, D.E. (Eds.). (1996). Systemic sclerosis (first edition). Baltimore: Williams & Wilkins.
3. Furst, D.E. (2000). Rational therapy in the treatment of systemic sclerosis. Current Opinion in Rheumatology, 12(6), 540-544.
4. Lambert, N.R. (2003). Arthritis Rheumatology 43rd supplement. Program and abstracts of the American College of Rheumatology 67th Annual Scientific Meeting; October 23-28, 2003; Orlando, FL. Abstract No. 857.
5. Mays, M.D. (1999). The scleroderma book: A guide for patients and families. New York: Oxford University Press.
6. Mayes, M.D. (1998). Classification and epidemiology of scleroderma. Seminars in Cutaneous Medicine and Surgery, 17(1), 22-26.
7. Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. (1980). 1980 criteria for the classification of systemic sclerosis. Retrieved May 26, 2009 from: http://www.rheumatology.org/publications/classification/systsclr.asp

Resources
1. American College of Rheumatology. http://www.rheumatology.org/
2. Clements, P.J., & Furst, D.E. (Eds.). (2003). Systemic sclerosis (second edition). Baltimore: Williams & Wilkins.
3. Gottesman, K. (2004). The first year: scleroderma: an essential guide for the newly diagnosed. New York: Marlowe & Company.
4. Mays, M.D. (1999). The scleroderma book: A guide for patients and families. New York: Oxford University Press.
5. National Center for Biotechnology Information. http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001465/
6. National Institutes of Arthritis and Musculoskeletal and Skin Diseases. http://www.niams.nih.gov/
7. The Scleroderma Foundation. http://www.scleroderma.org/






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