Authors

  1. Shepard Battle, Leslee H. EdD, MSN, RN, CMSRN
  2. Chappel Aiken, Lolita I. EdD, MSN, RN

Article Content

Scleroderma is a chronic connective tissue disorder in which the body produces an excessive amount of collagen. This rare, noncontagious, complex autoimmune rheumatic disease affects women more often than men and typically occurs between the ages of 20 and 50. The American College of Rheumatology estimates that between 75,000 and 100,000 people in the United States have scleroderma.

  
Figure. No caption a... - Click to enlarge in new windowFigure. No caption available.

When we think of scleroderma, our initial association is often skin hardening-the disease's most prevalent characteristic. However, collagen is an important protein for not only the skin's structure and strength, but also healing and repairing bones, cartilage, and other connective tissues, such as internal organs. Because of this widespread systemic potential, scleroderma is also known as systemic sclerosis.

 

Although the exact cause of collagen overproduction is unknown, research shows that there may be certain risk factors, such as genetics and environmental exposures (prolonged exposure to coal).

 

In this article, we give you the knowledge you need to manage the care of patients with scleroderma to promote the best outcomes.

 

Classifications

Scleroderma has two major classifications: localized and systemic.

 

Localized scleroderma typically results in mild symptoms, occurring in a few isolated areas of the skin or muscles. More common in children, it typically doesn't affect internal organs. Localized scleroderma has two forms: morphea and linear scleroderma. Morphea is characterized by waxy patches on the skin that come and go in various shapes and sizes. The patches may disappear as spontaneously as they appear. Linear scleroderma has the striking characteristic of a line or hardened, waxy skin on an extremity or the forehead. The scarlike appearance on the forehead resembles a saber or sword wound, referred to as en coup de sabre.

 

Systemic scleroderma is also known as systemic sclerosis (hardening). This classification of the disease can affect many parts of the body, from the skin to internal organs, and even blood vessels (see Picturing scleroderma). Systemic scleroderma is more common in female patients and can be categorized as limited or diffuse. When systemic scleroderma is limited, it means that the skin hardening isn't widespread. In most cases, limited scleroderma progresses slowly and only affects the fingers, hands, and face. When systemic scleroderma is diffuse, skin thickening occurs more rapidly and affects multiple areas, with a greater chance of internal organ involvement.

 

Symptoms

Although patients may experience symptom variability, CREST is an acronym for the five most predominant symptoms of scleroderma as defined by the Scleroderma Foundation:

 

* calcinosis

 

* Raynaud phenomenon

 

* esophageal dysfunction

 

* sclerodactyly

 

* telangiectasia.

 

 

See CREST symptoms with typical nursing interventions and medical management for a quick reference.

 

Patients may report changes in their fingers and toes when exposed to cold. If so, you should suspect Raynaud phenomenon and proceed to ask follow-up questions. Some patients may develop Raynaud phenomenon before the notable skin changes of scleroderma.

 

Esophageal hardening within the gastric region can cause symptoms ranging from weight loss, bloating, constipation, and diarrhea to malnutrition. These patients are at risk for aspiration due to the hardening of the esophagus and skin tightening around the face that restricts chewing.

  
Figure. Picturing sc... - Click to enlarge in new windowFigure. Picturing scleroderma

With diffuse systemic sclerosis, symptoms will continue to progress, leading to joint and muscle inflammation. The skin will become very dry, with a decreased ability to sweat. Poor circulation will cause hair loss on the extremities. Eventually, internal organs are affected. Lung-related symptoms include shortness of breath and cough. Heart involvement results in weight gain from fluid retention, leg swelling, and palpitations. Kidney involvement may result in scleroderma renal crisis due to a sudden decline in kidney function caused by renal blood vessel restriction.

 

Diagnosis

Scleroderma is a challenge to diagnose because it's similar to other types of autoimmune disorders and the presentation of the disease differs. Ask patients to provide a complete health history. Pay particular attention to the CREST symptoms. Ask patients about any unusual changes in their skin. When completing your health assessment, look for tightly stretched and shiny skin with no pitting edema.

 

Patients experiencing Raynaud phenomenon will report that they have episodes when their fingertips get very cold and turn white then blue from a sporadic loss of blood flow. Patients may report numbness and pain when this occurs; when blood returns, their fingers will turn red.

 

The healthcare provider may order routine blood work, including a complete blood cell count, to help detect complications such as anemia. A chemistry panel helps determine organ involvement, such as creatinine for kidney functioning. Antinuclear antibody testing may be ordered to determine the presence of antibodies. Erythrocyte sedimentation rate may be tested to determine the level of inflammation. A biopsy or small tissue sample may be taken and examined for abnormalities.

  
Table CREST symptoms... - Click to enlarge in new windowTable CREST symptoms with typical nursing interventions and medical management

Systemic scleroderma may affect the heart, lungs, kidneys, or other internal organs. When internal organs are involved, the healthcare provider will order tests dependent on the organ affected and symptoms present.

 

Management

When caring for patients with scleroderma, physiologic symptoms must be managed, along with emotional well-being. These patients are usually overwhelmed because they can experience symptoms that range from mild to severe, with hardening of the skin being the most prevalent. Patients may report feelings of fear, depression, sadness, anger, or denial. These feelings may come and go sporadically, but should be managed carefully.

 

Disease management requires understanding key signs and related symptoms because treatments and nursing interventions are dependent on patient symptoms. Take an interdisciplinary approach when managing patient care. In addition to the primary healthcare provider, other team members may include a nutritionist, occupational therapist, physical therapist, and relevant specialists.

 

Teach your patient about the disease process, with a primary focus on the presenting symptoms. Include education about prescribed medications and the need to reduce the risk of vasoconstriction by avoiding very cold temperatures, caffeinated beverages, chocolate, smoking, and medications known to cause vasoconstriction. Explain that wearing gloves when outside in cooler temperatures or handling cold items helps minimize the symptoms of Raynaud phenomenon.

 

Teach patients with esophageal dysfunction to avoid spicy foods, as well as very hot foods, because these stimulate gastric secretions. In addition, they should avoid large meals; instead encourage them to eat four to six small meals a day and remain upright at least 1 to 2 hours after eating to prevent gastroesophageal reflux.

 

Provide other education that directly relates to affected organs. When the lungs are affected, teach your patient energy conservation techniques for completing activities of daily living. You may need to instruct your patient how to use home oxygen safely. For heart involvement, teach your patient to take daily weights at the same time every day while wearing similar clothing. For kidney involvement, teach your patient how to take his or her BP, including normal readings and when to notify the healthcare provider.

 

Patients diagnosed with scleroderma may experience physical, mental, and functional disabilities. As with any chronic illness, social support is important for adequate well-being. Community-based support organizations, such as the Arthritis Foundation and the Scleroderma Foundation, are helpful for this patient population. Effective coping is a key to good self-care management. Because scleroderma can be disfiguring to the body, interventions that provide for positive body image, such as Internet-based cognitive-behavioral support programs, should be implemented with the aim of decreasing body image distress.

 

A quality future

Scleroderma is a rare disease that disproportionately affects women of childbearing age. Currently, there's no cure for scleroderma, so your role is essential for planning care, including self-management education. Early detection and management, and an interdisciplinary team approach can help patients maintain a good quality of life for years to come.

 

memory jogger

To remember the predominant symptoms of scleroderma, think CREST.

  
Figure. No caption a... - Click to enlarge in new windowFigure. No caption available.

C Calcinosis

 

R Raynaud phenomenon

 

E Esophageal dysfunction

 

S Sclerodactyly

 

T Telangiectasia

 

on the web

American College of Rheumatology:https://www.rheumatology.org

  
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Arthritis Foundation:http://www.arthritis.org

 

International Scleroderma Network:http://www.sclero.org

 

Scleroderma Education Project:http://sclerodermainfo.org

 

Scleroderma Foundation:http://www.scleroderma.org

 

REFERENCES

 

Hinkle JL, Cheever KH. Brunner & Suddarth's Textbook of Medical-Surgical Nursing. 13th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2014.

 

Hornboonherm P, Nanagara R, Kochamat A, Wantha O. Self_care behaviours and trajectory management by people with scleroderma in northeastern Thailand. Int J Nurs Pract. 2017;23:e12523.

 

Kwakkenbos L, Delisle VC, Fox RS, et al Psychosocial aspects of scleroderma. Rheum Dis Clin North Am. 2015;41(3):519-528.

 

Lachner KD. Caring for the patient with limited systemic scleroderma. Orthop Nurs. 2016;35(1):5-10.

 

Merz EL, Malcarne VL, Roesch SC, et al Longitudinal patterns of pain in patients with diffuse and limited systemic sclerosis: integrating medical, psychological, and social characteristics. Qual Life Res. 2107;26(1):85-94.