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Scleroderma in practice: the role of the nurse

Rachel Vincent
RGN
Scleroderma Specialist Nurse

Helen Wilson
SRN BA(Hons)
Senior Nurse Specialist
Centre for Rheumatology and Connective Tissue Diseases
Royal Free and University College Medical School
London

The word scleroderma comes from two Greek words: "sclero" meaning hard and "derma" meaning skin. Although the term scleroderma is often used as if it were a single disease, it is a generic or umbrella term for a family of diseases (see Table 1). First described in the 18th century, scleroderma occurs when immune dysfunction leads to damage of the small blood vessels and production of excess collagen, causing fibrosis of the skin and its underlying structures, and in the systemic forms affects the internal organs.(1)

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Raynaud's phenomenon
Raynaud's phenomenon is a common, episodic circulatory disorder in which the small blood vessels in the extremities are oversensitive to changes in temperature. It is most commonly found in females and affects between 3-5% of the general population.(2) The hallmark of Raynaud's is biphasic or triphasic colour changes (white and/or blue and red) of the extremities on exposure to the cold, or to sudden but slight temperature changes, or stress. The patient may also complain of pain, numbness or tingling. 
For the vast majority of sufferers, Raynaud's is a benign primary condition. However, patients who have Raynaud's phenomenon secondary to an underlying disease such as scleroderma will often suffer more acute symptoms and in severe cases may develop persistent finger ulcers, infection and ultimately gangrene.
The pathogenesis of Raynaud's phenomenon is as yet uncertain. However, recent advances in research lead Herrick to conclude that the cause is a combination of abnormalities of vascular, intravascular and neural function.(3)
 
Localised scleroderma
In the localised forms of scleroderma, areas of the skin and underlying subcutaneous tissue become fibrotic. There are two types of localised scleroderma:

  • Morphea: hard, oval-shaped plaques on the skin, which in turn can be either localised to one area of the body (commonly the trunk) or generalised. The plaques are usually whitish with a purple ring around them, varying in size. 
  • Linear: sclerotic lesions appearing as linear streaks or bands, usually on the extremities and less commonly on the trunk. 

Localised scleroderma accounts for approximately 10% of new diagnoses of scleroderma and is the form of the disease by which children are almost exclusively affected. Although it is generally considered to be a disease that is confined to the skin and subcutaneous tissue, it can cause serious growth defects if not treated. Zulian et al showed that a quarter of children developed extracutaneous manifestations, including articular, neurologic, vascular, ocular, gastrointestinal, respiratory, cardiac and renal symptoms.(4)
 
Systemic sclerosis
The most common subtype of scleroderma, accounting for approximately 90% of all cases, is the systemic form of the disease, which can be subdivided into diffuse cutaneous systemic sclerosis (dcSSc) and limited cutaneous systemic sclerosis (lcSSc) (see Boxes 1 and 2).

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The first symptom for the majority of patients is Raynaud's phenomenon, but from that point on these two types have different disease progression and organ involvement.

Epidemiology
Scleroderma occurs worldwide but more frequently in the USA than in Europe or the UK.(5) A recent study of the epidemiology of scleroderma in the UK estimated the prevalence at 8.2 per 100,000 and showed that women are five times more likely to be affected than men.(6)
 
Causes
Scleroderma is characterised by extensive fibrosis, vasculopathy and activation of the immune system. However, the pathogenesis behind this process is not yet fully understood. Most researchers agree that it is probable that there are a number of factors involved, and Mayes feels it is likely that there is a strong interplay among genetic factors, hormonal or reproductive-related events, and an external trigger that must interact to result in clinical disease.(5) One study showed that exposure to either cleaning products or solvents emerged as a risk factor.(7)
 
Diagnosis
Scleroderma is often difficult to diagnose, and a definitive diagnosis can sometimes take years, particularly as symptoms vary in prevalence and severity in each individual. Diagnosis of scleroderma is based on clinical history and physical and laboratory findings. An extensive battery of tests is used to determine extent, stage and visceral complications of the disease.

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Treatment
Although there is no cure for scleroderma, there are treatments available that aim to arrest the disease process. Other treatments are based on the individual patient's symptoms. Immunosuppressive drugs are used for patients with diffuse scleroderma to attempt to slow down the disease process. The choice of drug usually depends on the patient's organ involvement. Topical immunosuppression is used in localised scleroderma, although in refractory cases where spontaneous softening does not occur systemic immunosuppression is given. Most immunosuppressive drugs require regular monitoring of kidney and liver function and full blood count.
Continued scientific and clinical research has led to the possibility of substantial improvements in the management of scleroderma. Key areas of advance include the development of clinical and laboratory methods for early detection of complications and the integration of vascular, immunomodulatory and antifibrotic therapies.(8)
Physical and occupational therapies are used to minimise joint disability and functional impairment. These are of particular importance in juvenile localised scleroderma, where this condition may have devastating effects on growth and development such as limb asymmetry, flexion contractures and psychological disability.

Skincare
Skin changes are the most common presenting feature of scleroderma, with the skin becoming tight, shiny and thickened, particularly in the diffuse form of the disease. There are many practical measures that the patient can take themselves, most importantly in the area of skincare. Skin thickening can contribute to loss of movement, making day-to-day functioning difficult. All patients should therefore be encouraged to moisturise their skin several times a day using an oil-based moisturiser and to perform hand and face exercises. Itchy skin (more common in the first few years of active disease) can be relieved by antihistamine creams available over the counter or oral antihistamines.
The nurse should also take the opportunity to reassure the patient that the early aggressive phase of the disease should settle with time and with medical intervention may improve.(9)
Paraffin hand waxing carried out regularly either by physiotherapists, or nurses, or by the patient themselves, can also help to achieve skin softening, to improve circulation and joint stiffness and to help bring to the surface the small calcium deposits that sometimes collect beneath the skin in scleroderma (calcinosis).
Patients with scleroderma can suffer digital ulcers due to small vessel vasculopathy. In this situation patients are advised to use a clean dry dressing only. No evidence can be found that any medicated dressing is helpful in assisting wound healing.

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Role of the nurse in caring for patients
The role of the nurse in scleroderma is not just restricted to the physical care of patients. It is essential to be able to offer a holistic approach to care. Patients with scleroderma may experience a variety of nonspecific symptoms, including fatigue, lack of energy, generalised weakness, weight loss and aching of muscles, joints or bones. Nurses are invaluable in helping patients to develop practical managing strategies for these symptoms.(9)
There is an increasing number of resources available to both patients and healthcare professionals, which include:

  • Medical and nursing textbooks and journals.
  • Information leaflets.
  • Specialist nurses in dedicated scleroderma units.
  • Internet (many sites have an area dedicated to healthcare professionals).
  • Patient support groups.

An awareness of these resources will allow the nurse to empower the scleroderma sufferer to be active in the management of their own disease.(9)

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Prognosis
Life expectancy is not usually affected in localised scleroderma. However, for systemic forms of scleroderma, prognosis varies depending on organ involvement.  Mayes et al found that median survival in systemic sclerosis was 11 years.(10)
Anecdotally some patients have more concern for their loss of functionality and their inability to lead what they consider a normal life than for their shortened life expectancy.

Conclusion
Scleroderma is a rare disease that manifests itself differently in each patient. Although there are now many therapies that can help to halt disease progression or provide symptomatic relief, the nurse plays a vital role in helping patients to adopt a positive attitude and to develop strategies for managing their disease practically.

References

  1. Curzio C. Discussioni anatomicopratiche di un raro, e ftravagante morbo cutaneo in una Donna felicemente curato in quefto grande Ofpedale degl' incurabili. Napoli: Preffo Giovanni di Simone; 1753.
  2. Wigley FM, Flavahan NA. Raynaud's phenomenon. Rheum Dis Clin North Am 1996;22(4):765-81.
  3. Herrick AL. Pathogenesis of Raynaud's phenomenon. Rheumatology 2005;44(5):587-96.
  4. Zulian F, et al. Localised scleroderma in childhood is not just a skin disease. Arthritis Rheum 2005;52(9):2873-81. 
  5. Mayes MD. Scleroderma epidemiology. Rheum Dis Clin North Am 2003;29(2):239-54.
  6. Allcock RJ, et al. A study of the prevalence of systemic sclerosis in northeast England. Rheumatology 2004;43(5):596-602.
  7. Maitre A, et al. Systemic sclerosis and occupational risk factors: role of solvents and cleaning products.J Rheumatol 2004;31(12):2395-401.
  8. Denton CP, Black CM. Targeted therapy comes of age in scleroderma. Trends Immunol 2005;26(11):596-602.
  9. Vincent R, Wilson H. The role of the nurse in scleroderma. The Raynaud's and Scleroderma Association; 2004.
  10. Mayes MD, et al. Prevalence,incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum 2003;48(8):2246-55.
  11. Black CM. Systemic sclerosis. Baltimore: Wilkins and Wilkins; 1996.
  12. Black CM. Scleroderma moves on. Curr Opin Rheumatol 2001;13:493-4.
  13. Bryan C, Knight C, Black CM, Silman AJ. Prediction of five-year survival following presentation with scleroderma. Arthritis Rheum 1999;12:2660-5.
  14. Denton CP, Black CM. Systemic sclerosis: an overview of pathogenesis and treatment. CPD Rheumatol 1999;1(1):33-8.
  15. Denton CP, Black CM. Scleroderma and related disorders: therapeutic aspects. Balliere's Clin Rheumatol 2000;14(1):17-35.
  16. Kallenberg CG. Early detection of connective tissue disease in patients with Raynaud's phenomenon. Rheum Dis Clin North Am 1990;16(1):11-30.
  17. Klippel JH, Dieppe PA. Rheumatology. 2nd ed. Mosby: London; 1998. 
  18. Maddison PJ, Isenberg DA, Woo P, Glass DN. Oxford textbook of rheumatology. 2nd ed. New York: Oxford University Press; 1998. 
  19. Mayes MD. The scleroderma book. New York: Oxford University Press; 1999. 
  20. Sakkas LI. New developments in the pathogenesis of systemic sclerosis. Autoimmunity 2005;38(2):113-6.
  21. Vierra E, Cunningham BB. Morphea and localised scleroderma in children. Semin Cutan Med Surg 1999;18(3):210-25.

Resources
Arthritis Research Campaign
W:www.arc.org.uk

Raynaud's and Scleroderma Association
W:www.raynauds.org.uk

Scleroderma Society
W:www.sclero dermasociety.co.uk

Skin Care Campaign
W:www.skincare campaign.org