Treatment of complications associated with systemic sclerosis

Stacie C. Moore, Evelyn R.Hermes DeSantis

Research output: Contribution to journalReview articlepeer-review

11 Scopus citations

Abstract

Purpose. Current and emerging drug therapy options for patients suffering from the complications of systemic sclerosis are presented. Summary. Systemic sclerosis is a devastating and rare, chronic, autoimmune disease and is characterized by various disease complications due to skin thickening, vascular damage, and inflammation affecting numerous organs. There are two major subtypes of systemic sclerosis: limited cutaneous scleroderma and diffuse cutaneous scleroderma. Patients suffer from Raynaud's phenomenon, skin changes, musculoskeletal changes, gastrointestinal complications, pulmonary complications, scleroderma renal crisis, and dryness of the eyes and mouth. Currently, there is no cure for systemic sclerosis, but research is focusing on decreasing the progression and symptoms of this disease. Raynaud's phenomenon is the temporary vasoconstriction of the small vessels of the fingers, toes, tip of the nose, and earlobes. Skin thickening is the cardinal symptom of systemic sclerosis, with as many as 50% of patients developing digital ulcers. Care of these ulcers is crucial in the prevention of osteomyelitis and other infections. Malabsorption syndrome may also occur in patients, many of whom will eventually require parenteral nutrition to maintain their caloric needs. Pulmonary interstitial fibrosis and pulmonary arterial hypertension are additional serious complications of systemic sclerosis. The use of prostacyclin analogues, phosphodiesterase inhibitors, calcium-channel blockers, cyclophosphamide, bosentan, and other agents has been investigated in patients suffering from the complications of systemic sclerosis. Conclusion. Systemic sclerosis is characterized by various circulatory, dermatological, gastrointestinal, musculoskeletal, pulmonary, and renal complications. Although there is no cure for systemic sclerosis, management of its associated complications can help improve patients'quality of life.

Original languageEnglish (US)
Pages (from-to)315-321
Number of pages7
JournalAmerican Journal of Health-System Pharmacy
Volume65
Issue number4
DOIs
StatePublished - Feb 15 2008

All Science Journal Classification (ASJC) codes

  • Pharmacology
  • Health Policy

Keywords

  • Bosentan
  • Calcium antagonists
  • Cyclophosphamide
  • Hypotensive agents
  • Immunosuppressive agents
  • Nutrition
  • Phosphodiesterase inhibitors
  • Prostacyclin derivatives
  • Quality of life
  • Scleroderma

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