Treatment of complications associated with systemic sclerosis

Am J Health Syst Pharm. 2008 Feb 15;65(4):315-21. doi: 10.2146/ajhp070024.


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.

Publication types

  • Review

MeSH terms

  • Anti-Inflammatory Agents, Non-Steroidal / therapeutic use
  • Antihypertensive Agents / therapeutic use
  • Calcium Channel Blockers / therapeutic use
  • Epoprostenol / analogs & derivatives
  • Epoprostenol / therapeutic use
  • Female
  • Gastrointestinal Diseases / drug therapy
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Joint Diseases / drug therapy
  • Male
  • Muscle Weakness / drug therapy
  • Phosphodiesterase Inhibitors / therapeutic use
  • Pulmonary Fibrosis / drug therapy
  • Quality of Life
  • Raynaud Disease / drug therapy
  • Scleroderma, Systemic / drug therapy*
  • Scleroderma, Systemic / pathology
  • Sjogren's Syndrome / drug therapy
  • Skin Ulcer / drug therapy


  • Anti-Inflammatory Agents, Non-Steroidal
  • Antihypertensive Agents
  • Calcium Channel Blockers
  • Immunosuppressive Agents
  • Phosphodiesterase Inhibitors
  • Epoprostenol