Document Detail


Treatment of complications associated with systemic sclerosis.
MedLine Citation:
PMID:  18238768     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Stacie C Moore; Evelyn R Hermes Desantis
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  American journal of health-system pharmacy : AJHP : official journal of the American Society of Health-System Pharmacists     Volume:  65     ISSN:  1535-2900     ISO Abbreviation:  Am J Health Syst Pharm     Publication Date:  2008 Feb 
Date Detail:
Created Date:  2008-02-01     Completed Date:  2008-02-21     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9503023     Medline TA:  Am J Health Syst Pharm     Country:  United States    
Other Details:
Languages:  eng     Pagination:  315-21     Citation Subset:  IM    
Affiliation:
Robert Wood Johnson University Hospital (RWJUH), New Brunswick, NJ, USA.
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MeSH Terms
Descriptor/Qualifier:
Anti-Inflammatory Agents, Non-Steroidal / therapeutic use
Antihypertensive Agents / therapeutic use
Calcium Channel Blockers / therapeutic use
Epoprostenol / analogs & derivatives,  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*,  pathology
Sjogren's Syndrome / drug therapy
Skin Ulcer / drug therapy
Chemical
Reg. No./Substance:
0/Anti-Inflammatory Agents, Non-Steroidal; 0/Antihypertensive Agents; 0/Calcium Channel Blockers; 0/Immunosuppressive Agents; 0/Phosphodiesterase Inhibitors; 35121-78-9/Epoprostenol

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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