Document Detail


Clinical approach to scleroderma.
MedLine Citation:
PMID:  9759679     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Systemic sclerosis (SSc) is a heterogenous disease with a morbidity and mortality that varies widely. Nonetheless, the future clinical course of an individual patient can be estimated based on the severity of skin and internal organ involvement within the first several years of the disease. Patients with limited cutaneous SSc (ISSc) have skin thickening below the elbows or knees and may have face and neck involvement. Patients with this subtype of SSc have Raynaud's phenomenon, digital ulcers, and esophageal dysfunction. Significant morbidity and mortality arises in those patients with ISSc who develop interstitial lung disease or pulmonary artery hypertension. Patients with diffuse cutaneous SSc (dSSc) have skin thickening above the elbows and knees or on the trunk. These patients have a more abrupt onset of disease, often with constitutional symptoms and arthalgias. Severe heart, lung, gut, and renal involvement, if it occurs, tends to develop within the first 5 years of disease, especially within the first several years. Patients with significant internal organ involvement have a poorer prognosis than patients who do not. The goals of the initial history and physical and laboratory examinations are to classify the type of scleroderma as ISSc or dSSc, to estimate disease duration, and to define the extent and severity of organ involvement. Treatment of SSc is organ based. Treatment may reduce morbidity associated with Raynaud's phenomenon, digital ulcers, esophageal dysmotility, esophageal reflux, gut dysmotility, arthralgias, myositis, and pulmonary artery hypertension. Therapy may stabilize lung function in patients with interstitial lung disease with alveolitis and stabilize renal function in patients with renal crisis. The overall prognosis for patients with SSc appears to be improving. Patients with early dSSc should be considered for enrollment onto protocol testing of potential disease-modifying therapies.
Authors:
B White
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Publication Detail:
Type:  Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Seminars in cutaneous medicine and surgery     Volume:  17     ISSN:  1085-5629     ISO Abbreviation:  Semin Cutan Med Surg     Publication Date:  1998 Sep 
Date Detail:
Created Date:  1998-12-08     Completed Date:  1998-12-08     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  9617260     Medline TA:  Semin Cutan Med Surg     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  213-8     Citation Subset:  IM    
Affiliation:
Department of Medicine, University of Maryland and the Veterans Affairs Maryland Health Care System, Baltimore, USA.
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MeSH Terms
Descriptor/Qualifier:
Arthralgia / physiopathology
Esophageal Diseases / physiopathology
Female
Gastrointestinal Diseases / physiopathology
Hand Dermatoses / physiopathology
Heart Diseases / physiopathology
Humans
Hypertension, Pulmonary / physiopathology
Kidney Diseases / physiopathology
Lung Diseases, Interstitial / physiopathology
Patient Education as Topic
Prognosis
Raynaud Disease / physiopathology
Scleroderma, Systemic / classification,  diagnosis,  physiopathology*,  therapy
Skin / physiopathology
Skin Ulcer / physiopathology
Survival Rate
Time Factors
Grant Support
ID/Acronym/Agency:
1R01HL54163/HL/NHLBI NIH HHS

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


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