Document Detail

Heart involvement and systemic sclerosis.
MedLine Citation:
PMID:  16218471     Owner:  NLM     Status:  MEDLINE    
Scleroderma heart involvement (SHI) is often manifest, and virtually always present when accurately searched and holds a significant prognostic value. Myocardial involvement by patchy fibrosis (secondary to both repeated ischaemia and immunoinflammatory damage) leads to ventricular diastolic dysfunction, whereas right ventricle overload and failure may complicate pulmonary hypertension. Left ventricular systolic dysfunction is present in a minority of patients, namely those presenting atherosclerotic coronary artery disease and/or arterial hypertension, sometimes triggered by sclerodermic renal involvement. Dysrhythmias and conduction disturbances are considered an hallmark of SHI, facilitated by autonomic dysfunction. SHI is frequently linked to parenchimal and/or vascular lung disease; they determine symptom occurrence, particularly dyspnoea, fatigue, palpitations and chest pain when pericardium is affected. Accurate cardiologic baseline screening and subsequent follow-up are mandatory in all patients, initially consisting in some noninvasive diagnostic procedures: visit, electrocardiogram (EKG), chest X-ray, Doppler-echocardiography. When needed, these examinations should be integrated by EKG Holter-monitoring, cardiopulmonary stress tests, cardiac magnetic resonance imaging, nuclear studies of myocardial function and perfusion, cardiac catheterization to better estimate pulmonary hypertension, and cardiac natriuretic hormone evaluation. Several vasodilator approaches (prostacycline or NO/endothelin) may counteract the microvascular dysfunction at peripheral and cardiopulmonary level, and fight the sequelae of pulmonary hypertension.
C Ferri; D Giuggioli; M Sebastiani; M Colaci; M Emdin
Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Lupus     Volume:  14     ISSN:  0961-2033     ISO Abbreviation:  Lupus     Publication Date:  2005  
Date Detail:
Created Date:  2005-10-12     Completed Date:  2005-12-21     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9204265     Medline TA:  Lupus     Country:  England    
Other Details:
Languages:  eng     Pagination:  702-7     Citation Subset:  IM    
Rheumatology Unit, University of Modena & Reggio Emilia, Policlinico di Modena, Modena, Italy.
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MeSH Terms
Heart / physiopathology*
Lung / pathology,  physiopathology
Myocardium / pathology*
Scleroderma, Systemic / diagnosis,  pathology*,  physiopathology*,  therapy

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