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Scleroderma Lung
- Source :
- Drugs. 67:985-996
- Publication Year :
- 2007
- Publisher :
- Springer Science and Business Media LLC, 2007.
-
Abstract
- The lungs are frequently involved in systemic sclerosis ('scleroderma'), a rare, disabling disease of unknown origin, characterised by skin thickening and Raynaud's phenomenon. The pathogenesis of scleroderma is complex, but signs and symptoms of excessive fibrosis, vasculopathy and inflammation are almost universally present. Dyspnoea in scleroderma patients can be due to chest wall tightening from skin thickening, pleural disease, cardiac involvement, myositis of intercostal muscles, or so-called scleroderma lung disease. Scleroderma lung disease encompasses vascular (pulmonary artery hypertension) or interstitial lung disease, or both. A comprehensive work-up is required to delineate the underlying cause of dyspnoea in a scleroderma patient, and to establish the contribution of each component to the symptoms. This should include a 6-minute walk test, pulmonary function testing, high-resolution thoracic CT scanning, ECG, echocardiography and, if pulmonary artery hypertension is suspected, right-heart catheterisation; bronchoalveolar lavage is optional. Lung disease in scleroderma contributes significantly to excess morbidity and early mortality, especially when diffusion capacity drops below 40% and/or forced vital capacity below 50%. However, recent clinical studies have unequivocally demonstrated that scleroderma lung disease is amenable to treatment with new vasodilatory drugs that target specific pathways involved in vasoconstriction, or with cyclophosphamide for interstitial lung disease. Uncontrolled studies have suggested that these therapies also have an impact on survival, but controlled studies with a long follow-up are needed to corroborate this point.
- Subjects :
- medicine.medical_specialty
Vital capacity
Pathology
Endothelin A Receptor Antagonists
Hypertension, Pulmonary
Scleroderma
Pulmonary function testing
Pleural disease
Terminology as Topic
medicine.artery
Internal medicine
Prostaglandins, Synthetic
medicine
Humans
Pharmacology (medical)
skin and connective tissue diseases
Cyclophosphamide
Scleroderma, Systemic
Lung
integumentary system
medicine.diagnostic_test
business.industry
Interstitial lung disease
respiratory system
medicine.disease
respiratory tract diseases
medicine.anatomical_structure
Bronchoalveolar lavage
Pulmonary artery
Cardiology
Lung Diseases, Interstitial
business
Immunosuppressive Agents
Subjects
Details
- ISSN :
- 00126667
- Volume :
- 67
- Database :
- OpenAIRE
- Journal :
- Drugs
- Accession number :
- edsair.doi.dedup.....3206e26721232c3f1a8fd86d040b7faa