Abstract
Systemic sclerosis (SSc; scleroderma) is a poorly understood connective tissue disease that involves the skin, gastrointestinal tract, musculoskeletal system, kidneys, heart, and the lungs (Figures 20.1, 20.2, and 20.3). The earliest inflammatory changes occur in the microcirculation with endothelial cell activation, followed by perivascular infiltration of monocytes and lymphocytes. Subsequently, fibroblasts become activated and deposit increased extracellular matrix in affected tissue systems. There is evidence to support genetic factors in the development of SSc, but few candidate susceptibility or severity genes have yet been found.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
Bibliography
Veeraghavan S, Sharma O. Progressive systemic sclerosis and the lung. Curr Opin Pulm Med 1998;4:305–309.
Schurawitzki H, Stiglbauer R, Graninger W, Herold C, Polzleitner D, Burghuber OC, Tscholakoff D. Interstitial lung disease in progressive systemic sclerosis: High resolution CT versus radiography. Radiology 1990;176:755–759.
Johnson DA, Drane WE, Curran J, Cattau EL Jr, Ciarleglio C, Khan A, Cotelingam J, Benjamin SB. Pulmonary systemic sclerosis: A complication of gastro-esophageal reflux and occult aspiration. Arch Intern Med 1989;149:589–593.
Chang B, Wigley F, White B, Wise R. Scleroderma patients with combined pulmonary hypertension and interstitial lung disease. J Rheumatol 2003;30:2398–2405.
Kim DS, Yoo B, Lee JS, Kim EK, Lim CM, Lee SD, Koh Y, Kim WS, Kim WD, Colby TV, Kitiaichi M. The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Disease 2002;19:121–127.
Ho KT, Reveille JD. The clinical relevance of autoantibodies in scleroderma. Arthritis Res Ther 2003;5:80–93.
Rights and permissions
Copyright information
© 2006 Springer-Verlag London Limited
About this chapter
Cite this chapter
(2006). Systemic Sclerosis and the Lung. In: Clinical Atlas of Interstitial Lung Disease. Springer, London. https://doi.org/10.1007/978-1-84628-326-0_20
Download citation
DOI: https://doi.org/10.1007/978-1-84628-326-0_20
Publisher Name: Springer, London
Print ISBN: 978-1-84628-320-8
Online ISBN: 978-1-84628-326-0
eBook Packages: MedicineMedicine (R0)