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Review
. 2014 Aug;66(8):1967-78.
doi: 10.1002/art.38702.

Review: interstitial lung disease associated with systemic sclerosis and idiopathic pulmonary fibrosis: how similar and distinct?

Erica L Herzog  1 , Aditi Mathur, Andrew M Tager, Carol Feghali-Bostwick, Frank Schneider, John Varga
Affiliations
Review

Review: interstitial lung disease associated with systemic sclerosis and idiopathic pulmonary fibrosis: how similar and distinct?

Erica L Herzog et al. Arthritis Rheumatol. 2014 Aug.
No abstract available

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Figures

Figure 1
Figure 1
High-resolution computed tomography of systemic sclerosis (SSc)–associated interstitial lung disease (ILD) and idiopathic pulmonary fibrosis (IPF). A, IPF. Prone axial image shows subpleural cysts/honeycombing, architectural distortion, and reticular interstitial markings in a basilar distribution with absence of ground-glass opacities and nodules. B, Nonspecific interstitial pneumonia in SSc-associated ILD. Note increased reticular markings, traction bronchiectasis, and ground-glass opacities. C, Cellular nonspecific interstitial pneumonia in SSc-associated ILD. Note peripheral ground-glass opacities with areas of subpleural sparing. Arrow indicates dilated esophagus.
Figure 2
Figure 2
Systemic sclerosis (SSc)–associated interstitial lung disease (ILD). A, Nonspecific interstitial pneumonia. Note diffuse alveolar septal thickening throughout the lobule with lack of peripheral accentuation in the area of an interlobular septum on the left. B, Usual interstitial pneumonia. Note peripheral involvement of a pulmonary lobule sparing the centrilobular area containing the bronchovascular bundle. Arrows indicate fibroblastic foci. C, Pulmonary arterial hypertension. Note hypertensive arterial changes with prominent intimal fibrosis. Arrow indicates separation of the media and intima by the internal elastic lamina. D, Pleural fibrosis. Its presence supports the diagnosis of SSc-associated ILD in the appropriate clinical setting. Hematoxylin and eosin stained in A, B, and D; Verhoeff–van Gieson stained in C. Original magnification × 40 in A and B; × 200 in C; × 100 in D.
Figure 3
Figure 3
Schematic representation of key pathways implicated in systemic sclerosis (SSc)–associated interstitial lung disease (ILD) and idiopathic pulmonary fibrosis (IPF). Recurrent epithelial and/or endothelial injury promotes recruitment of macrophages and lymphocytes with resulting production of profibrotic mediators, including transforming growth factor β1 (TGFβ1), connective tissue growth factor (CTGF), and platelet-derived growth factor (PDGF). Together, this enhances fibroblast activation, proliferation, survival, and differentiation to a contractile myofibroblast phenotype with resulting overproduction and accumulation of extracellular matrix (ECM).
See this image and copyright information in PMC

References

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