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Review
. 2013 Mar 1;22(127):6-19.
doi: 10.1183/09059180.00005512.

Scleroderma lung disease

Joshua J Solomon  1 , Amy L Olson, Aryeh Fischer, Todd Bull, Kevin K Brown, Ganesh Raghu
Affiliations
Review

Scleroderma lung disease

Joshua J Solomon et al. Eur Respir Rev. .
No abstract available

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Conflict of interest statement

Statement of Interest

A. Fischer is an investigator for Scleroderma Lung Study II, and has received speaker and consultant fees from Actelion and is on medical advisory boards for Actelion. T. Bull has received two investigatorinitiated grants from United Therapeutics and has served as a consultant for Actelion and Lung Rx.

Figures

Figure 1.
Figure 1.
High-resolution computed tomography from a patient with systemic sclerosis showing basilar predominate reticulation and ground-glass opacities with an absence of significant honeycombing in a pattern consistent with nonspecific interstitial pneumonia. The patient also has an air–fluid level in the oesophagus consistent with scleroderma-associated oesophageal dysfunction.
Figure 2.
Figure 2.
High-resolution computed tomography from a patient with systemic sclerosis showing peripheral and basilar predominate reticulation and honeycombing with an absence of significant ground-glass opacities in a pattern consistent with usual interstitial pneumonia. The patient also has an air-filled oesophagus consistent with scleroderma-associated oesophageal dysfunction.
Figure 3.
Figure 3.
Histopathology results from a patient with systemic sclerosis and nonspecific interstitial pneumonia showing cellular interstitial infiltrates in a temporally uniform distribution.
Figure 4.
Figure 4.
Histopathology results from a patient with systemic sclerosis and usual interstitial pneumonia showing patchy interstitial fibrosis in close proximity to unaffected lung tissue.
Figure 5.
Figure 5.
A pulmonary arteriole from a patient with systemic sclerosis-associated pulmonary artery hypertension showing significant medial hypertrophy.
Figure 6.
Figure 6.
Extent of interstitial lung disease (ILD) in patients with systemic sclerosis-associated ILD. A simple stratification that utilises pulmonary function tests (PFTs) and extent of disease on high-resolution computed tomography (HRCT) to provide discriminatory prognostic information. FVC: forced vital capacity. Reproduced from [28] with permission from the publisher.
Figure 7.
Figure 7.
A suggested approach for the long-term follow-up of patients with systemic sclerosis-interstitial lung disease. PFTs: pulmonary function tests; HRCT: high-resolution computed tomography.
Figure 8.
Figure 8.
An algorithm for the early diagnosis of pulmonary arterial hypertension in systemic sclerosis. RV: right ventricle; TAPSE: tricuspid annular plane systolic excursion; PAP: pulmonary artery pressure; PA: pulmonary artery; BNP: B-type natriuretic protein; lcSSc: limited cutaneous systemic sclerosis; DL,CO: diffusing capacity of the lung for carbon monoxide; RVH: right ventricular hypertrophy; PO2: oxygen tension; TR: tricuspid regurgitation; CT: computed tomography; PE: pulmonary embolism; PF: pulmonary fibrosis; V′/Q′: ventilation/perfusion ratio; ILD: interstitial lung disease; mPAP: mean PAP; PVR: pulmonary vascular resistance; CCB: calcium channel blockers; WHO: World Health Organization; ETRA: endothelin receptor antagonist; PDE: phosphodiesterase. Reproduced from [110] with permission from the publisher.
See this image and copyright information in PMC

References

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    1. Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis 2007; 66: 940–944. - PMC - PubMed
    1. Morelli S, Barbieri C, Sgreccia A, et al. . Relationship between cutaneous and pulmonary involvement in systemic sclerosis. J Rheumatol 1997; 24: 81–85. - PubMed
    1. Highland KB, Garin MC, Brown KK. The spectrum of scleroderma lung disease. Semin Respir Crit Care Med 2007; 28: 418–429. - PubMed
    1. Toya SP, Tzelepis GE. The many faces of scleroderma sine scleroderma: a literature review focusing on cardiopulmonary complications. Rheumatol Int 2009; 29: 861–868. - PubMed

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