Pulmonary emphysema combined with systemic sclerosis (SSc)-associated interstitial lung disease (ILD) occurs more often in smokers but also in never-smokers. This study aimed to describe a new finding characterized by peculiar emphysematous change with SSc-associated ILD (SSc-ILD).
We conducted a retrospective review of 21 consecutive patients with SSc-ILD diagnosed by surgical lung biopsy and focused on the radio-pathological correlation of the emphysematous change.
Pathological pulmonary emphysema (p-PE) with SSc-ILD was the predominant complication in 16 patients (76.2%) with/without a smoking history, of whom 62.5% were never-smokers. A low attenuation area (LAA) within interstitial abnormality on high-resolution computed tomography (HRCT) was present in 31.3%. Diffusing capacity of the lung for carbon monoxide (DLCO) was lower, disease extent on HRCT higher, and intimal/medial thickening in muscular pulmonary arteries more common in the patients with p-PE with SSc-ILD. However, forced vital capacity (FVC) was well preserved regardless of whether p-PE was observed. Most SSc-ILD patients had pulmonary microvasculature changes in arterioles (90.5%), venules (85.7%), and interlobular veins (81.0%).
Pulmonary emphysematous changes (LAA within interstitial abnormalities on HRCT and destruction of fibrously thickened alveolar walls) are specific and novel radio-pathological features of SSc-ILD. Peripheral vasculopathy may help to destroy the fibrously thickened alveolar walls, resulting in emphysematous change in SSc-ILD.
Kim DS, Yoo B, Lee JS, Kim EK, Lim CM, lee SD, et al. the major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis. 2002;19:121–7. PubMed
Katzenstein AL, Fiorelli RF. Nonspecific interstitial pneumonia/fibrosis. Histologic features and clinical significance Am J Surg Pathol. 1994;18:136–47. PubMed
Nicholson AG, Colby TV, wells AU. Histopathological approach to patterns of interstitial pneumonia in patient with connective tissue disorders. Sarcoidosis Vasc Diffuse Lung Dis. 2002;19:10–7. PubMed
Antoniou KM, Margaritopoulos GA, Goh NS, Karagiannis K, Desai SR, Nicholson AG, et al. Combined pulmonary fibrosis and emphysema in scleroderma-related lung disease has a major confounding effect on lung physiology and screening for pulmonary hypertension. Arthritis Rheumatol. 2016;68:1004–12. CrossRefPubMed
American Thoracic Society. European Respiratory Society. American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS executive committee, June 2001. Am J Respir Crit Care Med. 2002;166:426.
Travis WD, Costabel U, Hansell DM, King TE Jr, Lynch DA, Nicholson AG, et al. ATS/ERS Committee on idiopathic interstitial pneumonias. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188:733–48. CrossRefPubMed
Corrin, B, Nicholson AG: Pathology of the lungs. 3rd ed. Philadelphia, Elsevier, 2011,pp 91–134.
Awano N, Inomata M, Ikushima S, Yamada D, Hotta M, Tsukuda S, et al. Histological analysis of vasculopathy associated with pulmonary hypertension in combined pulmonary fibrosis and emphysema: comparison with idiopathic pulmonary fibrosis and emphysema alone. Histopathology. 2017;70:896–905. CrossRefPubMed
Vivero M, Padera RF. Histopathology of lung disease in the connective tissue diseases. Rheum Dis Clin N Am. 2015;41:197–211. CrossRef
Fischer A, Antoniou KM, Brown KK, Cadranel J, Corte TJ, du bois RM, et al. “ERS/ATS task force on undifferentiated forms of CTD-ILD”. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J. 2015;46:976–87. CrossRefPubMed
- Emphysematous change with scleroderma-associated interstitial lung disease: the potential contribution of vasculopathy?
- BioMed Central
Neu im Fachgebiet Innere Medizin
Meistgelesene Bücher aus der Inneren Medizin
Mail Icon II