IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES Unilateral Pulmonary Fibrosis and Systemic Sclerosis Viktor N. Lesnyak1, Olesya V. Danilevskaya2, Alexander V. Averyanov3,4, and Lidia P. Ananyeva5 1 Department of Radiology, 2Department of Endoscopy, 3Department of Research, 4Department of Pulmonology, Federal Research Clinical Center Federal Medical and Biological Agency of Russia, Moscow, Russia; and 5Institute of Rheumatology, Russian Academy of Medical Science, Moscow, Russia
Figure 1. (A) Frontal computed tomography slice demonstrates a volume reduction of the right lung with the signs of diffuse interstitial fibrosis. Dilated esophagus is shown as well. (B) Axial computed tomography demonstrates ground-glass opacity, reticular pattern, and honeycombing only in the lower lobe of the right lung.
Figure 2. Confocal laser endomicroscopy picture shows highly fluorescent structures that appeared to be fibrotic tissue in the right lung (A), whereas in the left one (B), the normal alveolar pattern is preserved.
Unilateral interstitial pulmonary fibrosis is a very rare lung lesion associated with proximal interruption of the pulmonary artery, pulmonary vein thrombosis, ipsilateral singlelung ventilation, or radiation pneumonitis (1–4). We report a case of a 42-year-old woman with systemic sclerosis (SS) who has been noting dyspnea on exertion for the last 5 years. This diagnosis had been made 2 years before the onset of the respiratory symptoms and was based on the presence of skin thickening of the fingers of both hands extending proximal to the metacarpophalangeal joints, Raynaud’s phenomenon, and SS-related autoantibodies (5). The functional tests revealed a restrictive pattern and a slight decrease in carbon monoxide diffusion: FVC, 64.7%; FEV1, 71.9%; TLCO, 68.5%. High-resolution computed tomography showed the signs of diffuse interstitial fibrosis in the right lung: thickening of interlobular septa, traction bronchiectasis, and subpleural honeycombing mostly expressed in basal segments. The left lung was intact (Figure 1). Transbronchial lung biopsy revealed the features of usual interstitial pneumonia. Significant dilatation of the esophagus in the middle and lower parts was also found. Confocal laser endomicroscopy of distal airways confirmed the signs of pulmonary fibrosis of the right lung (Figure 2A), whereas the left lung had a normal alveolar pattern (Figure 2B). The confocal laser endomicroscopy picture in SS has not been previously described and demonstrates a significant difference between healthy lung and fibrosis with the total absence of alveolar structure. Among the possible causes of unilateral or predominantly unilateral pulmonary
Am J Respir Crit Care Med Vol 190, Iss 9, pp 1067–1068, Nov 1, 2014 Copyright © 2014 by the American Thoracic Society DOI: 10.1164/rccm.201407-1253IM Internet address: www.atsjournals.org
Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences
1067
IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES fibrosis, there are circulatory disorders, radiation-induced or ventilator-induced lung injury, and local chronic inflammatory disease such as bronchiectasis and gastroesophageal reflux disease. Tcherakian and colleagues identified 32 subjects with asymmetric lesion among 96 patients with idiopathic pulmonary fibrosis, but none with unilateral lesion (6). In addition, these irregularly distributed interstitial changes were more expressed in the right lung in those patients who had gastroesophageal reflux disease. Moreover, in recent years, gastroesophageal reflux disease has been considered one of the main triggers of idiopathic pulmonary fibrosis (7). On the basis of the exclusion of lung vessels pathology by computed tomography pulmonary angiogram, the most probable cause of unilateral computed tomography findings in this case study is esophageal dilatation with chronic microaspiration and sleep preference on the right side. n Author disclosures are available with the text of this article at www.atsjournals.org.
References 1. Sakai S, Murayama S, Soeda H, Furuya A, Ono M, Ro T, Akamine T, Masuda K. Unilateral proximal interruption of the pulmonary artery in adults: CT findings in eight patients. J Comput Assist Tomogr 2002; 26:777–783. 2. Cavaco RA, Kaul S, Chapman T, Casaretti R, Philips B, Rhodes A, Grounds MR. Idiopathic pulmonary fibrosis associated with pulmonary vein thrombosis: a case report. Cases J 2009;2:9156. 3. Kimura T, Mizutani T. [Unilateral pulmonary fibrosis following ipsilateral single-lung ventilation and anesthesia]. Masui 2001;50:651–654. 4. Mehta V. Radiation pneumonitis and pulmonary fibrosis in non-small-cell lung cancer: pulmonary function, prediction, and prevention. Int J Radiat Oncol Biol Phys 2005;63:5–24.
1068
5. van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA Jr, Carreira PE, et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis 2013;72: 1747–1755. 6. Tcherakian C, Cottin V, Brillet PY, Freynet O, Naggara N, Carton Z, Cordier JF, Brauner M, Valeyre D, Nunes H. Progression of idiopathic pulmonary fibrosis: lessons from asymmetrical disease. Thorax 2011;66:226–231. 7. Savarino E, Carbone R, Marabotto E, Furnari M, Sconfienza L, Ghio M, Zentilin P, Savarino V. Gastro-oesophageal reflux and gastric aspiration in idiopathic pulmonary fibrosis patients. Eur Respir J 2013;42:1322–1331.
American Journal of Respiratory and Critical Care Medicine Volume 190 Number 9 | November 1 2014
Figure 1. (A) Frontal computed tomography slice demonstrates a volume reduction of the right lung with the signs of diffuse interstitial fibrosis. Dilated esophagus is shown as well. (B) Axial computed tomography demonstrates ground-glass opacity, reticular pattern, and honeycombing only in the lower lobe of the right lung.
Figure 2. Confocal laser endomicroscopy picture shows highly fluorescent structures that appeared to be fibrotic tissue in the right lung (A), whereas in the left one (B), the normal alveolar pattern is preserved.
Unilateral interstitial pulmonary fibrosis is a very rare lung lesion associated with proximal interruption of the pulmonary artery, pulmonary vein thrombosis, ipsilateral singlelung ventilation, or radiation pneumonitis (1–4). We report a case of a 42-year-old woman with systemic sclerosis (SS) who has been noting dyspnea on exertion for the last 5 years. This diagnosis had been made 2 years before the onset of the respiratory symptoms and was based on the presence of skin thickening of the fingers of both hands extending proximal to the metacarpophalangeal joints, Raynaud’s phenomenon, and SS-related autoantibodies (5). The functional tests revealed a restrictive pattern and a slight decrease in carbon monoxide diffusion: FVC, 64.7%; FEV1, 71.9%; TLCO, 68.5%. High-resolution computed tomography showed the signs of diffuse interstitial fibrosis in the right lung: thickening of interlobular septa, traction bronchiectasis, and subpleural honeycombing mostly expressed in basal segments. The left lung was intact (Figure 1). Transbronchial lung biopsy revealed the features of usual interstitial pneumonia. Significant dilatation of the esophagus in the middle and lower parts was also found. Confocal laser endomicroscopy of distal airways confirmed the signs of pulmonary fibrosis of the right lung (Figure 2A), whereas the left lung had a normal alveolar pattern (Figure 2B). The confocal laser endomicroscopy picture in SS has not been previously described and demonstrates a significant difference between healthy lung and fibrosis with the total absence of alveolar structure. Among the possible causes of unilateral or predominantly unilateral pulmonary
Am J Respir Crit Care Med Vol 190, Iss 9, pp 1067–1068, Nov 1, 2014 Copyright © 2014 by the American Thoracic Society DOI: 10.1164/rccm.201407-1253IM Internet address: www.atsjournals.org
Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences
1067
IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES fibrosis, there are circulatory disorders, radiation-induced or ventilator-induced lung injury, and local chronic inflammatory disease such as bronchiectasis and gastroesophageal reflux disease. Tcherakian and colleagues identified 32 subjects with asymmetric lesion among 96 patients with idiopathic pulmonary fibrosis, but none with unilateral lesion (6). In addition, these irregularly distributed interstitial changes were more expressed in the right lung in those patients who had gastroesophageal reflux disease. Moreover, in recent years, gastroesophageal reflux disease has been considered one of the main triggers of idiopathic pulmonary fibrosis (7). On the basis of the exclusion of lung vessels pathology by computed tomography pulmonary angiogram, the most probable cause of unilateral computed tomography findings in this case study is esophageal dilatation with chronic microaspiration and sleep preference on the right side. n Author disclosures are available with the text of this article at www.atsjournals.org.
References 1. Sakai S, Murayama S, Soeda H, Furuya A, Ono M, Ro T, Akamine T, Masuda K. Unilateral proximal interruption of the pulmonary artery in adults: CT findings in eight patients. J Comput Assist Tomogr 2002; 26:777–783. 2. Cavaco RA, Kaul S, Chapman T, Casaretti R, Philips B, Rhodes A, Grounds MR. Idiopathic pulmonary fibrosis associated with pulmonary vein thrombosis: a case report. Cases J 2009;2:9156. 3. Kimura T, Mizutani T. [Unilateral pulmonary fibrosis following ipsilateral single-lung ventilation and anesthesia]. Masui 2001;50:651–654. 4. Mehta V. Radiation pneumonitis and pulmonary fibrosis in non-small-cell lung cancer: pulmonary function, prediction, and prevention. Int J Radiat Oncol Biol Phys 2005;63:5–24.
1068
5. van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA Jr, Carreira PE, et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis 2013;72: 1747–1755. 6. Tcherakian C, Cottin V, Brillet PY, Freynet O, Naggara N, Carton Z, Cordier JF, Brauner M, Valeyre D, Nunes H. Progression of idiopathic pulmonary fibrosis: lessons from asymmetrical disease. Thorax 2011;66:226–231. 7. Savarino E, Carbone R, Marabotto E, Furnari M, Sconfienza L, Ghio M, Zentilin P, Savarino V. Gastro-oesophageal reflux and gastric aspiration in idiopathic pulmonary fibrosis patients. Eur Respir J 2013;42:1322–1331.
American Journal of Respiratory and Critical Care Medicine Volume 190 Number 9 | November 1 2014
