IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES Multifocal Micronodular Pneumocyte Hyperplasia Mimicking Miliary Tuberculosis In Seon Lee1, Hyo Sup Shim2, Hye-Jeong Lee3, and Young Ae Kang1 1 Department of Internal Medicine, 2Department of Pathology, and 3Department of Radiology, Yonsei University College of Medicine, Seoul, Korea
Figure 1. Chest X-ray demonstrating diffuse nodular opacity of the entire lung field.
Figure 2. Chest computed tomography demonstrated sharply and poorly defined small nodules, varying in diameter from 1 to 5 mm, with a diffuse random distribution.
A 34-year-old female was referred for a diffuse nodular opacity of the entire lung field on simple chest radiograph during a routine health examination (Figure 1). She had no respiratory symptoms. Chest computed tomography demonstrated sharply and poorly defined small nodules, varying in diameter from 1 to 5 mm, with a diffuse random distribution in the secondary lobule throughout both lungs, suggesting miliary tuberculosis (Figure 2). Considering the endemicity of tuberculosis in Korea, she was empirically treated with antituberculosis medications. However, even after 1 month of treatment, there was no radiologic improvement. To diagnose the diffuse parenchymal lung opacities, we performed video-assisted lung biopsy. Multifocal micronodular pneumocyte hyperplasia (MMPH) was confirmed by histopathology (Figure 3). She did not demonstrate the classic triad of seizures, mental retardation, and skin lesions. Fundoscopy revealed retinal hamartomas on both eyes. Because she refused further evaluation for tuberous sclerosis complex (TSC), we could not check any other images of her brain and abdomen.
Am J Respir Crit Care Med Vol 189, Iss 2, pp 225–226, Jan 15, 2014 Copyright © 2014 by the American Thoracic Society DOI: 10.1164/rccm.201306-1078IM Internet address: www.atsjournals.org
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IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES
Figure 3. Histopathological findings revealed proliferation of enlarged cuboidal cells (arrows) lining the thickened alveolar septa on hematoxylin and eosin stain of the lung tissue (3200).
Through the review of her family history, we found that her son was diagnosed with TSC 3 years earlier. Genetic analysis revealed a mutation of the TSC2 gene at chromosome 16 (Arg611Gln), which her 3-year-old son and she both had, but her 6-year-old daughter did not. Lymphangioleiomyomatosis and MMPH are common pulmonary manifestations of TSC (1, 2). MMPH is characterized by aberrant proliferation of type 2 pneumocytes along thickened alveolar septa (3) in both female and male patients with TSC. The prognosis in patients with MMPH is relatively good, although respiratory failure can result (4). n Author disclosures are available with the text of this article at www.atsjournals.org.
References 1. Cudzilo CJ, Szczesniak RD, Brody AS, Rattan MS, Krueger DA, Bissler JJ, Franz DN, McCormack FX, Young LR. Lymphangioleiomyomatosis screening in women with tuberous sclerosis. Chest 2013;144: 578–585. 2. Muzykewicz DA, Black ME, Muse V, Numis AL, Rajagopal J, Thiele EA, Sharma A. Multifocal micronodular pneumocyte hyperplasia: computed tomographic appearance and follow-up in tuberous
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sclerosis complex. J Comput Assist Tomogr 2012;36: 518–522. 3. Kobashi Y, Sugiu T, Mouri K, Irei T, Nakata M, Oka M. Clinicopathological analysis of multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis in Japan. Respirology 2008;13:1076–1081. 4. Cancellieri A, Poletti V, Corrin B. Respiratory failure due to micronodular type II pneumocyte hyperplasia. Histopathology 2002;41:263–265.
American Journal of Respiratory and Critical Care Medicine Volume 189 Number 2 | January 15 2014
Figure 1. Chest X-ray demonstrating diffuse nodular opacity of the entire lung field.
Figure 2. Chest computed tomography demonstrated sharply and poorly defined small nodules, varying in diameter from 1 to 5 mm, with a diffuse random distribution.
A 34-year-old female was referred for a diffuse nodular opacity of the entire lung field on simple chest radiograph during a routine health examination (Figure 1). She had no respiratory symptoms. Chest computed tomography demonstrated sharply and poorly defined small nodules, varying in diameter from 1 to 5 mm, with a diffuse random distribution in the secondary lobule throughout both lungs, suggesting miliary tuberculosis (Figure 2). Considering the endemicity of tuberculosis in Korea, she was empirically treated with antituberculosis medications. However, even after 1 month of treatment, there was no radiologic improvement. To diagnose the diffuse parenchymal lung opacities, we performed video-assisted lung biopsy. Multifocal micronodular pneumocyte hyperplasia (MMPH) was confirmed by histopathology (Figure 3). She did not demonstrate the classic triad of seizures, mental retardation, and skin lesions. Fundoscopy revealed retinal hamartomas on both eyes. Because she refused further evaluation for tuberous sclerosis complex (TSC), we could not check any other images of her brain and abdomen.
Am J Respir Crit Care Med Vol 189, Iss 2, pp 225–226, Jan 15, 2014 Copyright © 2014 by the American Thoracic Society DOI: 10.1164/rccm.201306-1078IM Internet address: www.atsjournals.org
Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences
225
IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES
Figure 3. Histopathological findings revealed proliferation of enlarged cuboidal cells (arrows) lining the thickened alveolar septa on hematoxylin and eosin stain of the lung tissue (3200).
Through the review of her family history, we found that her son was diagnosed with TSC 3 years earlier. Genetic analysis revealed a mutation of the TSC2 gene at chromosome 16 (Arg611Gln), which her 3-year-old son and she both had, but her 6-year-old daughter did not. Lymphangioleiomyomatosis and MMPH are common pulmonary manifestations of TSC (1, 2). MMPH is characterized by aberrant proliferation of type 2 pneumocytes along thickened alveolar septa (3) in both female and male patients with TSC. The prognosis in patients with MMPH is relatively good, although respiratory failure can result (4). n Author disclosures are available with the text of this article at www.atsjournals.org.
References 1. Cudzilo CJ, Szczesniak RD, Brody AS, Rattan MS, Krueger DA, Bissler JJ, Franz DN, McCormack FX, Young LR. Lymphangioleiomyomatosis screening in women with tuberous sclerosis. Chest 2013;144: 578–585. 2. Muzykewicz DA, Black ME, Muse V, Numis AL, Rajagopal J, Thiele EA, Sharma A. Multifocal micronodular pneumocyte hyperplasia: computed tomographic appearance and follow-up in tuberous
226
sclerosis complex. J Comput Assist Tomogr 2012;36: 518–522. 3. Kobashi Y, Sugiu T, Mouri K, Irei T, Nakata M, Oka M. Clinicopathological analysis of multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis in Japan. Respirology 2008;13:1076–1081. 4. Cancellieri A, Poletti V, Corrin B. Respiratory failure due to micronodular type II pneumocyte hyperplasia. Histopathology 2002;41:263–265.
American Journal of Respiratory and Critical Care Medicine Volume 189 Number 2 | January 15 2014
