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Lung Transplantation as Successful Treatment of End-stage Idiopathic Pleuroparenchymal Fibroelastosis: A Case Report
Lung Transplantation as Successful Treatment of End-stage Idiopathic Pleuroparenchymal Fibroelastosis: A Case Report I. Righia,*, L. Morlacchib,c, V. Rossettib,c, P. Mendognia, A. Palleschia, D. Tosia, S. Pieropana, A. Del Gobbod, and M. Nosottia,b a Thoracic Surgery and Lung Transplant Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy; bDepartment of Pathophysiology and Transplantation, University of Milan, Milan, Italy; cDepartment of Internal Medicine, Respiratory Unit and Adult Cystic Fibrosis Center, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy; and dDivision of Pathology, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
ABSTRACT Pleuroparenchymal fibroelastosis (PPFE) is a rare condition, characterized by predominantly upper-lobe pleural and subjacent parenchymal fibrosis, the latter being intraalveolar with accompanying elastosis of the alveolar walls that leads a clinical progression to respiratory failure. This condition may not be as rare as it seems to be, because nowadays the increasing awareness among specialists is raising the number of new diagnoses. Limited data are available about the prognosis, both for secondary and idiopathic forms. Nevertheless, the idiopathic form seems to be rapidly progressive and no treatment can control the disease, which is why management is challenging. Since the disease was characterized, PPFE cases have been reported in the literature, but most have been secondary rather than idiopathic. Of these, few have successfully undergone lung transplantation as a treatment of end-stage respiratory failure. We here report a successful case of a 38-year-old man affected by idiopathic PPFE who underwent bilateral lung transplantation after extracorporeal membrane oxygenation bridging for an abrupt transition to critical clinical conditions. After a complex postoperative course and a first year characterized by acute rejection, the patient is alive at 5 years with a good quality of life. Our experience confirms that lung transplantation would be a valuable treatment option in case of end-stage idiopathic PPFE cases.
I
DIOPATHIC pleuroparenchymal fibroelastosis (IPPFE) is a rare condition, first described by Amitani et al as upper-lobe fibrosis [1] and recently included as a rare idiopathic interstitial pneumonia according to the updated American Thoracic Society/European Respiratory Society classification [2]. The disease has no sex predilection and no relation to smoking history. The patients’ ages range from 13 to 87 years old (mean 53 years) [3]. The disease is characterized by dense established intraalveolar fibrosis with the alveolar walls in these areas showing prominent elastosis and dense fibrous thickening of the visceral pleura; these changes have a striking upper-zone predominance [4]. Age at onset is variable and although the exact etiology is still unknown, risk factors may include bone marrow transplantation, chemotherapy, radiotherapy, recurrent infections, and genetic and autoimmune. Common symptoms are weight loss, exertional dyspnea, dry ª 2018 Elsevier Inc. All rights reserved. 230 Park Avenue, New York, NY 10169
Transplantation Proceedings, XX, 1e4 (2018)
cough, and pleuritic chest pain due to pneumothorax [5]. Evidence-based treatment options are not currently available. Lung transplantation itself maybe another underlying condition that could induce secondary pleuroparenchymal fibroelastosis (PPFE) as a form of chronic allograft dysfunction [6]. To the best of our knowledge, only a few cases of lung transplantation have been reported for this
*Address correspondence to Ilaria Righi, MD, Thoracic Surgery and Lung Transplant Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, University of Milan, Via Francesco Sforza, 35, Milan 20122 Italy. Tel: þ390255035570, þ393470556707; Fax: þ390255035587. E-mail: [email protected] 0041-1345/18 https://doi.org/10.1016/j.transproceed.2018.04.071
1
2
RIGHI, MORLACCHI, ROSSETTI ET AL
Fig 1. (A) Histopathologic features: dense subpleural fibrosis with elastosis and a network of elastin deposition into alveolar walls, which lead the entrapment of the terminal airways. (B) Radiological features: the disease is localized in the upper lobes, with the typical pleuroparenchymal thickening and subpleural fibrosis.
indication; moreover, the majority of these patients were affected by secondary forms of PPFE [7e10]. We here describe the case of a 42-year-old man who underwent bilateral lung transplant for IPPFE at our center in 2013. Written informed consent was obtained from the patient for publication of this case report and accompanying images. CASE REPORT In 2010, a 38-year-old patient for recurrent pneumothorax underwent thoracoscopy and bullectomy: at the histology the diagnosis was pleuroparenchymal fibroelastosis. Histologically, pleuropulmonary fibroelastosis is characterized by dense fibrosis of the visceral pleura that became thicker, associated with diffuse intraalveolar fibrosis, mainly localized into the upper lobes. In subpleural parenchyma, the alveolar septa usually present with elastosis highlighted by Van Gieson histochemical stain and with areas of parenchymal sparing that are sharply separated from one another. Distant parenchyma could show areas of obliterative bronchiolitis and areas of usual interstitial pneumonia with a honeycomb
pattern. Lymphocytes foci are usually associated, and small number of fibroblastic areas can also be seen (Fig 1A). During the preceding years the patient had been suffering from a tickly cough and chronic weight loss. At the time of clinical inspection the thoracic cage was flattened (plathythorax), and the patient was weak and unfit. After surgery, our pneumological team took care of the patient; in the ensuing years the flattened thoracic cage got worse along with the ventilatory dysfunction, and the necessity of oxygen therapy increased. A high-resolution computed tomography scan (Fig 1B) revealed irregular pleural thickening and reticular fibrosis predominantly in the upper lobes, with clear demarcation between the normal and the abnormal parenchyma, as described in literature [11]. Because of his poor and rapidly worsening general condition, at the end of 2012 the patient entered a rehabilitation center for both respiratory and nutritional reconditioning; however, this produced little improvement. The increasing need for oxygen for any physical exertion lead to muscle loss and eventually immobilization. In March 2013 the patient, then aged 42, was admitted to intensive care unit of our hospital for acute respiratory failure and underwent extracorporeal membrane oxygenation treatment bridging to lung transplant. The acute distress respiratory syndrome was probably
Fig 2. Chest radiograph 6 months after transplantation. In the lateral view it is evident that the flattened thoracic cage has become normal (the little one is the preoperative computed tomography scan scout).
SUCCESSFUL TREATMENT OF FIBROELASTOSIS due to lung infection. The organ suitable for this recipient had marginal function (Pa02 with 100% 02 was 320 mm Hg), so it was reconditioned using ex vivo lung perfusion with Toronto’s protocol [12], achieving a very good final function (Pa02 480 mm Hg with 100% 02). The postoperative course was characterized by primary graft dysfunction, so that the patient needed mechanical ventilation and extracorporeal membrane oxygenation for 5 days after the transplant. Pneumonia from Klebsiella pneumoniae occurred and was successfully treated with target antibiotic therapy. A tracheostomy was performed to resolve ventilation difficulties, and at 30 days after surgery the patient was sent to a rehabilitation ward, where gradually he reached a very good level of lung function and nutritional status (best postoperative forced expiratory volume in 1 second [FEV1] at 3 months: 75%). Unfortunately, in the first year acute rejection happened but was treated effectively with corticosteroids (we assisted to a prompt stabilization of FEV1 decline). From this event a chronic rejection has been developed, and since 2015 the patient has successfully received courses of extracorporeal photopheresis to stabilize respiratory function. In spite of chronic rejection, at 5 years from lung transplantation the patients is alive, with a good quality of life and an FEV1 of 69%. What it matters also is that there was an evident normalization of body mass index with improvement of the shape of thoracic cage, which became “tridimensional” instead of flattened as it was before (Fig 2).
DISCUSSION
Since 1992, when PPFE was first described by Amitani et al [1], few case series were reported in literature, divided into idiopathic and secondary type; the largest numbers included 12, 9, and 15 patients [5,13,14]. This disease has a typical clinical, radiological, and histologic pattern, and the idiopathic type is associated with a worse prognosis, as the secondary type is related to chronic graft-versus-host disease following bone marrow transplantation and to chronic rejection after lung transplant [6,15]. Despite this small number, recent studies highlight that this diagnosis has been under reported rather than rare [16]. In fact, besides fibroelastosis, in 75% of reviewed cases there was a coexistent interstitiopathy [13] that lead to a misleading diagnosis. The pathogenesis still remains unclear, and it has been proposed that in any case the trigger is acute lung injury or interstitial inflammation [3], as the history of recurrent infections reported in half of all cases suggests, with contributions from genetic predisposition and autoimmunity. Nevertheless, once the diagnosis of PPFE has been made, follow-up and therapeutic measures should be evaluated promptly because of the disease’s often rapidly progressive course and the lack of treatment guidelines. IPPFE has often a quick progression and it is refractory to corticosteroids and other immunosuppressive therapy [17]. Most of the studies that have been published deal with secondary PPFE; idiopathic cases have been less often described, particularly with regard to treatment options and prognoses. Our clinical case concerns a 42-year-old patient, in contrast to the IPPFE cases reported by Kusagaya et al
3
[18] (5 patients) where the range of age was 67 to 74 years old, but in accord with the case series (12 patients) of Reddy et al [13], where the ages ranged from 24 to 85 years old. The young age of the patient was the reason why our multidisciplinary transplant team decided to make any effort for care for him until lung transplantation, despite his poor nutritional condition and performance status. CONCLUSION
This case report demonstrates that lung transplantation is a valid treatment choice in case of IPPFE with end-stage lung disease, despite the weakness associated with this kind of disease. In particular, the thoracic cage reshapes itself after transplantation, in the absence of retraction of fibrosis and thanks to normal lung inflation (Fig 2). REFERENCES [1] Amitani R, Niimi A, Kuse F, et al. Idiopathic pulmonary upper-lobe fibrosis (IPUF). Kokyu 1992;11:693e9. [2] Travis WD, Costabel U, Hansell DM, et al. 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;188:733e48. [3] Cheng SK, Chuah KL. Pleuroparenchymal fibroelastosis of the lung: a review. Arch Pathol Lab Med 2016;140:849e53. [4] Frankel SK, Cool CD, Lynch DA, et al. Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest 2004;126:2007e13. [5] Watanabe K. Pleuroparenchymal fibroelastosis: its clinical characteristics. Curr Respir Med Rev 2013;9:229e37. [6] Hirota T, Fujita M, Matsumoto T, et al. Pleuroparenchymal fibroelastosis as a manifestation of chronic lung rejection? Eur Respir J 2013;41:243e5. [7] Chen F, Matsubara K, Miyagawa-Hayashino A, et al. Lung transplantation for pleuroparenchymal fibroelastosis after chemotherapy. Ann Thorac Surg 2014;98:e115e7. [8] Huang H, Feng R, Li S, et al. A CARE-compliant case report lung transplantation for a Chinese young man with idiopathic pleuroparenchymal fibroelastosis. Medicine (Baltimore) 2017;96: e6900. [9] Gomes PS, Shiang C, Szarf G, et al. Pleuroparenchymal fibroelastosis: report of two cases in Brazil. J Bras Pneumol 2017;43: 72e5. [10] Hata A, Nakajima T, Yoshida S, et al. Living donor lung transplantation for pleuroparenchymal fibroelastosis. Ann Thorac Surg 2016;101:1970e2. [11] Bonifazi M, Angeles Montero M, Renzoni EA. Idiopathic pleuroparenchymal fibroelastosis. Curr Pulmonol Rep 2017;6:9e15. [12] Cypel M, Yeung JC, Hirayama S, et al. Technique for prolonged normothermic ex vivo lung perfusion. J Heart Lung Transplant 2008;27:1319e25. [13] Reddy TL, Tominaga M, Hansell DM, von der Thusen J, Rassl D, Parfrey H, et al. Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J 2012;40:377e8. [14] Ofek E, Sato M, Saito T, Wagnetz U, Roberts HC, Chaparro C, et al. Restrictive allograft syndrome post lung transplantation is characterized by pleuroparenchymal fibroelastosis. Mod Pathol 2013;26:350e6.
4 [15] Fujikura Y, Kanoh S, Kouzaki Y, Hara Y, Matsubara O, Kawana A. Pleuroparenchymal fibroelastosis as a series of airway complications associated with chronic graft-versus-host disease following allogeneic bone marrow transplantation. Intern Med 2014;53:43e6. [16] Rosenbaum JN, Butt YM, Johnson KA, et al. Pleuroparenchymal fibroelastosis: a pattern of chronic lung injury. Hum Pathol 2015;46:137e46.
RIGHI, MORLACCHI, ROSSETTI ET AL [17] Kokosi MA, Nicholson AG, Hansell AG, Wells AU. Rare idiopathic interstitial pneumonias: LIP and PPFE and rare histologic patterns of interstitial pneumonias: AFOP and BPIP. Respirology 2016;21:600e14. [18] Kusagaya H, Nakamura Y, Kono M, et al. Idiopathic pleuroparenchymal fibroelastosis: consideration of a clinicopathological entity in a series of Japanese patients. BMC Pulm Med 2012;12:72.
ABSTRACT Pleuroparenchymal fibroelastosis (PPFE) is a rare condition, characterized by predominantly upper-lobe pleural and subjacent parenchymal fibrosis, the latter being intraalveolar with accompanying elastosis of the alveolar walls that leads a clinical progression to respiratory failure. This condition may not be as rare as it seems to be, because nowadays the increasing awareness among specialists is raising the number of new diagnoses. Limited data are available about the prognosis, both for secondary and idiopathic forms. Nevertheless, the idiopathic form seems to be rapidly progressive and no treatment can control the disease, which is why management is challenging. Since the disease was characterized, PPFE cases have been reported in the literature, but most have been secondary rather than idiopathic. Of these, few have successfully undergone lung transplantation as a treatment of end-stage respiratory failure. We here report a successful case of a 38-year-old man affected by idiopathic PPFE who underwent bilateral lung transplantation after extracorporeal membrane oxygenation bridging for an abrupt transition to critical clinical conditions. After a complex postoperative course and a first year characterized by acute rejection, the patient is alive at 5 years with a good quality of life. Our experience confirms that lung transplantation would be a valuable treatment option in case of end-stage idiopathic PPFE cases.
I
DIOPATHIC pleuroparenchymal fibroelastosis (IPPFE) is a rare condition, first described by Amitani et al as upper-lobe fibrosis [1] and recently included as a rare idiopathic interstitial pneumonia according to the updated American Thoracic Society/European Respiratory Society classification [2]. The disease has no sex predilection and no relation to smoking history. The patients’ ages range from 13 to 87 years old (mean 53 years) [3]. The disease is characterized by dense established intraalveolar fibrosis with the alveolar walls in these areas showing prominent elastosis and dense fibrous thickening of the visceral pleura; these changes have a striking upper-zone predominance [4]. Age at onset is variable and although the exact etiology is still unknown, risk factors may include bone marrow transplantation, chemotherapy, radiotherapy, recurrent infections, and genetic and autoimmune. Common symptoms are weight loss, exertional dyspnea, dry ª 2018 Elsevier Inc. All rights reserved. 230 Park Avenue, New York, NY 10169
Transplantation Proceedings, XX, 1e4 (2018)
cough, and pleuritic chest pain due to pneumothorax [5]. Evidence-based treatment options are not currently available. Lung transplantation itself maybe another underlying condition that could induce secondary pleuroparenchymal fibroelastosis (PPFE) as a form of chronic allograft dysfunction [6]. To the best of our knowledge, only a few cases of lung transplantation have been reported for this
*Address correspondence to Ilaria Righi, MD, Thoracic Surgery and Lung Transplant Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, University of Milan, Via Francesco Sforza, 35, Milan 20122 Italy. Tel: þ390255035570, þ393470556707; Fax: þ390255035587. E-mail: [email protected] 0041-1345/18 https://doi.org/10.1016/j.transproceed.2018.04.071
1
2
RIGHI, MORLACCHI, ROSSETTI ET AL
Fig 1. (A) Histopathologic features: dense subpleural fibrosis with elastosis and a network of elastin deposition into alveolar walls, which lead the entrapment of the terminal airways. (B) Radiological features: the disease is localized in the upper lobes, with the typical pleuroparenchymal thickening and subpleural fibrosis.
indication; moreover, the majority of these patients were affected by secondary forms of PPFE [7e10]. We here describe the case of a 42-year-old man who underwent bilateral lung transplant for IPPFE at our center in 2013. Written informed consent was obtained from the patient for publication of this case report and accompanying images. CASE REPORT In 2010, a 38-year-old patient for recurrent pneumothorax underwent thoracoscopy and bullectomy: at the histology the diagnosis was pleuroparenchymal fibroelastosis. Histologically, pleuropulmonary fibroelastosis is characterized by dense fibrosis of the visceral pleura that became thicker, associated with diffuse intraalveolar fibrosis, mainly localized into the upper lobes. In subpleural parenchyma, the alveolar septa usually present with elastosis highlighted by Van Gieson histochemical stain and with areas of parenchymal sparing that are sharply separated from one another. Distant parenchyma could show areas of obliterative bronchiolitis and areas of usual interstitial pneumonia with a honeycomb
pattern. Lymphocytes foci are usually associated, and small number of fibroblastic areas can also be seen (Fig 1A). During the preceding years the patient had been suffering from a tickly cough and chronic weight loss. At the time of clinical inspection the thoracic cage was flattened (plathythorax), and the patient was weak and unfit. After surgery, our pneumological team took care of the patient; in the ensuing years the flattened thoracic cage got worse along with the ventilatory dysfunction, and the necessity of oxygen therapy increased. A high-resolution computed tomography scan (Fig 1B) revealed irregular pleural thickening and reticular fibrosis predominantly in the upper lobes, with clear demarcation between the normal and the abnormal parenchyma, as described in literature [11]. Because of his poor and rapidly worsening general condition, at the end of 2012 the patient entered a rehabilitation center for both respiratory and nutritional reconditioning; however, this produced little improvement. The increasing need for oxygen for any physical exertion lead to muscle loss and eventually immobilization. In March 2013 the patient, then aged 42, was admitted to intensive care unit of our hospital for acute respiratory failure and underwent extracorporeal membrane oxygenation treatment bridging to lung transplant. The acute distress respiratory syndrome was probably
Fig 2. Chest radiograph 6 months after transplantation. In the lateral view it is evident that the flattened thoracic cage has become normal (the little one is the preoperative computed tomography scan scout).
SUCCESSFUL TREATMENT OF FIBROELASTOSIS due to lung infection. The organ suitable for this recipient had marginal function (Pa02 with 100% 02 was 320 mm Hg), so it was reconditioned using ex vivo lung perfusion with Toronto’s protocol [12], achieving a very good final function (Pa02 480 mm Hg with 100% 02). The postoperative course was characterized by primary graft dysfunction, so that the patient needed mechanical ventilation and extracorporeal membrane oxygenation for 5 days after the transplant. Pneumonia from Klebsiella pneumoniae occurred and was successfully treated with target antibiotic therapy. A tracheostomy was performed to resolve ventilation difficulties, and at 30 days after surgery the patient was sent to a rehabilitation ward, where gradually he reached a very good level of lung function and nutritional status (best postoperative forced expiratory volume in 1 second [FEV1] at 3 months: 75%). Unfortunately, in the first year acute rejection happened but was treated effectively with corticosteroids (we assisted to a prompt stabilization of FEV1 decline). From this event a chronic rejection has been developed, and since 2015 the patient has successfully received courses of extracorporeal photopheresis to stabilize respiratory function. In spite of chronic rejection, at 5 years from lung transplantation the patients is alive, with a good quality of life and an FEV1 of 69%. What it matters also is that there was an evident normalization of body mass index with improvement of the shape of thoracic cage, which became “tridimensional” instead of flattened as it was before (Fig 2).
DISCUSSION
Since 1992, when PPFE was first described by Amitani et al [1], few case series were reported in literature, divided into idiopathic and secondary type; the largest numbers included 12, 9, and 15 patients [5,13,14]. This disease has a typical clinical, radiological, and histologic pattern, and the idiopathic type is associated with a worse prognosis, as the secondary type is related to chronic graft-versus-host disease following bone marrow transplantation and to chronic rejection after lung transplant [6,15]. Despite this small number, recent studies highlight that this diagnosis has been under reported rather than rare [16]. In fact, besides fibroelastosis, in 75% of reviewed cases there was a coexistent interstitiopathy [13] that lead to a misleading diagnosis. The pathogenesis still remains unclear, and it has been proposed that in any case the trigger is acute lung injury or interstitial inflammation [3], as the history of recurrent infections reported in half of all cases suggests, with contributions from genetic predisposition and autoimmunity. Nevertheless, once the diagnosis of PPFE has been made, follow-up and therapeutic measures should be evaluated promptly because of the disease’s often rapidly progressive course and the lack of treatment guidelines. IPPFE has often a quick progression and it is refractory to corticosteroids and other immunosuppressive therapy [17]. Most of the studies that have been published deal with secondary PPFE; idiopathic cases have been less often described, particularly with regard to treatment options and prognoses. Our clinical case concerns a 42-year-old patient, in contrast to the IPPFE cases reported by Kusagaya et al
3
[18] (5 patients) where the range of age was 67 to 74 years old, but in accord with the case series (12 patients) of Reddy et al [13], where the ages ranged from 24 to 85 years old. The young age of the patient was the reason why our multidisciplinary transplant team decided to make any effort for care for him until lung transplantation, despite his poor nutritional condition and performance status. CONCLUSION
This case report demonstrates that lung transplantation is a valid treatment choice in case of IPPFE with end-stage lung disease, despite the weakness associated with this kind of disease. In particular, the thoracic cage reshapes itself after transplantation, in the absence of retraction of fibrosis and thanks to normal lung inflation (Fig 2). REFERENCES [1] Amitani R, Niimi A, Kuse F, et al. Idiopathic pulmonary upper-lobe fibrosis (IPUF). Kokyu 1992;11:693e9. [2] Travis WD, Costabel U, Hansell DM, et al. 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;188:733e48. [3] Cheng SK, Chuah KL. Pleuroparenchymal fibroelastosis of the lung: a review. Arch Pathol Lab Med 2016;140:849e53. [4] Frankel SK, Cool CD, Lynch DA, et al. Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest 2004;126:2007e13. [5] Watanabe K. Pleuroparenchymal fibroelastosis: its clinical characteristics. Curr Respir Med Rev 2013;9:229e37. [6] Hirota T, Fujita M, Matsumoto T, et al. Pleuroparenchymal fibroelastosis as a manifestation of chronic lung rejection? Eur Respir J 2013;41:243e5. [7] Chen F, Matsubara K, Miyagawa-Hayashino A, et al. Lung transplantation for pleuroparenchymal fibroelastosis after chemotherapy. Ann Thorac Surg 2014;98:e115e7. [8] Huang H, Feng R, Li S, et al. A CARE-compliant case report lung transplantation for a Chinese young man with idiopathic pleuroparenchymal fibroelastosis. Medicine (Baltimore) 2017;96: e6900. [9] Gomes PS, Shiang C, Szarf G, et al. Pleuroparenchymal fibroelastosis: report of two cases in Brazil. J Bras Pneumol 2017;43: 72e5. [10] Hata A, Nakajima T, Yoshida S, et al. Living donor lung transplantation for pleuroparenchymal fibroelastosis. Ann Thorac Surg 2016;101:1970e2. [11] Bonifazi M, Angeles Montero M, Renzoni EA. Idiopathic pleuroparenchymal fibroelastosis. Curr Pulmonol Rep 2017;6:9e15. [12] Cypel M, Yeung JC, Hirayama S, et al. Technique for prolonged normothermic ex vivo lung perfusion. J Heart Lung Transplant 2008;27:1319e25. [13] Reddy TL, Tominaga M, Hansell DM, von der Thusen J, Rassl D, Parfrey H, et al. Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J 2012;40:377e8. [14] Ofek E, Sato M, Saito T, Wagnetz U, Roberts HC, Chaparro C, et al. Restrictive allograft syndrome post lung transplantation is characterized by pleuroparenchymal fibroelastosis. Mod Pathol 2013;26:350e6.
4 [15] Fujikura Y, Kanoh S, Kouzaki Y, Hara Y, Matsubara O, Kawana A. Pleuroparenchymal fibroelastosis as a series of airway complications associated with chronic graft-versus-host disease following allogeneic bone marrow transplantation. Intern Med 2014;53:43e6. [16] Rosenbaum JN, Butt YM, Johnson KA, et al. Pleuroparenchymal fibroelastosis: a pattern of chronic lung injury. Hum Pathol 2015;46:137e46.
RIGHI, MORLACCHI, ROSSETTI ET AL [17] Kokosi MA, Nicholson AG, Hansell AG, Wells AU. Rare idiopathic interstitial pneumonias: LIP and PPFE and rare histologic patterns of interstitial pneumonias: AFOP and BPIP. Respirology 2016;21:600e14. [18] Kusagaya H, Nakamura Y, Kono M, et al. Idiopathic pleuroparenchymal fibroelastosis: consideration of a clinicopathological entity in a series of Japanese patients. BMC Pulm Med 2012;12:72.