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P237 Pleuroparenchymal fibroelastosis in a previously healthy woman
Poster Walks Poster Walk 8: Interstitial lung disease/ICU/Pediatrics
P237 Pleuroparenchymal fibroelastosis in a previously healthy woman J. Cravo, A. Catarino, L. Carvalho, T. Alfaro Pneumologia A, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal Pleuroparenchymal fibroelastosis (PPFE) is a rare disease characterized by a fibrosing process affecting the pleura and the subpleural lung parenchyma, with a predilection for the upper lobes. The etiology of PPFE is unclear at this juncture, about half of cases are felt to be idiopathic. It usually shows a progressive clinical course and has a poor prognosis. There is currently no specific therapy, but in progressive cases, lung transplantation may be required. Authors will present a clinical case. A 74 year-old nonsmoking lady was referred to the respiratory outpatient clinic for persistent chest X-ray infiltrates. Five months earlier, she was admitted to a medical ward for community-acquired pneumonia, with a good clinical response to antibiotics, but non-resolving imaging findings. She had no relevant past medical history or occupational exposures, but lived in a farm, and was in contact with a dog and some sheep. At observation she denied any respiratory complaints and had normal breath sounds and a room air oxygen saturation of 98%. Her chest x-ray showed worsening bilateral apical opacities. Lung function tests revealed a restrictive limitation with low diffusion capacity (vital capacity of 60% predicted and diffusion capacity of 59% predicted). A Chest CT was ordered, and showed fibrotic residual infiltrates in the apex of both lungs, with traction bronchiectasis and a possibly malignant 2.5 cm nodule with a radiologist’s recommendation for biopsy, which showed non-recent fibrosis and lymphocytes in the bronchiolar walls. Follow-up discussion in a multidisciplinary meeting led to suspicion of PPFE and a review of the biopsy, including staining for elastin using PAS and silver. This led to the identification of a pattern of fibrosis with an elastin rich matrix, compatible with PPFE. The patient remained asymptomatic and declined steroid treatment. Referral for lung transplantation was not considered due to her advanced age. She is currently under surveillance and under consideration for pirfenidone treatment.
DOI:
http://dx.doi.org/10.1016/j.chest.2017.04.144
Copyright ª 2017 American College of Chest Physicians and Swiss Respiratory Society SGP. Published by Elsevier Inc and Karger. All rights reserved.
journal.publications.chestnet.org
137A
POSTER WALKS
Although PPFE is a rare disease, advances in its recognition will probably lead to more frequent diagnoses, and improved knowledge. In our case, the discussion of this complex patient in a multi-disciplinary team meeting was instrumental for the correct diagnosis. This report illustrates how an elevated suspicion index in fundamental for the diagnosis of PPFE and a multidisciplinary discussion may facilitate this.
P237 Pleuroparenchymal fibroelastosis in a previously healthy woman J. Cravo, A. Catarino, L. Carvalho, T. Alfaro Pneumologia A, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal Pleuroparenchymal fibroelastosis (PPFE) is a rare disease characterized by a fibrosing process affecting the pleura and the subpleural lung parenchyma, with a predilection for the upper lobes. The etiology of PPFE is unclear at this juncture, about half of cases are felt to be idiopathic. It usually shows a progressive clinical course and has a poor prognosis. There is currently no specific therapy, but in progressive cases, lung transplantation may be required. Authors will present a clinical case. A 74 year-old nonsmoking lady was referred to the respiratory outpatient clinic for persistent chest X-ray infiltrates. Five months earlier, she was admitted to a medical ward for community-acquired pneumonia, with a good clinical response to antibiotics, but non-resolving imaging findings. She had no relevant past medical history or occupational exposures, but lived in a farm, and was in contact with a dog and some sheep. At observation she denied any respiratory complaints and had normal breath sounds and a room air oxygen saturation of 98%. Her chest x-ray showed worsening bilateral apical opacities. Lung function tests revealed a restrictive limitation with low diffusion capacity (vital capacity of 60% predicted and diffusion capacity of 59% predicted). A Chest CT was ordered, and showed fibrotic residual infiltrates in the apex of both lungs, with traction bronchiectasis and a possibly malignant 2.5 cm nodule with a radiologist’s recommendation for biopsy, which showed non-recent fibrosis and lymphocytes in the bronchiolar walls. Follow-up discussion in a multidisciplinary meeting led to suspicion of PPFE and a review of the biopsy, including staining for elastin using PAS and silver. This led to the identification of a pattern of fibrosis with an elastin rich matrix, compatible with PPFE. The patient remained asymptomatic and declined steroid treatment. Referral for lung transplantation was not considered due to her advanced age. She is currently under surveillance and under consideration for pirfenidone treatment.
DOI:
http://dx.doi.org/10.1016/j.chest.2017.04.144
Copyright ª 2017 American College of Chest Physicians and Swiss Respiratory Society SGP. Published by Elsevier Inc and Karger. All rights reserved.
journal.publications.chestnet.org
137A
POSTER WALKS
Although PPFE is a rare disease, advances in its recognition will probably lead to more frequent diagnoses, and improved knowledge. In our case, the discussion of this complex patient in a multi-disciplinary team meeting was instrumental for the correct diagnosis. This report illustrates how an elevated suspicion index in fundamental for the diagnosis of PPFE and a multidisciplinary discussion may facilitate this.