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Pulmonary capillary hemangiomatosis-predominant vasculopathy in a patient with rheumatoid arthritis-associated interstitial lung disease: An autopsy report
Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension (PH) associated with poor prognosis. Clinically, it is characterized by severe hypoxemia, centrilobular ground-glass opacities on computed tomography, and pulmonary congestion triggered by pulmonary vasodilating ther...
Autores principales: | , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7527749/ https://www.ncbi.nlm.nih.gov/pubmed/33024689 http://dx.doi.org/10.1016/j.rmcr.2020.101215 |
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author | Nakamura, Junichi Tsujino, Ichizo Yamamoto, Gaku Nakaya, Toshitaka Takahashi, Kei Kimura, Hirokazu Sato, Takahiro Watanabe, Taku Nakagawa, Shimpei Otsuka, Noriyuki Ohira, Hiroshi Konno, Satoshi |
author_facet | Nakamura, Junichi Tsujino, Ichizo Yamamoto, Gaku Nakaya, Toshitaka Takahashi, Kei Kimura, Hirokazu Sato, Takahiro Watanabe, Taku Nakagawa, Shimpei Otsuka, Noriyuki Ohira, Hiroshi Konno, Satoshi |
author_sort | Nakamura, Junichi |
collection | PubMed |
description | Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension (PH) associated with poor prognosis. Clinically, it is characterized by severe hypoxemia, centrilobular ground-glass opacities on computed tomography, and pulmonary congestion triggered by pulmonary vasodilating therapy. In some cases, PCH has been reported to develop with other disorders including connective tissue disease; however, to date, no reports have described PCH in a patient with rheumatoid arthritis. We report a case of a 59-year-old male PCH patient with rheumatoid arthritis and associated pulmonary fibrosis. He was initially diagnosed with severe group 3 PH and received sildenafil, which generated a favorable hemodynamic response. However, 5 years later, his pulmonary hemodynamics deteriorated, and he died at the age of 67. An autopsy was performed, and thickening of alveolar septa and capillary proliferation, pathological features of PCH, were extensively observed in both lungs. We discuss when PCH developed, how sildenafil improved his hemodynamics, and how PCH could be clinically detected by noninvasive evaluations. |
format | Online Article Text |
id | pubmed-7527749 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-75277492020-10-05 Pulmonary capillary hemangiomatosis-predominant vasculopathy in a patient with rheumatoid arthritis-associated interstitial lung disease: An autopsy report Nakamura, Junichi Tsujino, Ichizo Yamamoto, Gaku Nakaya, Toshitaka Takahashi, Kei Kimura, Hirokazu Sato, Takahiro Watanabe, Taku Nakagawa, Shimpei Otsuka, Noriyuki Ohira, Hiroshi Konno, Satoshi Respir Med Case Rep Case Report Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension (PH) associated with poor prognosis. Clinically, it is characterized by severe hypoxemia, centrilobular ground-glass opacities on computed tomography, and pulmonary congestion triggered by pulmonary vasodilating therapy. In some cases, PCH has been reported to develop with other disorders including connective tissue disease; however, to date, no reports have described PCH in a patient with rheumatoid arthritis. We report a case of a 59-year-old male PCH patient with rheumatoid arthritis and associated pulmonary fibrosis. He was initially diagnosed with severe group 3 PH and received sildenafil, which generated a favorable hemodynamic response. However, 5 years later, his pulmonary hemodynamics deteriorated, and he died at the age of 67. An autopsy was performed, and thickening of alveolar septa and capillary proliferation, pathological features of PCH, were extensively observed in both lungs. We discuss when PCH developed, how sildenafil improved his hemodynamics, and how PCH could be clinically detected by noninvasive evaluations. Elsevier 2020-09-08 /pmc/articles/PMC7527749/ /pubmed/33024689 http://dx.doi.org/10.1016/j.rmcr.2020.101215 Text en © 2020 The Authors http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). |
spellingShingle | Case Report Nakamura, Junichi Tsujino, Ichizo Yamamoto, Gaku Nakaya, Toshitaka Takahashi, Kei Kimura, Hirokazu Sato, Takahiro Watanabe, Taku Nakagawa, Shimpei Otsuka, Noriyuki Ohira, Hiroshi Konno, Satoshi Pulmonary capillary hemangiomatosis-predominant vasculopathy in a patient with rheumatoid arthritis-associated interstitial lung disease: An autopsy report |
title | Pulmonary capillary hemangiomatosis-predominant vasculopathy in a patient with rheumatoid arthritis-associated interstitial lung disease: An autopsy report |
title_full | Pulmonary capillary hemangiomatosis-predominant vasculopathy in a patient with rheumatoid arthritis-associated interstitial lung disease: An autopsy report |
title_fullStr | Pulmonary capillary hemangiomatosis-predominant vasculopathy in a patient with rheumatoid arthritis-associated interstitial lung disease: An autopsy report |
title_full_unstemmed | Pulmonary capillary hemangiomatosis-predominant vasculopathy in a patient with rheumatoid arthritis-associated interstitial lung disease: An autopsy report |
title_short | Pulmonary capillary hemangiomatosis-predominant vasculopathy in a patient with rheumatoid arthritis-associated interstitial lung disease: An autopsy report |
title_sort | pulmonary capillary hemangiomatosis-predominant vasculopathy in a patient with rheumatoid arthritis-associated interstitial lung disease: an autopsy report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7527749/ https://www.ncbi.nlm.nih.gov/pubmed/33024689 http://dx.doi.org/10.1016/j.rmcr.2020.101215 |
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