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Pulmonary capillary hemangiomatosis-predominant vasculopathy in a patient with rheumatoid arthritis-associated interstitial lung disease : An autopsy report

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Title: Pulmonary capillary hemangiomatosis-predominant vasculopathy in a patient with rheumatoid arthritis-associated interstitial lung disease : An autopsy report
Authors: Nakamura, Junichi Browse this author
Tsujino, Ichizo Browse this author →KAKEN DB
Yamamoto, Gaku Browse this author
Nakaya, Toshitaka Browse this author
Takahashi, Kei Browse this author
Kimura, Hirokazu Browse this author →KAKEN DB
Sato, Takahiro Browse this author
Watanabe, Taku Browse this author →KAKEN DB
Nakagawa, Shimpei Browse this author
Otsuka, Noriyuki Browse this author
Ohira, Hiroshi Browse this author →KAKEN DB
Konno, Satoshi Browse this author →KAKEN DB
Keywords: Computed tomography diffusion capacity of the lung for carbon monoxide
Pulmonary capillary hemangiomatosis
Pulmonary hypertension
Pulmonary veno-occlusive disease
Issue Date: 2020
Publisher: Elsevier
Journal Title: Respiratory Medicine Case Reports
Volume: 31
Start Page: 101215
Publisher DOI: 10.1016/j.rmcr.2020.101215
Abstract: 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.
Rights: https://creativecommons.org/licenses/by-nc-nd/4.0/
Type: article
URI: http://hdl.handle.net/2115/80440
Appears in Collections:医学院・医学研究院 (Graduate School of Medicine / Faculty of Medicine) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)

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