| 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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