| A case of pulmonary capillary hemangiomatosis with pulmonary fibrosis associated with MMP-9 related pulmonary remodeling. | |
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MedLine Citation:
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PMID: 21501297 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension. It is characterized capillary proliferation within the alveolar septa. Here, we report a case of PCH with extensive pulmonary fibrosis. A 52-year-old man with a clinical diagnosis of non-specific interstitial pneumonia died of respiratory failure with severe pulmonary hypertension. Autopsy revealed pronounced right ventricle hypertrophy and pulmonary fibrosis. Consistent with clinical diagnosis, histological examination revealed diffuse pulmonary fibrosis, in addition, it also disclosed marked capillary proliferation within the alveolar septa as well as the fibrotic pulmonary stroma, suggesting the presence of PCH. Hemosiderin-laden macrophages had accumulated in the capillary proliferative area, and bronchiolar-type metaplasia was conspicuous in the fibrotic lesion. Proliferated capillaries were surrounded by fine collagen and α-smooth muscle actin-positive myofibroblasts. Immunohistochemistry revealed that type IV collagen around capillaries in the area of the PCH without inflammation disappeared in the area with inflammation. In addition, the PCH lesion contained significant numbers of macrophages expressing matrix metalloproteinase (MMP) 9 and type II pneumocytes positive for vascular endothelial growth factor. Although pulmonary fibrosis is a distinctive disease entity, different from PCH, MMP-9-driven destruction of the basement membrane may promote unusual pulmonary remodeling, which, in this case, resulted in extensive pulmonary fibrosis. |
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Authors:
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Naomi Sakashita; Yashiro Motooka; Masato Suganuma; Koji Ohnishi; Yukio Fujiwara; Takenobu Nakagawa; Hidenori Ichiyasu; Motohiro Takeya |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Pathology international Volume: 61 ISSN: 1440-1827 ISO Abbreviation: Pathol. Int. Publication Date: 2011 May |
Date Detail:
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Created Date: 2011-04-19 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9431380 Medline TA: Pathol Int Country: Australia |
Other Details:
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Languages: eng Pagination: 306-12 Citation Subset: IM |
Copyright Information:
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© 2011 The Authors. Pathology International © 2011 Japanese Society of Pathology and Blackwell Publishing Asia Pty Ltd. |
Affiliation:
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Departments of Cell Pathology and Respiratory Medicine, Graduate School of Medical Sciences, Kumamoto University, and Kumamoto University School of Medicine, Kumamoto, Department of Human Pathology, Institute of Health Biosciences, The University of Tokushima, Tokushima, Japan. |
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