{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,27]],"date-time":"2026-03-27T11:08:03Z","timestamp":1774609683403,"version":"3.50.1"},"reference-count":20,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2026,1,5]],"date-time":"2026-01-05T00:00:00Z","timestamp":1767571200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,1,5]],"date-time":"2026-01-05T00:00:00Z","timestamp":1767571200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pathology International"],"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Loeys\u2013Dietz syndrome (LDS) is a rare autosomal dominant disorder whose clinical phenotype overlaps with that of Marfan syndrome (MFS) and vascular Ehlers\u2013Danlos syndrome (vEDS), including aortic and\/or arterial aneurysms, skeletal abnormalities, and spontaneous pneumothorax. Although the pathological features of MFS\u2010 and vEDS\u2010associated pulmonary lesions have been described, the pulmonary pathology of LDS remains virtually unknown. Herein, we report the detailed histopathological features of LDS\u2010associated pulmonary vascular abnormalities and pneumothorax. A 32\u2010year\u2010old Japanese woman underwent pulmonary surgery for spontaneous pneumothorax. She had undergone aortic valve surgery 7 years earlier, and genetic testing identified a heterozygous germline missense variant in\n                    <jats:italic>TGFBR2<\/jats:italic>\n                    (c.1150\u2009A\u2009&gt;\u2009C, p.Asn384His). Histologically, the resected lung showed a ruptured bulla\/bleb. Localized distal acinar emphysema was present in continuity with the bullous lesion. In the remaining parenchyma, the alveolar septa exhibited a distinctive proliferation of irregularly dilated and tortuous capillaries, reminiscent of pulmonary capillary hemangiomatosis. Hemosiderin\u2010laden macrophages were conspicuous in some air spaces. In addition, several pulmonary muscular arteries and veins showed irregular dilatation and tortuosity, with elastic fibers that were fragmented and disorganized. Aberrant TGF\u2010\u03b2 signaling, together with deranged matrix formation, may underlie both the dilated, tortuous vasculature and the emphysematous\/bullous changes leading to pneumothorax in the LDS lung.\n                  <\/jats:p>","DOI":"10.1111\/pin.70078","type":"journal-article","created":{"date-parts":[[2026,1,6]],"date-time":"2026-01-06T07:43:17Z","timestamp":1767685397000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Pulmonary Vascular Abnormalities and Spontaneous Pneumothorax in Loeys\u2010Dietz Syndrome"],"prefix":"10.1111","volume":"76","author":[{"given":"Wataru","family":"Arai","sequence":"first","affiliation":[{"name":"Department of Respiratory Surgery Sapporo City General Hospital Sapporo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6220-8179","authenticated-orcid":false,"given":"Mitsuko","family":"Furuya","sequence":"additional","affiliation":[{"name":"Department of Pathology Sapporo City General Hospital Sapporo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1241-8610","authenticated-orcid":false,"given":"Yukio","family":"Nakatani","sequence":"additional","affiliation":[{"name":"Department of Pathology Yokosuka Kyosai Hospital Yokosuka Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takako","family":"Yamaguchi","sequence":"additional","affiliation":[{"name":"Department of Pathology Sapporo City General Hospital Sapporo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Miho","family":"Aoyagi","sequence":"additional","affiliation":[{"name":"Department of Respiratory Surgery Sapporo City General Hospital Sapporo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4681-2808","authenticated-orcid":false,"given":"Tomomi","family":"Yamaguchi","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics Shinshu University School of Medicine Matsumoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8344-7507","authenticated-orcid":false,"given":"Tomoki","family":"Kosho","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics Shinshu University School of Medicine Matsumoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6558-4818","authenticated-orcid":false,"given":"Shujiro","family":"Hayashi","sequence":"additional","affiliation":[{"name":"Department of Dermatology Dokkyo Medical University Mibu Tochigi 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