title: An autopsy case of refractory pulmonary hypertension with sarcoidosis.
abstract: A 63-year-old man with sarcoidosis-associated pulmonary hypertension (SAPH) died suddenly of decompensated right heart failure. At autopsy, microscopy showed subpleural and interlobular fibrosis in both upper lobes, with marked broncho-bronchiolectasis, as well as bronchovascular bundle fibrosis and fibrotic organization in alveolar lumens, which are consistent with pulmonary sarcoidosis. Intimal fibrosis and medial hypertrophy were noted in the proximal elastic to distal muscular pulmonary arteries (Heath-Edwards, grades II-III) within intensive fibrotic lesions. Additionally, diffuse alveolar capillary multiplication (DACM) was present in macroscopically normal lung parenchymal lesions, associated with wall muscularization. In this case, muscularization of capillaries may have been induced by hypoxemia and hypoxic pulmonary vasoconstriction, resulting in pulmonary hypertension.
authors.affiliation: Department of Respiratory Medicine, Toho University Omori Medical Center, 6-11-1, Omori-nishi, Ota-ku, Tokyo 143-8541, Japan. Electronic address: otahiro@hotmail.com.
authors.forename: Hiroki
authors.initials: H
authors.lastname: Ota
authors.affiliation: Department of Respiratory Medicine, Toho University Omori Medical Center, 6-11-1, Omori-nishi, Ota-ku, Tokyo 143-8541, Japan. Electronic address: ks142129_ikusou@ybb.ne.jp.
authors.forename: Keishi
authors.initials: K
authors.lastname: Sugino
authors.affiliation: Department of Pathology, Labor Health and Welfare Organization Kanto Rosai Hospital, Kawasaki, Japan. Electronic address: uekusatoshimasa@kantoh.rofuku.go.jp.
authors.forename: Toshimasa
authors.initials: T
authors.lastname: Uekusa
authors.affiliation: Department of Pathology, Japanese Red Cross Medical Center, Tokyo, Japan. Electronic address: byori@med.jrc.or.jp.
authors.forename: Tamiko
authors.initials: T
authors.lastname: Takemura
authors.affiliation: Department of Respiratory Medicine, Toho University Omori Medical Center, 6-11-1, Omori-nishi, Ota-ku, Tokyo 143-8541, Japan. Electronic address: sahomma@med.toho-u.ac.jp.
authors.forename: Sakae
authors.initials: S
authors.lastname: Homma
mesh_terms: D001344:Autopsy; D016757:Death, Sudden, Cardiac; D017809:Fatal Outcome; D006333:Heart Failure / Q000209:etiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000473:pathology*; D008168:Lung / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D017565:Sarcoidosis, Pulmonary / Q000150:complications* / Q000473:pathology*
keywords: Alveolar capillary multiplication; Autopsy; Lung; Pulmonary hypertension; Sarcoidosis