Idiopathic Pulmonary Veno-Occlusive Disease

Mai Iwaki; Kazuyoshi Imaizumi; Toyoharu Yokoi; Masashi Kondo; Katsuhiro Kawaguchi; Yoshinori Hasegawa

doi:10.2169/internalmedicine.48.1935

CASE REPORTS

Idiopathic Pulmonary Veno-Occlusive Disease

Mai Iwaki, Kazuyoshi Imaizumi, Toyoharu Yokoi, Masashi Kondo, Katsuhiro Kawaguchi, Yoshinori Hasegawa

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Keywords: pulmonary veno-occlusive disease, diffuse centrilobular ground-glass opacities, pulmonary capillary hemangiomatosis, pulmonary hypertension

JOURNAL OPEN ACCESS

2009 Volume 48 Issue 15 Pages 1289-1292

DOI https://doi.org/10.2169/internalmedicine.48.1935

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Abstract

We report a case of idiopathic pulmonary veno-occlusive disease (PVOD). The patient experienced progressively worsening dyspnea. Heart catheterization revealed severe pulmonary hypertension. High-resolution computed tomography (HRCT) showed diffuse, poorly identified centrilobular ground-glass opacities. Surgical lung biopsy led to the diagnosis of PVOD. A microscopic examination revealed occlusions of pulmonary veins and venules over a wide area with prominent loop-like capillary dilatations. These pathological findings may be correlated with the radiological characteristics of HRCT in this case.

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