An autopsy case of pulmonary hypertension due to collagen disease with conspicuous stenotic lesions of small pulmonary veins

Abstract

An 81-year-old woman with a history of Sjogrenʼs syndrome and SLE was referred to our hospital because of dyspnea.　Echocardiography showed that the left ventricular function was preserved, but the right heart was enlarged and left ventricle was compressed, and the estimated systolic pulmonary artery pressure was elevated at 77 mmHg.　Blood tests showed inflammatory findings and elevated BNP, and chest CT showed frosted shadows, pleural effusion, and pericardial effusion.　She was admitted for exacerbation of pulmonary hypertension and complications of pneumonia, and was started on diuretics, dobutamine, and antibiotics.　On the second day, her blood pressure and oxygenation decreased and he died the same day.　On autopsy, no thrombus was found in the pulmonary artery, and intimal thickening of the small pulmonary artery was mild, but stenosis was conspicuous in the small pulmonary vein, and intimal thickening and vitrification were observed, as well as extensive pulmonary edema.　Connective tissue disease （CTD） is associated with a high risk of developing pulmonary hypertension, but not only pulmonary arterial hypertension （PAH） but also pulmonary veno-occlusive disease （PVOD）, pulmonary hypertension due to left heart disease, pulmonary hypertension due to lung diseases, chronic thromboembolic pulmonary hypertension （CTEPH）, and a mixture of these conditions can be seen in CTD.　Clinically, this case was considered PAH due to connective tissue disease, but autopsy revealed a PVOD-like post-capillary pulmonary hypertensive state.　Therefore, this was considered a rare case in which pulmonary edema was also observed despite preserved left heart function.