An autopsy case of refractory pulmonary hypertension with sarcoidosis

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.