Definition: sclerosis of the pulmonary arteries in chronic cor pulmonale; associated with severe cyanosis, it is a condition resembling polycythemia vera but resulting from primary pulmonary arteriosclerosis or primary pulmonary hypertension and characterized by plexiform lesions of arterioles.
Synonym(s): Ayerza disease, cardiopathia nigra, plexogenic pulmonary arteriopathy
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