plexogenic pulmonary arteriopathy

A·yer·za syn·drome

(ah-yār'shah), 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

plexogenic pulmonary arteriopathy

A vascular change seen in advanced pulmonary hypertension which is characterised by medial hypertrophy of muscular pulmonary arteries, intimal fibrosis, replacement of normal endothelium and fibrinoid necrosis.
 
Aetiology
Idiopathic; secondary to left-sided heart disease, lung disease, thrombosis, vasoconstriction of arteries (e.g., primary pulmonary hypertension), extravascular compression (e.g., due to disease of the chest wall), anorexiants (e.g., fenfluramine, possibly also phentermine).

plexogenic pulmonary arteriopathy

Pulmonary disease A vasculopathy seen in pulmonary HTN, characterized by medial hypertrophy of muscular pulmonary arteries, fibrinoid necrosis Etiology Anorexiants–eg, fenfluramine; possibly phentermine. See Fen-phen.

Ayerza,

Abel, Argentinian physician, 1861-1918. Ayerza-Arrillaga disease - Synonym(s): Ayerza syndromeAyerza disease - Synonym(s): Ayerza syndromeAyerza syndrome - sclerosis of the pulmonary arteries in chronic cor pulmonale. Synonym(s): Ayerza-Arrillaga disease; Ayerza disease; cardiopathia nigra; plexogenic pulmonary arteriopathy