The purpose of this paper is to call attention to a group of cases showing the clinical signs of Ayerza's disease (sclerosis of the pulmonary artery) due to another pathologic condition and to offer some points which will aid in differentiating the various pathologic processes which may cause this syndrome.
Parkes Weber1 classifies the pathologic conditions associated with "secondary" (symptomatic) forms of polycythemia rubra, and mentions the following classes of cases:
Polycythemia hypertonica, "Geisböcks disease."
Splenomegalic polycythemia rubra associated with tuberculosis of the spleen.
Secondary polycythemia rubra with splenomegaly connected with a condition of visceral blood stasis of chronic thrombotic origin in the portal and splenic veins.
"Cardiacos negros," "Ayerza's diseases," due to pulmonary sclerosis, pulmonary fibrosis, asthma, pulmonary emphysema, chronic bronchitis and pleural adhesions.
Secondary polycythemia rubra connected with congenital or acquired heart disease.
It will be seen that under Ayerza's disease, Parkes Weber groups all cases
MORSE PF. SYMPTOMATIC POLYCYTHEMIA WITH CYANOSIS AND DYSPNEA. Arch Intern Med (Chic). 1924;33(4):459-472. doi:10.1001/archinte.1924.00110280057006