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Ayerza's syndrome or disease

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Condition characterised by dyspnea, slowly developing asthma, bronchitis, and chronic cyanosis in association with polycythemia. Polycythemia usually results from chronic pulmonary insufficiency and sclerosis of the pulmonary vascular bed. Cardiac involvement may be present, usually in the form of dilatation and hypertrophy of the right heart. Pulmonary arterial hyperplasia and stenosis, emphysema, and fibrosis may be the contributing factors. Splenomegaly, clubbing of the fingers, congestion of the liver, and heart failure are associated.

In 1901 Abel Ayerza lectured on the syndrome of chronic cyanosis, dyspnoea, erythraemia, and sclerosis of the pulmonary artery. He did not publish this work, but an important discussion on the nomenclature was given by Luis Ayerza in 1925. The term Ayerza's disease was introduced by F. C. Arrilaga. Baron Jean-Nicolas Corvisart des Marest (1755-1821) mentioned the condition in 1806.


  • J. N. Corvisart des Marest:
    Essai sur les maladies et les lésions organiques du coeur et des gros vaisseeaux. Paris 1806.
  • A. Ayerza:
    Cardiacos negros. Buoenos Aires, Thesis No. 2536, 1912.
    A classical description of Ayerza's disease (cor pulmomale), to which Arrilaga gave the name.
  • Maladie d’Ayerza, sclérose secondaire de l’artère pulmonaire (cardiaques noirs).
    Sem méd, Buenos Aires, 1925, 32: 43-44.
  • F. C. Arrilaga:
    Esclerosis secundaria de la arteria pulmonaria y su quadro clinico (cardiacos negros). Buenos Aires, 1912.
  • Luis Ayerza:
    Consideraciones sobre la denominación de "Enfermedad de Ayerza".
    Semana méd., 1925, 32, pt. 2, 386-388.
    Luis Ayerza also published a paper on the topic in a previous paper in the same journal, 1925, 32, pt. 1: 43.

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