The exact cause of Ayerza's disease is not fully understood, but it is linked to primary sclerosis of the pulmonary artery which leads to pulmonary hypertension, and is now often referred to as idiopathic pulmonary hypertension.
Originally, Ayerza's disease was associated with chronic pulmonary processes. However, research shifted the understanding toward the pulmonary artery itself as a primary site of the disease, independent of other chronic lung conditions. This led to the recognition of what was then called "primary" pulmonary hypertension, and is currently known as "idiopathic" pulmonary hypertension. Therefore, while the initiating factors remain elusive, the pathological process involves thickening and hardening (sclerosis) of the pulmonary arteries.
In summary, the precise etiology of Ayerza's disease, understood now as a form of idiopathic pulmonary hypertension, remains undetermined, with research pointing towards primary sclerosis of the pulmonary artery playing a critical role.
