The authors related a right heart failure related to a primitive pulmonary arterial hypertension without past medical.
This diagnosis has been noticed on the cardiac catheterism as well as the cardiac echography (no shunt). Two years later systemic anomalies such as polyarthritis and salivary glands disease were noticed. The Biopsy revelated a primitive Gougerot-Sjorgen pseudolymphomas syndrom.
Three years under conventional treatment were a failure as the evolution of the disease resulted to death.