PULMONARY VASCULITIS WITH HYPEREOSINOPHILIA AND EPISODIC PULMONARY-HYPERTENSION - REPORT OF 3 SIBLINGS

Three siblings with eosinophilia who developed pulmonary hypertension are reported. They consisted of a 3 year old boy (case 1), a 9 year ol d boy (case 2) and a 13 year old girl (case 3), all of whom died withi n an 18 month period of severe episodic attacks of pulmonary hypertens ion and the resultant low cardiac output. Marked peripheral eosinophil ia was found in cases 1 and 2, and mild eosinophilia in case 3. Open l ung biopsy of case 1 revealed pulmonary arteritis with massive eosinop hilic infiltration and intimal thickening of muscular arteries of 300- 1500 mu m in diameter. At autopsy, cases 2 and 3 showed almost similar findings, comprising widespread obliteration of the pulmonary arterie s by concentric intimal thickening, medial hypertrophy and recanalized thrombi of arterioles. Rarely, there were foci of granulomas in the t hickened intima surrounding birefringent foreign bodies. There were sm all areas of infarction in the lungs and heart due to arterial thrombi . Vascular lesions other than those in the lungs were mild and almost limited to the branches of the coronary arteries. Therefore, the prese nt cases appear to be a single disease of pulmonary hypertension secon dary to endothelial injury and the resultant intimal fibrosis probably evoked by toxic substances, although such agents were not confirmed.