RAUL E. ESPINOSA, M. D. , WILLIAM D. EDWARDS, M. D. , EDWARD C

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Idiopathic Pulmonary Hilar Fibrosis: An Unusual Cause of Pulmonary Hypertension RAUL E. ESPINOSA, M.D., WILLIAM D. EDWARDS, M.D., EDWARD C. ROSENOW, M.D., HARTZELL V. SCHAFF, M.D. Mayo Clinic Proceedings Volume 68, Issue 8, Pages 778-782 (August 1993) DOI: 10.1016/S0025-6196(12)60636-3 Copyright © 1993 Mayo Foundation for Medical Education and Research Terms and Conditions

Fig. 1 Radiographic studies in 37-year-old man with pulmonary hypertension. A, Frontal chest roentgenogram, showing prominent pulmonary artery. B, Pulmonary angiogram, showing abrupt cutoff of right intermediate pulmonary artery (arrow), no flow to right middle and lower lobes, and diminished perfusion of left lower lobe. Mayo Clinic Proceedings 1993 68, 778-782DOI: (10.1016/S0025-6196(12)60636-3) Copyright © 1993 Mayo Foundation for Medical Education and Research Terms and Conditions

Fig. 2 Lung biopsy specimens. A, B, and C, Pulmonary arterial lesions, with medial hypertrophy (A), organized in situ thrombus (B), and fibrotic occlusion (C). D, Medial hypertrophy and so-called arterialization of pulmonary vein. (Elastic tissue-van Gieson; A and D, × 90; B and C, × 180.) Mayo Clinic Proceedings 1993 68, 778-782DOI: (10.1016/S0025-6196(12)60636-3) Copyright © 1993 Mayo Foundation for Medical Education and Research Terms and Conditions

Fig. 3 Autopsy findings. A, Pulmonary fibrosis involving primarily the hilum. Lower lobe is anemic. B, Hourglass constriction and occlusion of hilar pulmonary artery and vein. C, Cross section of specimen in B, showing densely fibrotic process (asterisk) and occluded artery (solid arrow) and vein (open arrow). D and E, Sections of hilar fibrosis, showing inflammatory infiltrate (D) and dense collagen bundles (E). (D and E, Hematoxylin-eosin; original magnification, × 90.) Mayo Clinic Proceedings 1993 68, 778-782DOI: (10.1016/S0025-6196(12)60636-3) Copyright © 1993 Mayo Foundation for Medical Education and Research Terms and Conditions