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From the Archives of the AFIP

Pulmonary Veno-occlusive Disease and Pulmonary Capillary Hemangiomatosis¹

¹ From the Departments of Radiologic Pathology (A.A.F., J.R.G.) and Pulmonary and Mediastinal Pathology (T.J.F.), Armed Forces Institute of Pathology, 14th St and Alaska Ave NW, Washington, DC 20306; Department of Diagnostic Radiology, University of Maryland School of Medicine, Baltimore, Md (A.A.F., J.R.G.); Section of Thoracic Imaging, Division of Radiology, Cleveland Clinic Foundation, Cleveland, Ohio (T.-L.H.M.); and Department of Pathology, Akdeniz University School of Medicine, Antalya, Turkey (I.H.O.). Received December 6, 2006; revision requested December 18 and received January 18, 2007; accepted January 25. All authors have no financial relationships to disclose. Address correspondence to A.A.F. (e-mail: frazier{at}afip.osd.mil ).

Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are two unusual idiopathic disorders that almost uniformly manifest to the clinician as pulmonary arterial hypertension (PAH). Impressive clinical signs and symptoms often obscure the true underlying capillary or postcapillary disorder, thus severely compromising timely and appropriately directed therapy. The hemodynamics of PVOD and PCH are the consequence of a widespread vascular obstructive process that originates in either the alveolar capillary bed (in cases of PCH) or the pulmonary venules and small veins (in PVOD). Since the earliest descriptions of PVOD and PCH, there has been a debate as to whether these are two distinct diseases or varied expressions of a single disorder. The cause of PVOD or PCH has not yet been identified, although there are several reported associations. Without curative lung or heart-lung transplantation, patients with these conditions face inexorable clinical deterioration and death within months to a few short years of initial presentation. Surgical lung biopsy is the definitive diagnostic test, but it is a risky undertaking in such critically ill patients. The imaging manifestations of PVOD and PCH often reflect the underlying hemodynamic derangements, and these findings may assist the clinician in discerning PAH from an underlying capillary or postcapillary process with findings of septal lines, characteristic ground-glass opacities, and occasionally pleural effusion.