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Pulmonary Capillary Hemangiomatosis

  • Edward D. Chan
  • Kathryn Chmura
  • Andrew Sullivan
Chapter
Part of the Respiratory Medicine book series (RM)

Abstract

Pulmonary capillary hemangiomatosis (PCH) is a rare lung disorder characterized by proliferation of thin-walled capillary channels that infiltrate the walls of alveolar septae, pulmonary blood vessels, airways, and pleura. In its most dramatic presentation, it manifests as dyspnea, hemoptysis, pulmonary hypertension, and right heart failure although PCH-like lesions may be found incidentally in lung tissues. In patients who present with pulmonary hypertension of unclear etiology, PCH should be considered in the differential diagnosis particularly in the presence of centrilobular pattern of ground-glass opacities, enhance septal lines, pleural effusion, and/or adenopathy on imaging. The etiology of PCH is unknown although histologic abnormalities indicate that the pathogenesis involves dysregulation of angio- or vasculogenesis that is distinct from idiopathic pulmonary hypertension. There is no effective treatment for PCH. Anecdotal reports indicate that alpha-interferon or doxycycline may have some efficacy. Given the very low incidence of PCH, it is plausible that effective anti-angiogenic agents developed for other diseases (i.e., cancer) may be used with some reasonable level of efficacy before the pathogenesis of PCH is more fully elucidated. Lung transplantation should be considered for severe cases.

Keywords

alveolar hemorrhage angiogenesis cor pulmonale pulmonary hypertension 

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Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Edward D. Chan
    • 1
  • Kathryn Chmura
    • 2
  • Andrew Sullivan
    • 3
  1. 1.Department of Internal MedicineNational Jewish Medical and Research CenterDenverUSA
  2. 2.Department of MedicineUniversity of Colorado School of MedicineDenverUSA
  3. 3.Department of Internal MedicineUniversity of Colorado School of MedicineDenverUSA

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