Pulmonary aneurysms and intracardiac thrombi due to Behçet's disease in an African-American adolescent with oculocutaneous albinism.

Academic Article

Abstract

  • Behçet's disease (BD) is a systemic vasculitis that may involve a variety of organs. We describe a girl with oculocutaneous albinism (OCA) who, on initial evaluation for popliteal artery occlusion, was noted to have multiple cardiac thrombi. She later developed hemoptysis, which was the result of bilateral pulmonary artery aneurysms. Her clinical picture was consistent with BD. She was initially treated with prednisone and cyclophosphamide, followed by maintenance therapy with infliximab. This case describes a unique patient with OCA who developed BD with large pulmonary aneurysms and has remained stable for over 1 year while receiving infliximab.
  • Published In

    Keywords

  • Adolescent, African Americans, Albinism, Oculocutaneous, Aneurysm, Antibodies, Monoclonal, Behcet Syndrome, Female, Heart Diseases, Humans, Infliximab, Pulmonary Artery, Thrombosis, Tumor Necrosis Factor-alpha, Vasculitis
  • Digital Object Identifier (doi)

    Author List

  • Endo LM; Rowe SM; Romp RL; Buckmaster MA; Atkinson TP
  • Start Page

  • 1537
  • End Page

  • 1539
  • Volume

  • 26
  • Issue

  • 9