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

Clin Rheumatol. 2007 Sep;26(9):1537-9. doi: 10.1007/s10067-006-0426-8. Epub 2006 Sep 19.

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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Albinism, Oculocutaneous / complications*
  • Aneurysm / etiology*
  • Antibodies, Monoclonal / therapeutic use
  • Behcet Syndrome / complications*
  • Behcet Syndrome / diagnosis
  • Behcet Syndrome / drug therapy
  • Black or African American
  • Female
  • Heart Diseases / etiology*
  • Humans
  • Infliximab
  • Pulmonary Artery / physiopathology
  • Thrombosis / etiology*
  • Tumor Necrosis Factor-alpha / antagonists & inhibitors
  • Vasculitis / drug therapy
  • Vasculitis / etiology

Substances

  • Antibodies, Monoclonal
  • Tumor Necrosis Factor-alpha
  • Infliximab