What are the characteristics of epistaxis in Osler-Weber-Rendu disease (OWRD) (hereditary hemorrhagic telangiectasia [HHT])?

Updated: Oct 06, 2020
  • Author: Klaus-Dieter Lessnau, MD, FCCP; Chief Editor: Vincent Lopez Rowe, MD  more...
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Answer

Recurrent epistaxis is usually the presenting symptom in HHT. Recurrent epistaxis is present in 90% of patients with HHT and appears at a young age, manifesting in most patients by age 21 years. [54] Bleeding occurs spontaneously from telangiectases of the nasal mucosa.

Recurrent epistaxis results in fatigue and anemia. Iron supplementation and blood transfusion may be required. Bleeding symptoms are progressive with increasing age. The presence of pulmonary AVM does not predict a better or worse natural history for epistaxis. [55]


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