Hereditary hemorrhagic telangiectasia with bilateral pulmonary vascular malformations: A case report

Author:

Loncarevic Olivera1,Rusovic Sinisa2,Stojisavljevic Marko1,Vukovic Jelena1,Plavec Goran3,Acimovic Slobodan1,Cvetkovic Gordana1,Petrovic Marina4ORCID

Affiliation:

1. Military Medical Academy, Clinic for lung diseases, Belgrade

2. Military Medical Academy, Institute for radiology, Belgrade

3. Military Medical Academy, Clinic for lung diseases, Belgrade + University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade

4. Clinical Centre Kragujevac, Department of Pulmology, Kragujevac + Faculty of Medical Sciences, Kragujevac

Abstract

Introduction. Hereditary hemorrhagic telangiectasia (HHT) also known as Osler-Weber-Rendu syndrome is an autosomal dominant disease that occurs due to vascular dysplasia associated with the disorder in the signaling pathway of transforming growth factor ? (TGF-?). The clinical consequence is a disorder of blood vessels in multiple organ systems with the existence of telangiectasia which causes dilation of capillaries and veins, are present from birth and are localized on the skin and mucosa of the mouth, respiratory, gastrointestinal and urinary tract. They can make a rupture with consequent serious bleeding that can end up with fatal outcome. Since there is a disruption of blood vessels of more than one organic system, the diagnosis is very complex and requires a multidisciplinary approach. Case report. We reported a 40-year-old female patient with a long-time evolution of problems, who was diagnosed and treated at the Clinic for Lung Diseases of the Military Medical Academy in Belgrade, Serbia, because of bilaterally pulmonary arteriovenous malformations associated with HHT. Embolization was performed in two acts, followed with normalization of clinical, radiological and functional findings with the cessation of hemoptysis, effort intolerance with a significant improvement of the quality of life. Conclusion. HHT is a rare dominant inherited multisystem disease that requires multidisciplinary approach to diagnosis and treatment. Embolization is the method of choice in the treatment of arteriovenous malformations with minor adverse effects and very satisfying therapeutic effect.

Publisher

National Library of Serbia

Subject

Pharmacology (medical),General Medicine

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