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August 29 2018

Research News: HHT-WG’s Outcome Measures Published in the OJRD!


We are thrilled to announce the publication of European Reference Network for Rare Vascular Diseases (VASCERN) Outcome Measures For the Hereditary Haemorrhagic Telangiectasia (HHT) in the Orphanet Journal of Rare Diseases (OJRD).

This Clinical Position Statement is based on the 5 Outcome Measures selected by members of the VASCERN HHT-WG.

“Hereditary haemorrhagic telangiectasia (HHT) is a multisystemic vascular dysplasia that leads to nosebleeds, anaemia due to blood loss, and arteriovenous malformations (AVMs) in organs such as the lungs, liver and brain. HHT is estimated to affect 85,000 European citizens, but most health care providers have limited prior HHT exposure or training.

Outcome Measures were developed and implemented by the HHT Working Group of the European Reference Network for Rare Vascular Diseases (VASCERN), in order to maximise the number of patients receiving good care. The measures specifically target areas where optimal management reduces morbidity and mortality in HHT patients, and were designed to be robust to emerging new evidence. Thresholds are the percentage of patients in particular settings who have been recommended screening, or provided with written advice. The 5 Outcome Measures cover (1) pulmonary AVM screening; (2) written nosebleed advice, (3) assessment of iron deficiency; (4) antibiotic prophylaxis prior to dental and surgical procedures for patients with pulmonary AVMs, and (5) written advice on pregnancy. They are not a blueprint for detailed HHT management, but are suitable for all clinicians to be aware of and implement.

In summary, these 5 Outcome Measures provide metrics to identify healthcare providers of good care, and encourage care improvement by all healthcare providers.”

To read the full article, click here

The Outcome measures were developed by the HHT-WG between March and June 2016. The discussion text was developed during monthly telecons, a face-to-face meeting and by email correspondence between October 2017–February 2018. The production of these outcome measures are a part of our Action Plan through the work package on outcome measures and clinical recommendations/expert consensus statement.

We congratulate the HHT-WG on this achievement and hope that this publication will lead to the improvement of care for all HHT patients.


European Reference Network For Rare Vascular Diseases (VASCERN) Outcome Measures For Hereditary Haemorrhagic Telangiectasia (HHT). Shovlin CL, Buscarini E, Kjeldsen AD, Mager HJ, Sabba C, Droege F, Geisthoff U, Ugolini S, Dupuis-Girod S. Orphanet J Rare Dis. 2018 Aug 15;13(1):136. doi: 10.1186/s13023-018-0850-2. PMID: 30111344 Free Article

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