Biomaterial Database

Graves' orbitopathy as a rare disease in Europe: a European Group on Graves' Orbitopathy (EUGOGO) position statement. 2017

P Perros, and L Hegedüs, and L Bartalena, and C Marcocci, and G J Kahaly, and L Baldeschi, and M Salvi, and J H Lazarus, and A Eckstein, and S Pitz, and K Boboridis, and P Anagnostis, and G Ayvaz, and A Boschi, and T H Brix, and N Currò, and O Konuk, and M Marinò, and A L Mitchell, and B Stankovic, and F B Törüner, and G von Arx, and M Zarković, and W M Wiersinga

Institute of Genetic Medicine, Newcastle University, Central Parkway, Newcastle upon Tyne, Tyne, NE1 3BZ, UK. petros.perros@ncl.ac.uk.

Graves' orbitopathy (GO) is an autoimmune condition, which is associated with poor clinical outcomes including impaired quality of life and socio-economic status. Current evidence suggests that the incidence of GO in Europe may be declining, however data on the prevalence of this disease are sparse. Several clinical variants of GO exist, including euthyroid GO, recently listed as a rare disease in Europe (ORPHA466682). The objective was to estimate the prevalence of GO and its clinical variants in Europe, based on available literature, and to consider whether they may potentially qualify as rare. Recent published data on the incidence of GO and Graves' hyperthyroidism in Europe were used to estimate the prevalence of GO. The position statement was developed by a series of reviews of drafts and electronic discussions by members of the European Group on Graves' Orbitopathy. The prevalence of GO in Europe is about 10/10,000 persons. The prevalence of other clinical variants is also low: hypothyroid GO 0.02-1.10/10,000; GO associated with dermopathy 0.15/10,000; GO associated with acropachy 0.03/10,000; asymmetrical GO 1.00-5.00/10,000; unilateral GO 0.50-1.50/10,000. GO has a prevalence that is clearly above the threshold for rarity in Europe. However, each of its clinical variants have a low prevalence and could potentially qualify for being considered as a rare condition, providing that future research establishes that they have a distinct pathophysiology. EUGOGO considers this area of academic activity a priority.

UI	MeSH Term	Description	Entries
D011788	Quality of Life	A generic concept reflecting concern with the modification and enhancement of life attributes, e.g., physical, political, moral, social environment as well as health and disease.	HRQOL,Health-Related Quality Of Life,Life Quality,Health Related Quality Of Life
D005060	Europe	The continent north of AFRICA, west of ASIA and east of the ATLANTIC OCEAN.	Northern Europe,Southern Europe,Western Europe
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D017410	Practice Guidelines as Topic	Works about directions or principles presenting current or future rules of policy for assisting health care practitioners in patient care decisions regarding diagnosis, therapy, or related clinical circumstances. The guidelines may be developed by government agencies at any level, institutions, professional societies, governing boards, or by the convening of expert panels. The guidelines form a basis for the evaluation of all aspects of health care and delivery.	Clinical Guidelines as Topic,Best Practices,Best Practice
D049970	Graves Ophthalmopathy	An autoimmune disorder of the EYE, occurring in patients with Graves disease. Subtypes include congestive (inflammation of the orbital connective tissue), myopathic (swelling and dysfunction of the extraocular muscles), and mixed congestive-myopathic ophthalmopathy.	Graves Eye Disease,Ophthalmopathy, Infiltrative,Ophthalmopathy, Thyroid-Associated,Thyroid Eye Disease,Thyroid-Associated Ophthalmopathy,Congestive Ophthalmopathy,Dysthyroid Ophthalmopathy,Edematous Ophthalmopathy,Graves Orbitopathy,Myopathic Ophthalmopathy,Ophthalmopathies, Thyroid-Associated,Thyroid-Associated Ophthalmopathies,Congestive Ophthalmopathies,Disease, Graves Eye,Disease, Thyroid Eye,Dysthyroid Ophthalmopathies,Edematous Ophthalmopathies,Eye Disease, Graves,Eye Disease, Thyroid,Infiltrative Ophthalmopathies,Infiltrative Ophthalmopathy,Myopathic Ophthalmopathies,Ophthalmopathy, Congestive,Ophthalmopathy, Dysthyroid,Ophthalmopathy, Edematous,Ophthalmopathy, Graves,Ophthalmopathy, Myopathic,Ophthalmopathy, Thyroid Associated,Orbitopathy, Graves,Thyroid Associated Ophthalmopathies,Thyroid Associated Ophthalmopathy,Thyroid Eye Diseases
D035583	Rare Diseases	A large group of diseases which are characterized by a low prevalence in the population. They frequently are associated with problems in diagnosis and treatment.	Orphan Diseases,Disease, Orphan,Disease, Rare,Orphan Disease,Rare Disease