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The authors report an unusual limited form of orbital GPA in a 35-year-old woman presenting with bilateral dacryoadenitis, evolving later to locally aggressive bilateral orbital pseudotumour leading to proptosis, extraocular myositis, diplopia and medial deviation of the nasal septum. She had never had systemic manifestations but her disease was persistently active and unresponsive to corticosteroids and immunosuppressors. The aim of this paper is to provide further evidence of aggressive and refractory limited forms of GPA.<\/jats:p>","DOI":"10.1136\/bcr-2015-214099","type":"journal-article","created":{"date-parts":[[2016,5,11]],"date-time":"2016-05-11T05:02:33Z","timestamp":1462942953000},"page":"bcr2015214099","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":15,"title":["Bilateral dacryoadenitis as initial presentation of a locally aggressive and unresponsive limited form of orbital granulomatosis with polyangiitis"],"prefix":"10.1136","volume":"2016","author":[{"given":"Rute","family":"Lopes Ca\u00e7ola","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, Hospital Pedro Hispano, Matosinhos, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra Alves","family":"Morais","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Hospital Pedro Hispano, Matosinhos, 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