title: Bilateral dacryoadenitis as initial presentation of a locally aggressive and unresponsive limited form of orbital granulomatosis with polyangiitis.
abstract: Limited orbital granulomatosis with polyangiitis (GPA) is uncommon and its diagnosis may be delayed, especially when isolated lacrimal involvement is the initial presentation, because clinical manifestations are non-specific and systemic diagnostic criteria are not applicable. Making an early diagnosis despite the absence of systemic progression is extremely important because in some cases the disease is locally destructive, with irreversible visual and functional loss, and it can be refractory to corticosteroids and conventional immunosuppressive drugs to induce remission. 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.
authors.affiliation: Department of Internal Medicine, Hospital Pedro Hispano, Matosinhos, Portugal.
authors.forename: Rute
authors.initials: R
authors.lastname: Lopes Caçola
authors.affiliation: Department of Internal Medicine, Hospital Pedro Hispano, Matosinhos, Portugal.
authors.forename: Sandra Alves
authors.initials: SA
authors.lastname: Morais
authors.affiliation: Department of Ophthalmology, Hospital Pedro Hispano, Matosinhos, Portugal.
authors.forename: Rui
authors.initials: R
authors.lastname: Carvalho
authors.affiliation: Department of Internal Medicine, Hospital Pedro Hispano, Matosinhos, Portugal.
authors.forename: Rui
authors.initials: R
authors.lastname: Môço
mesh_terms: D000328:Adult; D003607:Dacryocystitis / Q000209:etiology*; D003937:Diagnosis, Differential; D019468:Disease Management; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000000981:diagnostic imaging*; D006801:Humans; D008279:Magnetic Resonance Imaging; D009916:Orbital Diseases / Q000000981:diagnostic imaging*