title: [Refractory form of orbital granulomatosis with polyangiitis: A clinical and morphological study].
abstract: Orbital granulomatosis with polyangiitis (Wegener's granulomatosis, GPA), which is characterized by granulomatous inflammation with small-vessel vasculitis, can develop in local and generalized forms of the disease. The introduction of current immunosuppressive therapy regimens has improved the prognosis of the disease; however, there are immunosuppressive treatment-refractory forms of GPA, the morphology of which has been inadequately investigated. The paper describes a clinical case of refractory GPA involving the orbit, as evidenced by histological and immunohistochemical examinations. The specific feature of the case is the development of severe fibrosis with the accumulation of mainly type III collagen and the persistence of granulomatous inflammation and productive-destructive vasculitis.
authors.affiliation: I.M. Sechenov First Moscow State Medical University, Moscow, Russia.
authors.forename: E A
authors.initials: EA
authors.lastname: Kogan
authors.affiliation: I.M. Sechenov First Moscow State Medical University, Moscow, Russia; Research Institute of Eye Diseases, Moscow, Russia.
authors.forename: Ya O
authors.initials: YO
authors.lastname: Grusha
authors.affiliation: I.M. Sechenov First Moscow State Medical University, Moscow, Russia; Research Institute of Eye Diseases, Moscow, Russia.
authors.forename: D S
authors.initials: DS
authors.lastname: Ismailova
authors.affiliation: I.M. Sechenov First Moscow State Medical University, Moscow, Russia; University Clinical Hospital Three, I.M. Sechenov First Moscow State Medical University, Moscow, Russia.
authors.forename: P I
authors.initials: PI
authors.lastname: Novikov
authors.affiliation: Research Institute of Eye Diseases, Moscow, Russia.
authors.forename: Yu V
authors.initials: YV
authors.lastname: Abramova
authors.affiliation: I.M. Sechenov First Moscow State Medical University, Moscow, Russia; University Clinical Hospital Three, I.M. Sechenov First Moscow State Medical University, Moscow, Russia.
authors.forename: A D
authors.initials: AD
authors.lastname: Meshkov
authors.affiliation: Research Institute of Eye Diseases, Moscow, Russia.
authors.forename: E F
authors.initials: EF
authors.lastname: Rizopulu
mesh_terms: D000328:Adult; D001808:Blood Vessels / Q000473:pathology; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007249:Inflammation / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D008297:Male; D011379:Prognosis; D014657:Vasculitis / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy