title: Ophthalmic manifestations in IgG4-related disease: Clinical presentation and response to treatment in a French case-series.
abstract: IgG4-related disease (IgG4-RD) is characterized by variable tissue or organ involvements sharing common pathological findings. Orbital or orbital adnexa involvement of the disease has been reported in a few case series. The aim of our study was to characterize and analyze ophthalmic manifestations from a nationwide French case-series.Patients with IgG4-RD and orbital or orbital adnexa involvement included in the French multicentric IgG4-RD case-registry were identified. Only patients fulfilling "modified" comprehensive diagnostic criteria with pathological documentation were retained for the study. Clinical, biological, pathological, radiological findings and data regarding the response to treatment were retrospectively analyzed.According to our data registry, the frequency of IgG4-related ophthalmic disease (IgG4-ROD) was 17%. Mean age at diagnosis was 55.1 ± 7.1 years with a male/female ratio of 2.2. The 19 cases of IgG4-ROD consisted of lacrimal gland (68.4%), soft tissue (57.9%), extra-ocular muscles (36.8%), palpebral (21.1%), optical nerve (10.5%), orbital bone (10.5%), and mononeuritis (V1 and/or V2, 10.5%) involvements. IgG4-ROD was bilateral in 57.9% of cases. Extra-ophthalmic manifestations were reported in 78.9% of cases. All patients responded to prednisone but two-thirds of patients relapsed within a mean (SD) of 9.8 (3.5) months and 72.2% required long-term glucocorticoids and/or immunosuppressive agents. Eight patients were treated by rituximab with a favorable response in 87.5% of cases.Lacrimal involvement is the most frequent ophthalmic manifestation of IgG4-RD and is frequently associated with extra-orbital manifestations. Despite initial favorable response to steroids, the long-term management of relapsing patients needs to be improved.
authors.affiliation: Médecine Interne, Groupe Hospitalier Timone, AP-HM, Aix-Marseille Université, Marseille Médecine Interne, Hopital Cochin, Université Paris-Descartes, Paris Médecine Interne, Centre hospitalier de Pau, Pau CHRU-Hôpital Claude Huriez, Université de Lille, Lille Médecine Interne, Groupe hospitalier Pitie Salpêtrière AP-HP, Université Pierre et Marie Curie, Paris Médecine interne, CHU le Bocage, Université de Bourgogne, Dijon Médecine Interne, CHU Rennes, Rennes Médecine interne, CHU Mondor, Créteil CHG Saint Denis de la Réunion, Réunion, France.
authors.forename: Mikael
authors.initials: M
authors.lastname: Ebbo
authors.forename: Matthieu
authors.initials: M
authors.lastname: Patient
authors.forename: Aurelie
authors.initials: A
authors.lastname: Grados
authors.forename: Matthieu
authors.initials: M
authors.lastname: Groh
authors.forename: Julien
authors.initials: J
authors.lastname: Desblaches
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
authors.forename: David
authors.initials: D
authors.lastname: Saadoun
authors.forename: Sylvain
authors.initials: S
authors.lastname: Audia
authors.forename: Aude
authors.initials: A
authors.lastname: Rigolet
authors.forename: Benjamin
authors.initials: B
authors.lastname: Terrier
authors.forename: Antoinette
authors.initials: A
authors.lastname: Perlat
authors.forename: Constance
authors.initials: C
authors.lastname: Guillaud
authors.forename: Frederic
authors.initials: F
authors.lastname: Renou
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Bernit
authors.forename: Nathalie
authors.initials: N
authors.lastname: Costedoat-Chalumeau
authors.forename: Jean-Robert
authors.initials: JR
authors.lastname: Harlé
authors.forename: Nicolas
authors.initials: N
authors.lastname: Schleinitz
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D005123:Eye / Q000473:pathology*; D005128:Eye Diseases / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D005260:Female; D006801:Humans; D007154:Immune System Diseases / Q000150:complications*; D007074:Immunoglobulin G; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D007074:Immunoglobulin G; D011241:Prednisone