title: Clinical features of IgG4-related dacryoadenitis.
abstract: BACKGROUND
  To elucidate the clinical characteristics of IgG4-related dacryoadenitis.
  
  
  METHODS
  Clinical features, laboratory findings, radiological findings, associated diseases, treatment, and prognosis were prospectively examined in 12 patients (seven men, five women; mean age, 60.9 ± 15.1 years) with IgG4-related dacryoadenitis.
  
  
  RESULTS
  In addition to eyelid swelling, other ophthalmologic symptoms were observed in seven patients, including diplopia (n = 4), ptosis (n = 2), visual field disturbance (n = 2), eye pain (n = 2), decrease of visual acuity (n = 2), eye-movement disturbance (n = 1), dry eye (n = 1), corneal ulcer (n = 1), and epiphora (n = 1). Swelling of the lacrimal glands was bilateral in half of the patients. Other IgG4-related diseases were present in nine patients, including sialadenitis (n = 5), autoimmune pancreatitis (n = 4), retroperitoneal fibrosis (n = 2), and lymphadenopathy (n = 8). Serum IgG4 levels were significantly higher in patients with other IgG4-related disease (1070 ± 813 mg/dl) than in those without (197 ± 59 mg/dl, p = 0.017). Allergic histories and elevated serum IgE levels were each detected in six patients. Eight patients showed inflammatory extension beyond the lacrimal gland, such as thickened rectus muscle (n = 6), inflammation of the optic nerve (n = 2), and retrobulbar inflammation (n = 3). Steroid therapy was effective in seven patients, but dacryoadenitis relapsed in two patients with markedly higher serum IgG4 levels and autoimmune pancreatitis.
  
  
  CONCLUSIONS
  IgG4-related dacryoadenitis showed various ophthalmologic symptoms due to extensive inflammation beyond the lacrimal gland, frequent association with other IgG4-related disease or allergic phenomena, and steroid responsiveness.
authors: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo, 113-8677, Japan.
authors: Satomi
authors: S
authors: Koizumi
authors: Terumi
authors: T
authors: Kamisawa
authors: Sawako
authors: S
authors: Kuruma
authors: Taku
authors: T
authors: Tabata
authors: Susumu
authors: S
authors: Iwasaki
authors: Kazuro
authors: K
authors: Chiba
authors: Keigo
authors: K
authors: Setoguchi
authors: Shinichiro
authors: S
authors: Horiguchi
authors: Noriko
authors: N
authors: Ozaki
mesh_terms: D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000276:immunology; D003316:Corneal Diseases / Q000175:diagnosis / Q000276:immunology; D003607:Dacryocystitis / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D004172:Diplopia / Q000175:diagnosis / Q000276:immunology; D005128:Eye Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D005141:Eyelid Diseases / Q000175:diagnosis / Q000276:immunology; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D015835:Ocular Motility Disorders / Q000175:diagnosis / Q000276:immunology; D009801:Oculomotor Muscles / Q000473:pathology; D009902:Optic Neuritis / Q000175:diagnosis / Q000276:immunology; D011446:Prospective Studies; D012793:Sialadenitis / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D014057:Tomography, X-Ray Computed; D014786:Vision Disorders / Q000175:diagnosis / Q000276:immunology
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G