[Comparative study of clinical features between unilateral and bilateral orbital IgG4-related disease]

Arch Soc Esp Oftalmol. 2015 Sep;90(9):407-13. doi: 10.1016/j.oftal.2015.02.018. Epub 2015 Apr 10.
[Article in Spanish]

Abstract

Objective: Descriptive and comparative study of patients with orbital IgG4-related disease.

Material and methods: A review and analysis of the cases diagnosed with inflammatory orbital lesion related to IgG4 by the Ophthalmic Pathology Service in the Dr. Luis Sánchez Bulnes Hospital.

Results: A total of 9 cases were found, in which 66% were women, and with a mean age of 48 years and time to diagnosis of 2 years. Unilateral involvement was observed in 56% of cases. All the females experienced pain, and there was an optimal response to corticosteroid treatment in 100% of patients who required medical treatment (one case showed spontaneous resolution). In bilateral cases (44%), only 25% were female, and none had pain as a presenting symptom. Furthermore, 25% of these patients required a combination with immunosuppressants to control inflammation.

Conclusions: Clinical presentation of patients with unilateral orbital IgG4-related disease differs from those with bilateral involvement.

Keywords: Dacrioadenitis; Dacryoadenitis; Enfermedad asociada a IgG4; Enfermedad de Mikulicz; Enfermedad inflamatoria de la órbita; IgG4-related disease; Inflammatory orbital disease; Mikulicz's disease; Orbital tumor; Tumor orbitario.

Publication types

  • Observational Study

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Aged
  • Chronic Disease
  • Cross-Sectional Studies
  • Dacryocystitis / diagnosis
  • Dacryocystitis / drug therapy
  • Dacryocystitis / etiology*
  • Dacryocystitis / immunology
  • Delayed Diagnosis
  • Eye Pain / etiology
  • Female
  • Humans
  • Hypergammaglobulinemia / complications*
  • Immunoglobulin G* / analysis
  • Male
  • Middle Aged
  • Mikulicz' Disease / diagnosis
  • Mikulicz' Disease / drug therapy
  • Mikulicz' Disease / etiology*
  • Mikulicz' Disease / immunology
  • Orbital Diseases / diagnosis
  • Orbital Diseases / drug therapy
  • Orbital Diseases / etiology*
  • Orbital Diseases / immunology

Substances

  • Adrenal Cortex Hormones
  • Immunoglobulin G