Intermediate uveitis: pattern of etiology, complications, treatment and outcome in a tertiary academic center

Orphanet J Rare Dis. 2017 Apr 27;12(1):81. doi: 10.1186/s13023-017-0638-9.

Abstract

Background: Patients with intermediate uveitis (IU) represent a heterogenous group characterized by a wide spectrum of etiologies and regional differences. Aim of the study was to analyze the characteristics of patients with IU examined in an academic center in Germany.

Methods: We conducted a retrospective analysis of the clinical records of all patients with intermediate uveitis referred to the Eye Center, University of Freiburg from 2007 to 2014. Diagnosis followed the Standardization in Uveitis Nomenclature (SUN) criteria. Data analysis included: etiology of IU, demographics, complications, treatment and visual acuity.

Results: We identified 159 patients with intermediate uveitis during that period. Mean age at diagnosis was 35 years. Most are female (64%), and the mean duration of IU was 6.1 years (range 1 month - 35 years). Etiology of IU was idiopathic in 59%. Multiple sclerosis (MS) (20%) and sarcoidosis (10%) were frequent systemic causes of IU. Other etiologies including infectious diseases (tuberculosis, borreliosis) or immune-mediated conditions (eg, after vaccination) were present in 11%. The pattern of complications included macular edema (CME) (36%), cataract (24%), secondary glaucoma (7%), and epiretinal membrane formation (19%). Periphlebitis and optic neuritis were more frequent in conjunction with MS. Treatment comprised local and systemic steroids, immunosuppressive agents, biologics, and surgery. Best corrected visual acuity was better than 20/25 in 60% of the eyes after more than 10 years of follow-up.

Conclusions: In our German academic center, most IU cases were idiopathic or associated with MS or sarcoidosis. In contrast to other countries, infectious cases were rare. Patients' overall visual prognosis is favorable even when the duration of IU has been long and and despite numerous complications.

Keywords: Complications; Etiology; Intermediate uveitis; Systemic associations; Treatment.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Cataract / etiology*
  • Cataract / physiopathology
  • Child
  • Child, Preschool
  • Female
  • Glaucoma / etiology*
  • Glaucoma / physiopathology
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Macular Edema / etiology
  • Macular Edema / physiopathology
  • Male
  • Middle Aged
  • Multiple Sclerosis / complications*
  • Multiple Sclerosis / physiopathology
  • Retrospective Studies
  • Sarcoidosis / complications*
  • Sarcoidosis / physiopathology
  • Steroids / therapeutic use
  • Treatment Outcome
  • Uveitis, Intermediate* / complications
  • Uveitis, Intermediate* / drug therapy
  • Uveitis, Intermediate* / etiology
  • Uveitis, Intermediate* / physiopathology
  • Visual Acuity / physiology
  • Young Adult

Substances

  • Immunosuppressive Agents
  • Steroids