Sarcoid-like granulomatous orbitopathy-presentation, systemic involvement and clinical outcome.

Item request has been placed! ×
Item request cannot be made. ×
loading   Processing Request
Read More Add to Saved list
  • Author(s): Vahdani K;Vahdani K; Rose GE; Rose GE
  • Source:
    Eye (London, England) [Eye (Lond)] 2021 Feb; Vol. 35 (2), pp. 470-476. Date of Electronic Publication: 2020 Apr 21.
  • Publication Type:
    Journal Article; Research Support, Non-U.S. Gov't
  • Language:
    English
  • Additional Information
    • Source:
      Publisher: Nature Publishing Group Country of Publication: England NLM ID: 8703986 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1476-5454 (Electronic) Linking ISSN: 0950222X NLM ISO Abbreviation: Eye (Lond) Subsets: MEDLINE
    • Publication Information:
      Publication: <2003->: London : Nature Publishing Group
      Original Publication: [London : Ophthalmological Society of the United Kingdom, 1987-
    • Subject Terms:
      Graves Ophthalmopathy* ; Lacrimal Apparatus Diseases* ; Orbital Diseases*/diagnosis ; Orbital Diseases*/drug therapy ; Sarcoidosis*/diagnosis ; Sarcoidosis*/drug therapy; Adolescent ; Adult ; Aged ; Female ; Humans ; Middle Aged ; Retrospective Studies ; Young Adult
    • Abstract:
      Aim: To describe patients with sarcoid-like granulomatous orbitopathy (SLGO), the rate of known and subsequent systemic sarcoidosis, and the treatment and outcome for the condition.
      Patients and Methods: Retrospective review of patients with SLGO presenting between 1990 and 2018, excluding solely lid or lacrimal drainage disease.
      Results: Sixty-one patients (45 female; 74%) were identified, 54 having biopsy-proven sarcoidosis (47 orbital, 24 prior extra-orbital), and half were Afro-Caribbean. The average age at presentation was 45.3 years (range 18-78 years), with the commonest symptoms being swelling, pain and diplopia; of clinical signs, most patients (87%) had eyelid swelling, reduced motility (25%), and disease-related visual impairment (10%). Localized dacryoadenitis was present in 49/61 (21/49 bilateral) patients, and more diffuse disease in 28/61 (8/28 bilateral). Systemic involvement was found in 23 (62%) of the 37 first presenting with orbital disease. Twenty-three (38%) patients were observed and two-thirds received oral corticosteroids, with 53/61 (87%) patients having subjective and objective clinical improvement. The average follow-up was 53.4 months (range 1-315 months) and clinical recurrence occurred in 12 (20%) patients at 26.5 months (range 1-115 months) after first diagnosis.
      Conclusions: Systemic sarcoidosis may be discovered in about two-thirds of patients presenting with SLGO (that tends to present with inflammatory features), but the treatment response appears similar in patients with known sarcoidosis and those with newly-diagnosed systemic disease after orbital presentation. With long-term follow-up, a third of patients have spontaneous regression of orbital disease, but 20% have recurrence after reducing or stopping systemic immunosuppression.
    • References:
      Jamilloux Y, Kodjikian L, Broussolle C, Seve P. Sarcoidosis and uveitis. Autoimmun Rev. 2014;13:840–9. (PMID: 10.1016/j.autrev.2014.04.001)
      Mavrikakis I, Rootman J. Diverse clinical presentations of orbital sarcoid. Am J Ophthalmol. 2007;144:769–75. (PMID: 10.1016/j.ajo.2007.07.019)
      Prabhakaran VC, Saeed P, Esmaeli B, Sullivan TJ, McNab A, Davis G, et al. Orbital and adnexal sarcoidosis. Arch Ophthalmol. 2007;125:1657–62. (PMID: 10.1001/archopht.125.12.1657)
      Mombaerts I, Reinier SO, Goldschmeding R, Koornneef L. Idiopathic granulomatous orbital inflammation. Ophthalmology. 1996;103:2135–41. (PMID: 10.1016/S0161-6420(96)30378-3)
      Obenauf CD, Shaw HE, Sydnor CF, Klintworth GK. Sarcoidosis and its ophthalmic manifestations. Am J Ophthalmol. 1978;86:648–55. (PMID: 10.1016/0002-9394(78)90184-8)
      Collison JM, Miller NR, Green WR. Involvement of orbital tissues by sarcoid. Am J Ophthalmol. 1986;102:302–7. (PMID: 10.1016/0002-9394(86)90002-4)
      Henkind P. Sarcoidosis: an expanding ophthalmic horizon. J R Soc Med. 1982;75:153–9. (PMID: 72001411437549)
      Stein HA, Hendersen JW. Sarcoidosis of the orbit. Am J Ophthalmol. 1956;41:1054–6. (PMID: 10.1016/0002-9394(56)91057-1)
      Siltzbach LE, James DG, Neville E, Turiaf J, Battesti JP, Sharma OP, et al. Course and prognosis of sarcoidosis around the world. Am J Med. 1974;57:847–52. (PMID: 10.1016/0002-9343(74)90160-0)
      Demirci H, Christianson MD. Orbital and adnexal involvement in sarcoidosis: analysis of clinical features and systemic disease in 30 cases. Am J Ophthalmol. 2011;151:1074–80. (PMID: 10.1016/j.ajo.2010.12.011)
      American Thoracic Society. Statement on sarcoidosis. Am J Respir Crit Care Med. 1999;160:736–55. (PMID: 10.1164/ajrccm.160.2.ats4-99)
    • Publication Date:
      Date Created: 20200423 Date Completed: 20210618 Latest Revision: 20220202
    • Publication Date:
      20231215
    • Accession Number:
      PMC8027433
    • Accession Number:
      10.1038/s41433-020-0874-4
    • Accession Number:
      32317795