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

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.

Access options

Rent or Buy article

Get time limited or full article access on ReadCube.

from$8.99

All prices are NET prices.

References

  1. 1.

    Jamilloux Y, Kodjikian L, Broussolle C, Seve P. Sarcoidosis and uveitis. Autoimmun Rev. 2014;13:840–9.

    Article  Google Scholar 

  2. 2.

    Mavrikakis I, Rootman J. Diverse clinical presentations of orbital sarcoid. Am J Ophthalmol. 2007;144:769–75.

    Article  Google Scholar 

  3. 3.

    Prabhakaran VC, Saeed P, Esmaeli B, Sullivan TJ, McNab A, Davis G, et al. Orbital and adnexal sarcoidosis. Arch Ophthalmol. 2007;125:1657–62.

    Article  Google Scholar 

  4. 4.

    Mombaerts I, Reinier SO, Goldschmeding R, Koornneef L. Idiopathic granulomatous orbital inflammation. Ophthalmology. 1996;103:2135–41.

    CAS  Article  Google Scholar 

  5. 5.

    Obenauf CD, Shaw HE, Sydnor CF, Klintworth GK. Sarcoidosis and its ophthalmic manifestations. Am J Ophthalmol. 1978;86:648–55.

    CAS  Article  Google Scholar 

  6. 6.

    Collison JM, Miller NR, Green WR. Involvement of orbital tissues by sarcoid. Am J Ophthalmol. 1986;102:302–7.

    CAS  Article  Google Scholar 

  7. 7.

    Henkind P. Sarcoidosis: an expanding ophthalmic horizon. J R Soc Med. 1982;75:153–9.

    CAS  PubMed  PubMed Central  Google Scholar 

  8. 8.

    Stein HA, Hendersen JW. Sarcoidosis of the orbit. Am J Ophthalmol. 1956;41:1054–6.

    CAS  Article  Google Scholar 

  9. 9.

    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.

    CAS  Article  Google Scholar 

  10. 10.

    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.

    Article  Google Scholar 

  11. 11.

    American Thoracic Society. Statement on sarcoidosis. Am J Respir Crit Care Med. 1999;160:736–55.

    Article  Google Scholar 

Download references

Acknowledgements

GER receives some funding from the National Institute of Health Research (NIHR) Biomedical Research Centre at Moorfields Eye Hospital NHS Foundation Trust and UCL Institute of Ophthalmology.

Author information

Affiliations

Authors

Corresponding author

Correspondence to Geoffrey E. Rose.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Additional information

Publisher’s note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Vahdani, K., Rose, G.E. Sarcoid-like granulomatous orbitopathy—presentation, systemic involvement and clinical outcome. Eye (2020). https://doi.org/10.1038/s41433-020-0874-4

Download citation