Disease modifying drugs in idiopathic sclerosing orbital inflammatory syndrome

ABSTRACT

Purpose

Idiopathic sclerosing orbital inflammatory syndrome (ISOIS) is a rare, progressive and hard to control disease. There is a deep gap of evidence regarding application of disease-modifying drugs (DMD) regimen as a potentially effective treatment for orbital inflammatory diseases. We aimed to report the results of using DMDs and discuss the concept of applying this modality of treatment in patients with ISOIS.

Methods

This was a prospective interventional case series conducted in a tertiary university-based hospital. Biopsy proven patients with active ISOIS were included. Systematic criteria were developed to define and measure disease activity and monitor response to treatment. A DMD regimen including an anti-tumor necrosis factor alpha (anti-TNF alpha) agent plus azathioprine and low-dose corticosteroids were used. Comprehensive ophthalmic, orbital and systemic assessments were performed during each visit.

Results

Five eligible patients with primary ISOIS were included. Mean age was 34.20 (SD = 13.33, range 19–53) years. Three had unilateral and two had bilateral involvement. Four had diffuse orbital involvement pattern and progressive worsening of visual functions, reduced extraocular motility and proptosis. In one patient the disease was localized to extraocular muscle and lacrimal gland. Disease activity was decreased and stabilized after DMDs regimen in all patients. Mean follow up was 32.80 (SD = 30.80, range: 12–86) months.

Conclusion

Biologic DMD (b-DMD) including anti-TNF alpha, corticosteroid and azathioprine were effective in decreasing disease activity and could change course of the disease. This study supports the concept of using b-DMD regimen in treatment of ISOIS.

KEYWORDS:

• Adalimumab
• anti-TNF agent
• biologic agents
• disease modifying drugs
• etanercept
• idiopathic sclerosing orbital inflammatory disease
• idiopathic sclerosing orbital inflammatory syndrome
• inflammatory orbital pseudotumor
• infliximab
• non-specific orbital inflammatory syndrome
• orbital imaging
• orbital MRI
• xanthelasma

Acknowledgments

We would like to thank Ms Elham Roshan and Ms Marjan Marvi for their valuable help to receive consent forms and arrange frequent patients’ visits.

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the article.

Supplementary material

Supplemental data for this article can be accessed on the publisher’s website

Additional information

Funding

Authors received no fund for performing this study. Part of this study was presented at ASOPRS meeting 2016, Chicago, USA and at SOE meeting 2017, Barcelona, Spain as lecture.