Long-term follow up of systemic rituximab therapy as first-line and salvage therapy for idiopathic orbital inflammation and review of the literature

ABSTRACT

Purpose

To report long-term outcomes of systemic rituximab therapy for idiopathic orbital inflammation (IOI) as both primary and salvage therapy and to review the English literature.

Methods

A retrospective review of four consecutive biopsy-proven IOI cases managed with systemic rituximab including demographics, management, and outcomes, and review of English literature, were performed. Primary outcome measures included resolution of symptoms, recurrence, and length of follow up.

Results

Of four cases, systemic rituximab was the first-line therapy in two cases and salvage therapy in two cases. The mean age of the patients was 62 years (range, 50–68 years). The orbit was involved in three cases and extraocular muscle in one case. Systemic rituximab (1 g weekly for 4 weeks) was given for one session in three patients and for 12 sessions in 1 patient. All four patients responded with the resolution of all symptoms without recurrence after at least 5 years of follow up. Review of the literature showed systemic rituximab had provided clinical improvement at shorter follow up in 14 of 15 cases when used as a salvage therapy.

Conclusions

Systemic rituximab therapy seems to be an effective therapy for IOI as salvage or first-line therapy with long-term clinical durability.

KEYWORDS:

• Corticosteroids
• first line therapy
• idiopathic orbital inflammation
• orbital pseudotumor
• rituxan
• rituximab
• salvage therapy

Author contributions

Jacob Abou-Hanna: Conceptualization, Data Curation, Formal Analysis, Investigation, Methodology, Writing – original draft, Writing – review & editing. Honeylen M. Tiu Teo: Formal Analysis, Investigation, Methodology, Writing – review & editing. Rajarathna Thangavel: Conceptualization, Data Curation, Writing – original draft. Victor M. Elner: Conceptualization, Writing – review & editing. Hakan Demirci: Conceptualization, Investigation, Methodology, Writing – review & editing.

Disclosure statement

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

Meeting presentation

This material has not been previously presented.

Additional information

Funding

This is supported by Richard N and Marilyn K Witham Professorships. They have no effect on the design, conduct, or results of the study.