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To present the spectrum of clinical manifestations in thirty cases of orbital IgG4-related disease along with their epidemiological, radiological and histopathological data and to evaluate the clinical outcomes of the disease with targeted therapies like Immunosuppressants and Rituximab.

A prospective interventional study of thirty cases presenting with wide spectrum of clinical manifestations, that included upper eyelid swelling resulting from dacryoadenitis (16/30 cases), proptosis with extraocular muscles or intraconal fat involvement (10/30) and proptosis with lid swelling due to both lacrimal gland and extraocular muscles involvement (4/30), was carried at a tertiary hospital in New Delhi, India, between 2014-2019. Epidemiological profile and lab results were noted. Incision biopsy was carried out after orbital imaging and the diagnosis was made based on comprehensive diagnostic criteria by Umehara et al (2012). All the patients were started on oral steroids along with Immunosuppressants (Azathioprine or Methotrexate). Rituximab was used in cases non-responsive to the aforementioned drugs.

The average age at presentation was 40 years. The patients were diagnosed as Definite IgG4 disease in 12/30 cases, Possible IgG4 in 5/30 cases and Probable IgG4 in 13/30 cases. Twenty two of thirty cases had good clinical outcome with steroids (30/30 cases) and Immunosuppresants (Azathioprine 27/30 or Methotrexate 3/30). Eight of thirty cases resistant to steroids were stabilised with Rituximab therapy.

The diagnosis of IgG4-Related Disease is critical as it responds well to targeted therapies like Immunosuppressive agents and Rituximab resulting in better clinical outcomes.