Ocular manifestation in a patient with IgG4 related disease

IgG4-related disease is a newly recognized fibroinflammatory disease presenting with multiple features including mass forming lesion; a dense lymphoplasmacytic infiltrate; a characteristic histopathological appearance and often elevated serum of IgG4. This disease can potentially affect any organ and interestingly, the affected organs share common histopathological features including a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and mild to moderate eosinophilia.

A 45-year-old man presented complaining of proptosis and gradual decrease in visual acuity of right eye. He had undergone many work-ups but without any definitive diagnosis. Through a combination of clinical and para-clinical investigations, the diagnosis of IgG4-RD was established. 693 mg/dL). Aggressive treatment (pulse of cyclophosphamide and pulse of corticosteroid) was started hoping to save the patient's vision. Two weeks following the treatment, there was improvement with his visual acuity and proptosis.

In any patient with chronic tumor like lesions and pseudotumors without the evidence of malignancy, we should think of IgG4-related disease. In this circumstance, biopsy may lead us to the definitive diagnosis. Early diagnosis and treatment of IgG4-RD may inhibit further irreversible organ damages.