Multiple Organ Involvements in a Case of IgG4 Related Disease

Abstract

A 64-year-old woman with presentation of proptosis referred to Rheumatologic clinic at Amir- Alam hospital. The result of physical exam revealed that there was a left eye proptosis with 29 mm in size and no light perception. Computed tomography showed mass infiltration to left carotid sheet and mandibular foramen. Besides, there was an increase in hepatic vascularity and peri-portal infiltration predominantly in the left lobe that caused intra-hepatic duct dilation. Despite of the deterioration of the proptosis and visual acuity, probably due to limited knowledge about IgG4-RD, the patient did not receive the appropriate treatment. Unfortunately, this issue caused significant reduction in the visual acuity (no light perception). Finally, pathology and IHC study revealed presence of CD20+ plasma cell up to 60% per High power field and with the diagnosis of IgG4-RD, methylprednisolone and cyclophosphamide initiated and we observed a significant reduction of symptoms.