DR. OBAIDUR REHMAN

DR.(MRS.) KASTURI BHATTACHARJEE, DR. DIPANKAR DAS, DR. VATSALYA VENKATRAMAN

Abstract

A 66-year-old male presented with bilateral lid swelling, ptosis and bilateral submandibular gland enlargement. Contrast enhanced CT scans revealed homogenously enhanced, diffusely enlarged lacrimal glands. Diagnoses of Lymphoma, Sjogren syndrome, Sarcoidosis, IgG4-related disease (IgG4-RD) and metastases were kept in mind. Incision biopsy and histopathology from lacrimal gland showed lymphoplasmacytic infiltrates in storiform pattern. Immunohistochemistry showed 35% plasma cells positive for IgG4. Blood investigations showed raised serum IgG4 levels. A diagnosis of IgG4-Related disease was made, owing to supportive histopathology, immunohistochemistry and serum IgG4 levels. The patient showed excellent response to systemic immunomodulators. Clinicians must be aware of this clinical entity and its varied presentations to diagnose early and tackle it effectively. IgG4-RD may rarely convert into a low-grade lymphoma; thus, these patients require a close follow-up and monitoring.