Abstract: Objective To explore the clinical features, pathological features and imaging manifestations of IgG4-related disease. Methods The clinical data of four cases of IgG4-related disease were analyzed and the related literatures were summarized. Results The average age was 55 years old. The proportion of male: female was 3∶1. 2 cases occurred in the submandibular gland, 1 in the eye socket and 1 in the facial region. There were no significant specificity, and the clinical manifestations were painless progressive swelling. Pathologic changes were lymphoid tissue and fibrous tissue hyperplasia, which had more plasma cell infiltration(IgG4 positive plasma cells more than 50/HPF, IgG4 positive plasma cells were greater than 40% IgG positive plasma cells). Inflammatory changes were lesions on imaging area. Conclusion IgG4 related disease is a rarely clinical disease, pathogenesis is unclear. Combined with clinical manifestations, serological detection, pathological features and imaging findings, diagnosis results are obtained, and timely accurate treatment can be carried out.

Key words: IgG4-related disease, Submadibular gland, Lacrimal gland, Diagnosis