IgG4-RD is a fibroinflammatory disease affecting multiple organs, with B and T cells playing key roles in its pathogenesis. Diagnosis requires clinicopathological correlation due to the lack of specific tests. Glucocorticoids are effective but limited by toxicity, prompting research into glucocorticoid-sparing agents. B cell-targeted therapies like rituximab show promise, though more randomized trials are needed. Untreated IgG4-RD can lead to irreversible organ damage, emphasizing the need for close monitoring and long-term immunosuppression in some cases.