The clinical practice and the topics of IgG4-related dacryoadenitis and sialadenitis, so called Mikulicz’s dsease

Abstract

Introduction: Only a few years have passed since the concept of IgG4 related diseases was formed. There are also many unclear points in daily clinical practice. We aim to clarify its clinical features by development of an IgG4 related disease case registration system (Sapporo Medical University and related institutes database for investigation and best treatments against IgG4-related disease: SMART) and analyzing the data obtained from it. In this article, I would like to outline the clinical features of IgG4-related dacryoadenitis and sialadenitis (IgG4-DS), the actuality of daily clinical practice, the problems and new possibilities in the treatment.
Clinical features: The sex ratio of the patients with IgG4-DS is almost same. The average age of the onset is over sixties. About 60% of the patients have other organ involvements. There are some cases that malignancies are found at the diagnosis of IgG4-DS. Although glucocorticoid can easily induce the clinical remission, almost patients require maintenance therapy. It is easy to relapse as the steroid gradually decreases. In patients presented with repeating relapse, biological agents may be useful.
Conclusion: It is important to perform systemic screening for the detection of other organ involvements and malignancies at the diagnosis of IgG4-DS. Further, it is necessary to carry out the histopathological examination to rule out malignant lymphoma. It is highly probable that immunosuppressants including biological agents are useful for the patients who need high dose of prednisolone or present with repeated relapse, but how to position them in the treatment algorithms is a future subject.