Objective: Mikulicz's disease (MD) is characterized by symmetrical and persistent enlargement of the lacrimal and salivary glands. Recently it has been categorized as an 'Ig (immunoglobulin) G4-related disease.' It presents with elevated serum levels of IgG4 and abundant infiltration of IgG4-bearing plasmacytes in involved organs. Allergic symptoms are often observed in patients with IgG4-related disease. On the other hand, allergic diseases are often complicated with Churg-Strauss syndrome (CSS). Here we focused on CSS and analyzed the relation of IgG4 in its pathogenesis.
Materials and methods: We analyzed five patients (2 men and 3 women) with CSS and 51 patients (20 men and 31 women) with MD who presented at Sapporo Medical University Hospital since 2001. We measured the serum concentrations of IgG subclasses in the patients with MD and CSS, and evaluated renal specimens from CSS patients, staining them for anti-IgG4 antibody.
Results: We surprisingly found elevated serum levels of IgG4 not only in MD but also in CSS patients. The renal specimens in CSS patients revealed the infiltration of IgG4-positive plasmacytes.
Conclusion: IgG4-bearing plasmacytes may be involved in the pathogenesis of CSS, and it is possible that an allergic reaction plays an important role in the pathogenesis of IgG4-related disease.