Mikulicz Disease in Males: A Comprehensive Review

Abstract

IgG4-related disease (IgG4-RD) is an intricate autoimmune disorder that have an effect on multiple organs in the body, including the lachrymal and salivary glands, liver, biliary duct, thyroid, pancreas, gastrointestinal tract, kidney, aorta, prostate and retroperitoneum. Mikulicz disease, a subtype of IgG4-RD, specifically targets the lachrymal and salivary glands, leading to glandular swelling, xerostomia, and xerophthalmia. Diagnosis of Mikulicz disease is established on the presence of proportional and persistent inflammation of at least two pairs of lachrymal, submandibular, and parotid glands, along with exalted serum IgG4 levels and/or marked penetration of IgG4-positive plasmacytes into the affected glandular tissues. Treatment with glucocorticoids has shown to be beneficial in managing IgG4-related MD, but the efficacy of combination therapy in preventing relapse is still a topic of debate. The underlying causes of the disease can vary, but lymphoepithelial sialadenitis is the widespread cause. The disease typically presents in individuals with an average age ranging from 55 to 65 years, and the incidence among individuals under the age of 20 is exceedingly rare. Mikulicz disease is an enigmatic ailment that is still being researched. However, identifying its hallmark symptoms and distinguishing it from other similar conditions is essential for effective diagnosis and treatment. Further research is essential to better comprehend the fundamental causes of the disease and to promote more effective treatment options for patients with Mikulicz disease and other subtypes of IgG4-RD.