Mikulicz's Disease with hypophysitis - a new IgG4-mediated disorder

Area of Science:

• Endocrinology
• Immunology
• Rheumatology

Background:

• Mikulicz's Disease is a rare condition, often associated with IgG4-related disease.
• IgG4-related diseases are systemic autoimmune conditions characterized by IgG4-positive plasma cell infiltration.

Observation:

• A 55-year-old male with Mikulicz's Disease developed panhypopituitarism two years after initial treatment.
• Clinical presentation included fatigue, weakness, and significant weight loss.
• Pituitary MRI revealed enlargement and stalk thickening, with elevated serum IgG4 levels.

Findings:

• The patient was diagnosed with IgG4-related hypophysitis.
• Treatment with methylprednisolone pulse therapy and oral steroids led to significant radiological improvement.
• Diabetes insipidus emerged as a complication during steroid therapy.

Implications:

• This case underscores the importance of recognizing IgG4-related hypophysitis in patients with Mikulicz's Disease.
• Elevated serum IgG4 levels are crucial for diagnosis.
• Prompt steroid therapy can effectively manage IgG4-related hypophysitis, reversing inflammatory changes.