A 58-year-old man presents with painless progressive swelling of the submandibular and lacrimal glands bilaterally. Serum IgG4 level is elevated at 285 mg/dL. CT shows diffuse glandular enlargement without focal masses. Biopsy shows lymphoplasmacytic infiltrate with storiform fibrosis and IgG4-positive plasma cells. What is the diagnosis?
- A Mikulicz disease (IgG4-related disease) ✓
- B Sjogren syndrome
- C MALT lymphoma
- D Sarcoidosis
Correct answer: A. Mikulicz disease (IgG4-related disease)
Explanation
IgG4-related disease (historically called Mikulicz disease when involving salivary and lacrimal glands) shows elevated serum IgG4, storiform fibrosis on biopsy, and IgG4-positive plasma cell infiltrates. It responds dramatically to glucocorticoids. Sjogren syndrome features anti-Ro/anti-La and lymphocytic (not IgG4-plasmacytic) infiltration. MALT lymphoma shows monoclonal A cells. Sarcoidosis shows non-caseating granulomas.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.