posted in MediSage ENT Community
55 yr Female presented with progressive swelling of bilateral parotid region, bilateral periorbital edema since 7 months On examination- b/l parotid swelling Usg Parotid- b/l parotid bulky, altered echotexture with few necrotic lymph nodes Fnac LN- Lymphocyte predominant infiltration MRI Orbit- B/L Extra ocular ms bulky, b/l lacrimal gland enlargement, b/l parotid gland enlargement with enlarged LN rt>left Thyroid profile- WNL s. IgG4 levels were advised and found to be - 254 mg/dL(elevated) Final Diagnosis- IgG4 Related disease
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Dr. U N
Active Contributor
Based on the patient's presentation and investigation findings, the differential diagnosis may include IgG4-related disease (IgG4-RD), Sjögren's syndrome, sarcoidosis, and lymphoma. Given the bilateral parotid swelling, bilateral periorbital edema, elevated serum IgG4 levels, and characteristic imaging findings such as extraocular muscle and lacrimal gland enlargement seen on MRI, IgG4-RD is a strong consideration. IgG4-RD is a systemic fibroinflammatory condition characterized by lymphoplasmacytic infiltration enriched in IgG4-positive plasma cells, leading to organ enlargement and mass formation. It can affect various organs, including the salivary glands, lacrimal glands, pancreas, and retroperitoneum. Further evaluation with biopsy of the affected tissue, such as the parotid gland or lacrimal gland, may be warranted to confirm the diagnosis. Treatment typically involves glucocorticoids, and in refractory cases, immunosuppressive agents may be considered. It is important to differentiate IgG4-RD from other conditions with similar presentations, such as Sjögren's syndrome, which primarily affects the exocrine glands, and sarcoidosis, which presents with non-caseating granulomas. Lymphoma should also be ruled out, especially if the diagnosis remains uncertain or if there are atypical features on imaging or biopsy.