Abstract

Abstract Introduction Immunoglobulin G4-related disease is a fibrous-inflammatory disease characterized by lymphoplasmacytic tissue infiltration of IgG4 positive plasmablasts and small lymphocytes, that may be related to storiform fibrosis, obliterative phlebitis and modestly increased tissue eosinophils. This condition was recently recognized in 2013. It has unknown pathophysiology. The common characteristics shared by the different entities that make up the IgG4-related disease are: raised serum IgG4 levels, alterations in the imaging tests with neoplastic-like swelling of the affected organs, specific histopathological characteristics, and in immunostaining, as well as a good response to treatment with glucocorticoids. Clinical Case A 75-year-old male with a history of Grave's disease with ophthalmopathy, osteopenia, gastroesophageal reflux disease, obstructive sleep apnea, and benign prostatic hypertrophy presented to the clinic after a neck ultrasound and CT neck were ordered for investigation of bilateral "neck lumps". Neck ultrasound was evident for the heterogeneous appearance of submandibular glands and a hypoechoic nodule in the left neck with internal vascularity below the left jaw. CT neck with IV contrast showed evidence of possibly sialadenitis and lobular soft tissue thickening in the inferior aspect of the left submandibular gland measuring approximately 3.5 cm. Surgical pathology report of the left submandibular gland demonstrated a significant increase with IgG and IgG4 plasma cells and CD38 confirmed a dense plasmacytic infiltrate. IN the laboratory, IgG4 was elevated, 462 mg/dL (2-96 mg/dl) Conclusion As with many autoimmune diseases, it is unknown what triggers the Immunoglobulin G4-related disease. Given that the latter has just recently been diagnosed and recognized it is imperative to continue to record and develop data regarding it to better understand it. Multiple endocrine organs could be involved in the pathology of IgG4 related disease. The health care providers need to be cognizant of this condition and the wide variety of manifestations it could have. This disease can be first encountered by any medical field due to the broad spectrum of clinical findings that it could encompass. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

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