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• W4387247951 abstract "SESSION TITLE: Pulmonary Manifestations of Systemic Disease Case Report Posters 11 SESSION TYPE: Case Report Posters PRESENTED ON: 10/10/2023 09:40 am - 10:25 am INTRODUCTION: IgG 4 related disease is a rare systemic fibro-inflammatory disease characterized by infiltration of IgG4-positive plasma cells in the affected tissue(s) with or without elevated plasma levels of IgG4. It is unclear whether the antibodies are actively causing the pathogensis in the infiltrative disease or rather they are a marker of the disease. IgG4 subclass has been thought to be mostly anti-inflammatory in nature. Pulmonary involvement in IgG4 disease is variable and complex with case reports describing an interstitial process to others describing airway disease or mediastinitis. CASE PRESENTATION: 66 year old male with history of cirrhosis comes to the hospital with AKI, worsening dyspnea. He reports a sedentary lifestyle but progressively worsening dyspnea for 1 year previous to admission. SPEP evaluation revealed markedly elevated IgG which later led to diagnosis of IgG4 related disease. ECHO cardiogram showed grade 3 diastolic dysfunction with restrictive features. CT scan of the chest was also performed and revealed multifocal ground glass opacification on a background of septal thickening, bronchiectasis suggestive of pulmonary fibrosis. A recent salivary gland biopsy revealed lymphoplasmacytic infiltrate, a characteristic finding of IgG4 related disease. DISCUSSION: IgG4 is a multisystem disease which consists of lymphoplasmacytic infiltrate which can result in fibrotic disease in almost any tissue. Serum evaluations of immunoglobulins should help diagnose the disease however these elevations can be present in certain patients without teh disease process. Biopsy of involved tissue can lead to diagnosis for many patients. CONCLUSIONS: Patient eventually was started on prednisone and followed up in the outpatient setting where his symptoms had improved significantly. Physicians should be aware of this disease entity in order to identify patients with fibrotic lung disease in the presence of multiple non specific pathological findings. REFERENCE #1: Campbell SN, Rubio E, Loschner AL. Clinical review of pulmonary manifes-tations of IgG4-related disease. Ann Am Thorac Soc 2014;11(9):1466–1475 REFERENCE #2: Perugino CA, Stone JH (2020) IgG4-related disease: an update on pathophysiology and implications for clinical care. Nat Rev Rheumatol 16:702–714 REFERENCE #3: Nambiar S, Oliver TI. IgG4 Related Disease. [Updated 2022 Aug 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 DISCLOSURES: No relevant relationships by Joseph Bahgat No relevant relationships by Ameer Rasheed" @default.
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• W4387247951 date "2023-10-01" @default.
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• W4387247951 title "IGG4-RELATED ILD: CASE REPORT OF A MULTISYSTEM DISEASE PROCESS" @default.
• W4387247951 doi "https://doi.org/10.1016/j.chest.2023.07.3567" @default.
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