Abstract
SESSION TITLE: Pulmonary Manifestations of Systemic Disease 4 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Immunoglobulin G4 Related Disease (IgG4-RD) has been recognized in pulmonary and extra-pulmonary inflammatory states. IgG4-RD encompasses a multitude of diseases once thought to be separate entities unto themselves. We herein present a case of IgG4-RD in a 72-year-old man, which was thought to be a recurrence of his distant renal cell carcinoma. CASE PRESENTATION: A 72-year-old man presented to his pulmonologist’s office for an incidental finding of pulmonary opacities with an adjacent axillary solitary lymphadenopathy on a cancer follow-up screening CT of the chest. Further testing with a PET/CT revealed uptake in the both the lymph node and affected lung tissue. Due to concern for distant metastatic disease, an excisional biopsy was performed. Tissue sampling revealed florid reactive follicular hyperplasia with intrafollicular histiocytes and an abundance of IgG4-positive plasma cells, which is the hallmark of IgG4-RD. The patient received corticosteroids, which caused resolution of the pulmonary opacities. The patient returned to the hospital three months later with severe abdominal pain and was diagnosed with acute pancreatitis. At first, he was managed with conservative medical therapy, but his condition worsened. He was then given a course of corticosteroids and his symptoms began to slowly resolve. DISCUSSION: IgG4-RD was termed in 2010 for a systemic fibroinflammatory disease, which presents with tumefactive lesions. A definite diagnosis of IgG4-RD depends on clinical findings such as masses, hematological findings of elevated serum IgG4 levels, and histopathologic findings of lymphoplasmacytic infiltration of IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. Treatment is recommended initially with steroids, and other immunosuppressants such as rituximab can be used as steroid sparing treatment. IgG4-RD has many organ system involvements, among them are autoimmune pancreatitis, Mikulicz’s disease, Riedel’s thyroiditis, multi-organ inflammatory pseudotumors, Ormond’s disease, idiopathic hypocomplementemic tubulointersitital nephritis, and multifocal fibrosclerosis. Pulmonary parenchymal involvement frequently presents radiographically as a mass-like, focal opacity, which may mimic a neoplasm, and may show uptake on PET/CT. CONCLUSIONS: IgG4-RD closely resembles malignancies, and has a favorable response to treatment. Therefore, heightened awareness of IgG4-RD and its early diagnosis may significantly improve patients’ morbidity. Reference #1: Campbell SN, Rubio E, Loschner AL. Clinical review of pulmonary manifestations of IgG4-related disease. Ann Am Thorac Soc 2014;11:1466-75. Reference #2: Wolfson AR, Hamilos DL. Recent advances in understanding and managing IgG4-related disease. F1000Res 2017;6 Reference #3: Khosroshahi A, Stone JH. A clinical overview of IgG4-related systemic disease. Curr Opin Rheumatol. United States 2011:57-66. DISCLOSURE: The following authors have nothing to disclose: Shimshon Wiesel, Anna Gutman, Paul Catella, Phyllis Suen, Abdul Siddiqui, Ali Abou Yassine, Dany El-Sayegh No Product/Research Disclosure Information
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