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Kidney Week

Abstract: FR-PO687

Lung Cancer Before and Acute Myeloid Leukemia (AML) After the Diagnosis of IgG4-Related Disease

Session Information

Category: Trainee Case Report

  • 1500 Onco-Nephrology

Authors

  • Metwally, Sherif, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
  • Ayoub, Isabelle, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
  • Satoskar, Anjali A., Ohio State University, Columbus, Ohio, United States
Introduction

IgG4-related disease (IgG4-RD) is a multi-organ immune mediated fibro-inflammatory condition. The common diagnostic features of IgG4-RD are tumefactive lesions of involved organs, lymphoplasmacytic infiltrate enriched in IgG4-positive plasma cells and storiform fibrosis. Elevated serum concentrations of IgG4 are present in 70% of cases. Glucocorticoids are first line therapy. Recurrent and refractory cases are common and may benefit from steroid sparing maintenance therapy. This case illustrates the association of IgG4-RD with an increased risk of malignancy.

Case Description

A 53 -year old woman with history of epithelioid neoplasm of the lung s/p wedge resection in 2016 presented 10 months later with dyspnea. Diagnostic work up was significant for an exudative pleural effusion without malignant cells and bilateral hydroureteronephrosis with Scr of 3.5 mg/dl and urine PCR 1.1g/g despite stent placement. Kidney biopsy showed extensive dense fibrosis with one focus of positive IgG4 staining (20cells/hpf). Archived lung tissue from wedge resection as well as thyroid tissue from remote partial thyroidectomy stained positive for IgG4. Serum IgG4 was 106 mg/dl. Remission was successfully achieved with glucocorticoids. Scr improved to 1mg/dl and IgG4 level to 30.9mg/dl. However her IgG4-RD relapsed once prednisone was tapered below 10 mg/dl. Rituximab 1g q 14 days x2 doses along with prednisone taper did not help. PET scan showed hypermetabolic activity in the cervical and chest lymph nodes, thyroid, right lung, right hepatic lobe and both kidneys. Bone marrow biopsy with flow cytometry was negative for malignancy. Oral cyclophosphamide plus rituximab and prednisone were trialed without success. While on this regimen, she continued to deteriorate and was readmitted with fever and worsening shortness of breath. The plan was to exclude infection and consider a plasma cell inhibitor for refractory IgG4-RD. Repeat work up including bone marrow biopsy was notable for AML for which she is now being treated.

Discussion

Malignancy is associated with subsequent development of IgG4-RD. Some studies also suggest that prior IgG4-RD is associated with increased risk of developing malignancy. Our patient had cancer pre- and developed a different cancer post-IgG4-RD diagnosis. This case sheds light on the importance of cancer screening in IgG4-RD.