Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits Is Not Only a Disease in Elderly Patients but Also a Disease in Young Patients

Introduction: Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is a special category of disease within the group of disorders, monoclonal gammopathy of renal significance (MGRS). Though it is an uncommon disorder, PGNMID is increasingly recognized in the recent times. Though PGNMID can occur at any age, the average age of onset is usually around 55 years. Case Description: We report a young 31 year old female patient with history of resistant hypertension, type 2 diabetes mellitus and morbid obesity admitted to the hopsital with acute kidney injury and and noted to be having nephrotic syndrome with urine protein of 21 gm/24 hour, from biopsy-proven PGNMID with IgG3 kappa light chain deposistion. The patient failed treatments with Bortezomib, Lenalidomide, Dexamethasone (VRd) and daratumumab, bortezomib and dexamethasone. She had partial response to cyclophosphamide, bortezomib and dexamethasone (CyBorD) along with daratumamab with eventual worsening of proteinuria again. During the course of her disease, she continued to have significant problems with resistant hypertension as well as cardiac problems including coronary artery disease requiring 4-vessel bypass grafting. Unfortunately, despite multiple lines of chemoterapeutic/immunosuppressive agents the patient eventually progressed to end stage renal disease requiring initiation of hemodialysis. Discussion: Though MGRS/PGNMID are rare causes of renal dysfunction, early recognition and early initiation of proper management of these patients is of utmost importance for improved renal outcomes. Unfortunately, due to unclear pathogenic mechanisms effective treatment options remains limited and usually treatment regimens are aimed at clone-directed therapies in patients with detectable clones as well as without detectable clones where the treatment is directed at potential hypotehtical clones. Moreover, our case also demonstrates that PGNMID can happen even in younger patients and may be associated with more aggressive disease with limited response to the traditional chemotherapeutic agents.