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Kidney Week

Abstract: PO1816

Rare Association of Monoclonal Gammopathy of Renal Significance with Acquired Angiodema

Session Information

Category: Glomerular Diseases

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Roy, Sasmit, Lynchburg General Hospital, Lynchburg, Virginia, United States
  • Bose, Subhasish, Lynchburg General Hospital, Lynchburg, Virginia, United States
Introduction

Acquired angioedema(AA) due to deficiency of C1 esterase inhibitor is also abbreviated as C1INH-AAE.This rare syndrome presents with recurrent angioedema episodes, without urticaria, and sometimes is associated with B-cell lymphoproliferative disorders.Kidney involvement is rare with AA.The monoclonal immunoglobulins are secreted by a nonmalignant B-cell or plasma cell clone,causing renal damage representing a group of disorders called monoclonal gammopathy of renal significance (MGRS).We present a rare association of these two entities.

Case Description

64 year old female patient came to the emergency department with complaints of 2 week duration waxing, waning maculopapular rashes in all extremities,chills,hoarseness of voice and lower extremity swelling.She had no family history of angiodema.Positive examination findings were rashes and bilateral pedal edema.With a normal baseline creatine, admission serum creatine was high at 2.4 mg/dl.Positive laboratory findings were very low complements level( C4> C3),low C1q level,high C1 esterase inhibitor level.Other immunological workup including serum,urine immunoelectrophoresis,kappa lambda ratio,serum immunofixation were normal.Kidney biopsy undertaken revealed monoclonal gammopathy–associated diffuse proliferative glomerulopathy.She responded well to steroids only and is in clinical remission with normal renal function.

Discussion

Paraproteinemia is characterized by clonal proliferation of B-cells and/or plasma cells resulting in overproduction of monoclonal proteins and can cause significant renal dysfunction.Paraprotein-induced renal disease can occur without malignancy,now termed as monoclonal gammopathy of renal significance.MGRS includes a wide spectrum of disorders like light/heavy chain deposition disease,C3 glomerulopathy, proliferative glomerulonephritis with monoclonal immunoglobulin deposition(PGNMID), and primary amyloidosis.MGRS necessitates strict monitoring,early interventions to prevent renal damage.Acquired angioedema generally presents with head and neck symptoms,mainly swollen upper airways, cheeks,and tongue.AA has low C1q levels unlike hereditary angiodema.Systemic manifestations are far common with AA than hereditary angioedema.No literature has shown an association of MGRS/PGNMID with AA and low complement levels.Further case studies needs to be done to discover any large-scale association of MGRS with AA.