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Kidney Week

Abstract: PO1499

PLAR2-Positive Membranous Nephropathy: A Renal Manifestation of Scleromyxedema

Session Information

Category: Glomerular Diseases

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Hlepas, Alexander, Rush University Medical Center, Chicago, Illinois, United States
  • Gashti, Casey N., Rush University Medical Center, Chicago, Illinois, United States
  • Rodby, Roger A., Rush University Medical Center, Chicago, Illinois, United States

Group or Team Name

  • Rush Nephrology
Introduction

Scleromyxedema (SMX) is a rare primary cutaneous mucinosis that usually occurs in association with a monoclonal gammopathy. Systemic manifestations may involve the musculoskeletal, cardiovascular, gastrointestinal, pulmonary, nervous and renal systems and may lead to significant morbidity and mortality. Renal manifestation has been reported, but histopathologic description of renal involvement has been vague and histopathologic classification has not been implemented. Therapeutic management of the renal disease has included plasmapheresis and cyclophosphamide, but the roles of each of these is not determined.

Case Description

We report a case of a 53-year-old male with SMX and monoclonal gammopathy who developed renal manifestation of sclerodermoid disease. His skin manifestations of SMX had been previously controlled with intravenous immunoglobulins (IVIG) until he developed presumed central nervous system involvement manifestating as an isolated seizure. He failed a series of treatments including lenalidomide, melphalan, and bortezomib. He subsequently underwent an autologous stem cell transplant with initial improvement in cutaneous manifestations for 6 months but experienced a relapse thereafter. In addition, he developed acute kidney injury associated with minimal proteinuria (<1000 mg). A kidney biopsy demonstrated thrombotic microangiopathy (TMA) and tissue phospholipase A2 receptor antibody (PLAR2) positive membranous nephropathy. Ravulizumab was instituted but had to be abandoned as the patient was hospitalized on two occasions with fever and culture negative pulmonary infiltrates after each infusion. Alternatively, he was treated with intravenous cyclophosphamide, oral prednisone and plasmapheresis with improvement in skin disease and stabilization of proteinuria and renal function.

Discussion

Renal manifestations of SMX are poorly defined and treatment is unknown. Membranous nephropathy has been rarely reported in SMX, but PLA2R status has not been available. Most cases focus on treatment of the underlying monoclonal gammopathy but outcomes with conventional therapy remain poor. We report a patient with SMX who developed TMA and PLA2R positive membranous nephropathy who failed clone directed therapy but was successfully treated with IV Cytoxan (an accepted option for PLA2R+ MGN), steroids and plasmapheresis.