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Kidney Week

Abstract: PO1510

Rituximab for Membranous Nephropathy in a Patient with Sjögren Syndrome and Mixed-Connective Tissue Disease

Session Information

Category: Glomerular Diseases

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Pollock, Joshua D., Madigan Army Medical Center, Tacoma, Washington, United States
  • Khayat, Maurice I., Madigan Army Medical Center, Tacoma, Washington, United States
Introduction

Membranous nephropathy (MN), a cause of nephrotic syndrome, is characterized by the deposition of immune complexes in the glomerular basement membrane with resultant subepithelial “spikes” visualized under light microscopy. Although often a primary disease process, several secondary etiologies exist, including Sjögren’s syndrome (SS) and mixed-connective tissue disease (MCTD). Treatment typically targets the underlying condition. Despite rituximab’s demonstrated efficacy in MN, MCTD and SS, case reports for specific treatment of MN secondary to these conditions have primarily described regimens of systemic corticosteroids with or without cyclophosphamide.

Case Description

A 54-year-old-male was diagnosed with MN on renal biopsy in 2001. Despite features suggestive of a secondary etiology (tubulo-reticular structures, mild cellular increase by light microscopy, mesangial deposits, and irregular distribution of deposits by EM), both his initial presentation and subsequent relapse in 2010 responded to antiproteinuric therapy alone. Following treatment-resistent relapse, cyclophosphamide and prednisone were initiated in 2019 without improvement. A secondary evaluation was significant for strongly positive anti-ribonucleoprotein and anti-SSA antibodies with negative serologies for systemic lupus erythematosus. The patient reported only mild polyarthralgia and sicca symptoms. Following rheumatology consultation and salivary gland biopsy the patient was diagnosed with MCTD and SS. Rituximab 1000 mg on day 0 and 14 was administered, with partial remission noted 4 months following therapy. Complete remission was achieved 8 months following therapy and has been sustained for 13 months. His extrarenal symptoms of MCTD and SS have also resolved.

Discussion

Rituximab is a well-described treatment for primary MN, MCTD and SS. However, there is a paucity of literature evaluating its use in MN as the specific renal manifestation of MCTD and SS. This case illustrates the potential role for rituximab as an effective treatment for membranous nephropathy secondary to SS and MCTD when deciding on immunomodulatory therapy.

The views expressed in this abstract are those of the authors and do not reflect the official policy of the Department of Army/Navy/Air Force, Department of Defense (DoD), or the U.S. Government.