Abstract: SA-PO255
A Terrible Triad: A Case of Anti-GBM, Membranous, and Waldenstrom’s
Session Information
- Trainee Case Reports - V
October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Gunasekaran, Niranjan, Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States
- Kanwar, Yashpal S., Northwestern University Medical School, Chicago, Illinois, United States
- Eng, Eudora, Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States
Introduction
Anti-GBM disease is a rare disease with a very rapid and destructive course. Much of the knowledge gained has been through case reports and case series. Here, we describe a case of anti-GBM complicated by many factors and the treatment regimen used.
Case Description
A 52y/o man with cryoglobulinemia manifested only by pruritus presented with 12 days of fever, malaise, night sweats and anorexia. Admission creatinine was 1.7, from a baseline 3 days prior of 0.9. Creatinine rapidly worsened and he became oliguric by the 3rd hospital day. Renal biopsy was done on day 4. A dialysis catheter was placed on day 5, anti-GBM resulted at 7.6 and light and IF of renal biopsy showed membranous nephropathy, anti-GBM with necrotizing crescentic GN and severe tubulointerstitial disease. He was pulsed with steroids, started on plasmapheresis, dialysis, and IV cyclophosphamide. Because of the membranous nephropathy, history of paraproteinemia, increased IgM lambda and lymphadenopathy, bone marrow biopsy was done, showing Waldenstrom’s macroglobulinemia. However, due to prior reports that Waldenstrom’s is linked to a number of autoimmune diseases including cases of RPGN, he was switched to rituximab, with increasing urine volume up to 2L/day, and decreasing pre-dialysis BUN and creatinine. However, after 6 of 8 cycles of ritumab, anti-GBM titer rebounded to 144. He was re-pulsed with steroids, restarted on cyclophosphamide and underwent daily plasmapheresis for 3 weeks until the GBM titer was zero. Despite preserved urine volume, he remains on hemodialysis.
Discussion
Although there are reports of simultaneous membranous and anti-GBM, or Waldenstrom’s and membranous, we could find only one case report of anti-GBM disese and Waldenstrom’s, and none with all three. Rituximab, an accepted treatment for Waldenstrom’s and membranous nephropathy has been used in a few patients with anti-GBM, mostly those who failed cyclophosphamide. Despite an initial response to therapy, he relapsed after completion of plasmapheresis, while still receiving rituximab. We suspect membranous nephropathy secondary to Waldenstrom’s. PLA2R was negative but obtained after plasmapheresis/dialysis. We speculate uncovering of the Goodpasture’s epitope, secondary to another underlying process.