Abstract: FR-PO040

Alemtuzumab Induced Anti-Glomerular Basement Membrane Disease with Histological Evidence of Perihilar Giant Cell Vasculitis

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Gately, Ryan P., Gold Coast University Hospital, City of Gold Coast, Queensland, Australia
  • San, Aye, Gold Coast University Hospital, Southport, Queensland, Australia
  • Stevenson, Tegan, Gold Coast Hospital, Ashmore, New South Wales, Australia
  • Nigam, Sonu, Pathology Queensland, Gold Coast, QLD, New South Wales, Australia
  • Kurtkoti, Jagadeesh, Gold Coast University Hospital, Southport, Queensland, Australia
  • Divi, Dakshinamurthy, Gold Coast University Hospital, Southport, Queensland, Australia
Background

To our knowledge there have been three published cases of alemtuzumab induced anti-glomerular basement membrane(GBM) disease to date.Here we report a case of fulminant anti-GBM disease that was exceptional given its unusual histological features

Methods

A 34 year-old Caucasian female presented with a one-week history of malaise and painless,frank hematuria.She had a background of relapsing-remitting multiple sclerosis(RRMS) previously treated with two courses of alemtuzumab(anti-CD52 monoclonal antibody) the most recent course completed six months prior.On admission creatinine was 2.26mg/dL and imaging did not reveal any nephrolithiasis or masses.Renal biopsy showed granulomatous vasculitis in a perihilar distribution with evidence of arteriolitis and glomerulitis with giant cells(figure 1).Immunofluorescence confirmed linear IGG staining along the GBM.ANCA titers were negative however anti-GBM antibodies were markedly elevated at 855 CU(<20).Plasma exchange,methylprednisolone and cyclophosphamide failed to prevent worsening of the patient's renal function and six days after admission haemodialysis was initiated.Cyclophosphamide was ceased due to intolerable side effects however two days subsequent to this the patient developed severe hemoptysis.Chest X-ray was suggestive of pulmonary hemorrhage.Daily plasma exchange and pulse methylprednisone were reinitiated resulting in attenuation of haemoptysis without improvement in renal function.The patient remains dialysis dependent with slowly falling antibody titers and it is hoped that she will ultimately be eligible for renal transplantation

Conclusion

Anti-GBM disease is an extremely rare but catastrophic complication of alemtuzumab therapy.With increasing use of alemtuzumab in RRMS we believe it is imperative to consider this condition in those exposed to this medication with worsening renal function or hematuria