Dr Amy Chew1,3, Dr Nichita Gavrilescu1, Dr Peggy Teh1, Dr Alan Pham2, Dr Kate Robson1
1Department of Nephrology, Alfred Health, Melbourne, Australia, 2Department of Anatomical Pathology, Alfred Health, Melbourne, Australia, 3Department of Endocrinology, Monash Health, Clayton, Australia
Biography:
Amy currently works as the diabetes and women's reproductive health fellow at Monash Health, and is pursuing endocrinology advanced training. She continues to have research affiliations with Alfred Health, with whom she completed her basic physicians training. She is passionate about the care of patients with shared endocrine and renal disease.
Background:
Anti-glomerular basement membrane (anti-GBM) disease is a rare autoimmune disorder characterised by pathogenic circulating autoantibodies against the glomerular basement membrane. Standard of care generally includes glucocorticoids, plasma exchange, and cyclophosphamide to rapidly reduce pathogenic autoantibody levels. Rituximab has been described as a viable option for refractory disease.
This is the first published case report of a transgender man with anti-GBM disease. Despite a number of poor prognostic factors, the patient achieved renal recovery using rituximab in addition to standard care.
Case Report:
A 21-year-old transgender man (female sex) presented with bilateral flank pain and oliguria. Investigations revealed an initial creatinine of 374 µmol/L which doubled to 619 µmol/L by day 3 of admission. Anti-GBM titre was strongly positive at 688 U/mL and MPO antibody titre was low positive. Renal biopsy revealed diffuse, crescentic glomerulonephritis with 23 of 32 (71%) glomeruli containing cellular crescents and immunohistochemistry consistent with anti-GBM glomerulonephritis.
The patient was treated with plasma exchange (total of 37 exchanges), 3g of IV methylprednisolone induction in divided doses followed by 1mg/kg oral prednisolone daily, and 100mg oral cyclophosphamide daily. The patient commenced haemodialysis on day 10. Despite an initial decline, the anti-GBM titre increased to 127 U/mL 21 days after first plasma exchange. The patient was then given 1g IV rituximab in 2 doses, 14 days apart. The anti-GBM titre steadily declined and became undetectable within 1 month. By 6 months, there was evidence of renal recovery and the patient ceased dialysis. At 2 years, the patient’s creatinine is 157 and eGFR 53.
Conclusions:
This case report adds to the existing evidence supporting the use of rituximab for treatment of refractory anti-GBM disease.