Recurrent dense deposit disease after renal transplantation: an emerging role for complementary therapies.
Dense deposit disease is a rare glomerulonephritis caused by uncontrolled stimulation of the alternative complement pathway. Allograft survival after kidney transplantation is significantly reduced by the high rate of disease recurrence. No therapeutic interventions have consistently improved outcomes for patients with primary or recurrent disease. This is the first reported case of recurrent dense deposit disease being managed with eculizumab. Within 4 weeks of renal transplantation, deteriorating graft function and increasing proteinuria were evident. A transplant biopsy confirmed the diagnosis of recurrent dense deposit disease. Eculizumab was considered after the failure of corticosteroid, rituximab and plasmapheresis to attenuate the rate of decline in allograft function. There was a marked clinical and biochemical response following the administration of eculizumab. This case provides the first evidence that eculizumab may have a place in the management of crescentic dense deposit disease. More information is necessary to clarify the effectiveness and role of eculizumab in dense deposit disease but the response in this patient was encouraging. The results of clinical trials of eculizumab in this condition are eagerly awaited.
Regional Nephrology Unit, Belfast City Hospital, Belfast, Northern Ireland. jamccaughan@doctors.org.uk
J A
JA
McCaughan
D M
DM
O'Rourke
A E
AE
Courtney
D000328:Adult; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D003170:Complement Pathway, Alternative / Q000187:drug effects*; D005260:Female; D005921:Glomerulonephritis / Q000188:drug therapy / Q000209:etiology / Q000473:pathology; D015432:Glomerulonephritis, Membranoproliferative / Q000188:drug therapy* / Q000209:etiology* / Q000473:pathology; D006801:Humans; D016030:Kidney Transplantation / Q000009:adverse effects*; D010956:Plasmapheresis; D011379:Prognosis; D055502:Secondary Prevention; D014184:Transplantation, Homologous
D061067:Antibodies, Monoclonal, Humanized; C481642:eculizumab