18 May 2023 : Case report
[In Press] A 78-Year-Old Man with Chronic Kidney Disease and Monoclonal Gammopathy Who Developed Post-Transplant C3 Glomerulopathy – Recurrence or De Novo? A Case Report and Literature Review
Challenging differential diagnosis, Rare coexistence of disease or pathologyMaría Carmen Ruiz-Fuentes 1ABEF, Mercedes Caba-Molina 2D, Aurora Polo-Moyano1B, Magdalena Palomares-Bayo1B, Pilar Galindo-Sacristan1B, Carmen De Gracia-Guindo 1E
Am J Case Rep In Press; DOI: 10.12659/AJCR.939726
Available online: 2023-05-18, In Press, Corrected Proof
Publication in the "In-Press" formula aims at speeding up the public availability of the pending manuscript while waiting for the final publication. The assigned DOI number is active and citable. The availability of the article in the Medline, PubMed and PMC databases as well as Web of Science will be obtained after the final publication according to the journal schedule
The incidence of glomerular disease recurrence in kidney transplant patients varies according to type of glomerulopathy; therefore, it is important to know the primary chronic kidney disease etiology. C3 glomerulopathy (C3G) is characterized by deposits of C3 in immunofluorescence and its pathogeny is based on the dysregulation of the alternative complement pathway. C3G has a high recurrence rate and, given its low prevalence, only case series have been published. A higher rate of recurrence and a more aggressive course have been described in association with monoclonal gammopathy (MG).
We describe the case of a 78-year-old man with chronic kidney disease of unknown etiology (no significant proteinuria) and monoclonal IgGl gammopathy with low risk of progression, who received a kidney transplant, presenting accelerated deterioration of kidney function. Histopathology showed predominant C3 deposits in immunofluorescence, compatible with C3 glomerulonephritis (C3GN). He was treated with eculizumab during 4 weeks while the study was completed. The response to treatment was not favorable and the patient remained in the dialysis program.
Further studies are needed to explain the pathogenic mechanisms of complement alternative pathway dysregulation mediated by monoclonal component in patients with C3GN and MG. Patients older than 50 years who are on a waiting list for kidney transplantation should have an MG detection study. The information provided to patients with MG on a waiting list for kidney transplantation should include not only the possibility of hematologic progression but also the recurrence/de novo appearance of associated kidney pathology.
Keywords: Kidney Transplantation; Paraproteinemias; Recurrence
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