Published April 1, 2022 | Version v1
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278. Risk of Recurrent Disease in Vasculitis Patients Following Renal Transplantation. Are serological markers of value?

  • 1. 1Belfast City Hospital, Belfast Trust, Belfast, United Kingdom

Description

Background: Vasculitis is an important cause of end stage renal disease (ESRD), and many patients proceed to renal transplantation. However, recurrent disease following transplantation can cause graft failure. The value of serological markers in predicting recurrent disease in this group is unclear. We considered this in vasculitis patients following renal transplantation in Northern Ireland (NI).

 

Methods: Transplant patients with a primary renal disease of vasculitis, including polyarteritis nodosa, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and ANCA-associated vasculitis (AAV), were identified using the Northern Ireland Kidney Transplant database. Additional information was collected using the NI Electronic Care Record (NIECR) and regional renal-specific electronic system (eMED). Patients from 2010 to present were identified.

 

Results: There were 26 transplants in 25 patients. Table 1 summarises relevant patient and donor information. One patient was lost to follow up due to emigration, for the remaining 24 patients the median follow up was 60 months from first transplant. At time of transplant, ANCA titres were detectable in 9 cases and had normalised in 12 (having previously been positive). Titres were unavailable in 5 cases. ANCA titres were checked in 18 cases (73.1%) as part of transplant follow-up. Of these; 4 (22.2%) were persistently positive, 2 (11.1%) fluctuated between positive and negative, 7 (38.9%) went from positive to negative, 3 (16.7%) stayed negative, and in 2 (11.1%) the titre became positive having been negative. Clinically significant recurrent disease was evident in 3 cases (11.5%). The first patient had pauci-immune crescentic glomerulonephritis on graft biopsy (prompted by acute graft dysfunction) at 41 months from transplant, in association with an elevated MPO of 2.6 AI (normal <0.9 AI). This prompted revision of his primary disease diagnosis of IgA nephropathy to AAV. This patient returned to dialysis but received a second transplant with no current evidence of recurrent disease (29 months from time of transplant). MPO has been consistently normal following second transplant. The second patient had focal segmental necrotising glomerulonephritis on biopsy (prompted by haematuria and respiratory symptoms) 47 months from transplant. MPO had been persistently positive, but subsequent to treatment for recurrent disease reduced significantly. Non-visible haematuria resolved and respiratory symptoms settled. Renal function remains excellent however MPO remains positive at low level. The third patient had necrotizing granulomatous inflammation on cutaneous biopsy, consistent with extra-renal manifestation of GPA, 78 months from transplant. Serology demonstrated a marginally elevated PR3 of 1.3 AI (normal <0.9 AI). PR3 had been significantly elevated prior to transplant but had not been checked in the interim. Renal function remains stable. There was no histological evidence of recurrent disease reported in the remaining 23 cases (88.5%). Neither ANCA positivity nor MPO/PR3 level appeared to be associated with clinical evidence of recurrent disease.

 

Conclusions: Renal transplantation is appropriate for patients with ESRD due to vasculitis with good outcomes. Recurrent renal disease is uncommon but can lead to graft failure. Serological markers alone are not a reliable marker of disease activity but may be of use when accompanied by other clinical features of recurrent vasculitis.  

 

Disclosures: None.

 

Table 1. Recipient and donor characteristics

 

 

 

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