POS1428 VALIDATION OF ANCA-ASSOCIATED VASCULITIS AS THE CAUSE OF END-STAGE RENAL DISEASE IN THE UNITED STATES RENAL DATA SYSTEM
Authors: C. Cook, H. Choi, Z. Wallace
Journal: Annals of the Rheumatic Diseases (2021)

Background: Glomerulonephritis and other renal manifestations are common in ANCA-associated vasculitis (AAV). Renal involvement in AAV is associated with adverse outcomes, including end-stage renal disease (ESRD) in up to 25% of patients (1). The United States Renal Data System (USRDS), a national registry of ESRD patients, represents a unique nationwide data source for studying AAV patients with ESRD. Prior research has assessed how often patients with ESRD attributed to AAV have biopsy-proven glomerulonephritis in USRDS (2), but the validity of the diagnosis of AAV as the cause of ESRD in the USRDS remains unknown. Objectives: We aim to validate the diagnosis of AAV as the primary cause of ESRD listed in USRDS. Methods: We identified all patients in the Mass General Brigham (MGB) healthcare system with a billing code for advanced chronic kidney disease or end-stage renal disease or procedure code for dialysis or renal transplantation. We identified all MGB patients fulfilling these criteria to records in the USRDS by name, sex, date of birth, and social security number. From this cohort of patients, we identified those with AAV or related diagnoses listed as the primary disease causing ESRD (ICD9: 446.0, 446.4 or ICD10: M31.3X, M31.7). Two authors reviewed medical records to collect information on whether or not a physician had diagnosed AAV, details of AAV history, renal and non-renal biopsies, and antineutrophil cytoplasmic antibody (ANCA) tests. Discrepancies were resolved through consensus. Details regarding initial ESRD onset date were obtained from the USRDS. To calculate the positive predictive value (PPV) for AAV as the primary cause of ESRD a definite physician diagnosis of AAV (a diagnosis confirmed by two physicians based on available data) in the MGB medical record was used as the gold standard. To calculate sensitivity, we linked the Partners (MGB) AAV Cohort to USRDS records using the same methods. A diagnosis code of AAV as the cause of ESRD was considered a true positive and a diagnosis code for other types of nephritis was considered a false negative. Results: We identified 89 USRDS records linked to MGB medical records in which the primary cause of ESRD was attributed to AAV. Of these, 85 were confirmed to be true cases of AAV after medical record review (PPV=96%) (Table 1). Among the cases classified as AAV, 84 (99%) had a positive ANCA test, which was predominantly MPO/P-ANCA (47, 55%); 36 (42%) had a renal biopsy, all of which were supportive of the diagnosis. The majority of cases were identified as AAV by ICD9 or 10 codes for Wegener’s granulomatosis (446.4 or M313.1). Within the Partners (MGB) AAV cohort linked to USRDS records, 33 (55%) of 60 identified cases had AAV listed as the cause of ESRD; in the remainder, ESRD was attributed to non-specific nephritis codes. Table 1. AAV and non-AAV patients in the USRDS with ESRD due to AAV (N=89) Physician-Diagnosed AAV (N=85) ANCA type n (%) 84 (98.8) MPO/P-ANCA+ 47 (55.3) PR3/C-ANCA+ 33 (38.8) Renal biopsy n (%) 36 (42.4) Pauci-Immune Glomerulonephritis n (%) 16 (44%) Non-renal biopsy n (%) Yes 10 (11.8) No 74 (87.1) Years from AAV diagnosis to ESRD median [IQR] 1 [0, 6] Principal diagnosis code (ICD9/ICD10) n (%) Wegener’s granulomatosis (446.4, 446.4B, or M313.1) 81 (95.3) Conclusion: We found that the diagnosis of AAV as the primary cause of ESRD in the USRD had a high PPV, suggesting accurate classification of ESRD due to AAV in the USRDS, but that sensitivity was moderate. These findings support the past and future use of the USRDS for research with ESRD attributed to AAV. References: [1]Moiseev S, Novikov P, Jayne D, Mukhin N. End-stage renal disease in ANCA-associated vasculitis. Nephrol Dial Transplant. 2017;32(2):248-53. [2]Layton JB, Hogan SL, Jennette CE, Kenderes B, Krisher J, Jennette JC, et al. Discrepancy between Medical Evidence Form 2728 and renal biopsy for glomerular diseases. Clin J Am Soc Nephrol. 2010;5(11):2046-52. Disclosure of Interests: None declared