Status of initial treatment for rapidly progressive glomerulonephritis in Japan: analysis of a personal clinical records database

Background: As a joint project with the Ministry of Health, Labour and Welfare (MHLW), the Research Group on Intractable Renal Diseases is examining the feasibility of utilizing its personal clinical records database. We examine the validity of the initial-treatment data from the personal clinical records of patients with new-onset rapidly progressive glomerulonephritis (RPGN). Methods: Personal clinical records for patients with either RPGN or anti-glomerular basement membrane (GBM) antibody nephritis were used. The data from 454 newly enrolled RPGN patients were compiled for analysis in 2 cohort studies (CS1 for all case analysis and CS2 for selective analysis of new-onset cases). Results: In CS1, the serotypes of the 362 registered RPGN cases included 200 myeloperoxidase (MPO)-ANCA-positive, 98 anti-GBM-positive, and 9 proteinase-3 (PR3)-ANCA-positive cases, etc. CS2 included 96 of the MPO-ANCA-positive RPGN and 55 of the anti-GBM antibody-positive RPGN cases. For the initial treatment of MPO-ANCA-positive RPGN, the rates of glucocorticoid (GC) and GC pulse treatment were similar between the personal clinical records database and the nationwide questionnaire survey, but the rates of intravenous cyclophosphamide (CY) or rituximab were statistically significant lower in the personal clinical records database. For the initial treatment of anti-GBM antibody-positive RPGN, the rate of plasma exchange was similar between the two databases, but the rates of GC and per os CY tended to be lower in the personal clinical records database, although not statistically significant. Conclusion: Clear differences in initial treatment for new-onset RPGN patients were found between a personal clinical records database and another reported database.