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Abstract: PO1714

Diagnostic Delay and the Clinical Prodrome in US Adults with Systemic Light Chain (AL) Amyloidosis with Renal Involvement

Session Information

Category: Glomerular Diseases

  • 1201 Glomerular Diseases: Fibrosis and Extracellular Matrix

Authors

  • Hester, Laura, Janssen Research & Development LLC, Titusville, New Jersey, United States
  • Gifkins, Dina, Janssen Research & Development LLC, Titusville, New Jersey, United States
  • Bellew, Kevin, Janssen Research & Development LLC, Spring House, Pennsylvania, United States
  • Vermeulen, Jessica, Janssen Research & Development LLC, Leiden, Netherlands
  • Schecter, Jordan, Janssen Research & Development, LLC, Raritan, New Jersey, United States
  • Dishy, Victor, Janssen Research & Development, LLC, Raritan, New Jersey, United States
  • Weiss, Brendan, Janssen Research & Development LLC, Spring House, Pennsylvania, United States
Background

Early therapy for AL can reverse renal impairment, but AL diagnosis (dx) is often delayed. We report the first population-level study of the diagnostic delay and prodrome in systemic AL patients (pts) with prior signs/symptoms (S/Sx) of renal impairment.

Methods

Pts with renal S/Sx at AL dx were identified in the US Optum Clinformatics® claims data since June 2001. AL was defined as >1 inpatient or >2 outpatient AL codes, followed by >1 anti-plasma cell therapy in 2 yrs. Renal S/Sx were defined as ≥1 prior dx code for stage 1-3 chronic kidney disease (CKD), renal failure/ESRD, nephrotic syndrome, acute kidney injury, or proteinuria. We described prevalence and overlap of S/Sx and time from first S/Sx to AL dx in renal AL pts. Kaplan-Meier estimates and log-rank tests compared time to AL diagnosis by prior monoclonal gammopathy (MG).

Results

Of 870 renal AL pts (67% of AL pts), 70% had CKD, 46% had renal failure, 58% had acute renal failure, 29% had nephrotic syndrome, and 61% had proteinuria by AL dx. Median time since first renal S/Sx and AL dx was 196 days, with a median of 205 days since CKD dx and 23 days from first nephrotic syndrome dx (Figure). Among renal AL pts, 89% had cardiac S/Sx, 67% had neurologic S/Sx, and 57% had ≥3 systems involved. Median time from first nephrology visit for renal S/Sx and AL dx was 67 days (6 visits). AL dx was earlier for pts with prior MG than without (median 83 vs 210 days, P=0.002).

Conclusion

The median time to AL dx after the first renal S/Sx was 196 days and 67 days after the first nephrology visit. The presence of a prior MG shortened the time to AL dx.

Funding

  • Commercial Support