Abstract: PUB165
Silent Deposits, Systemic Consequences: A Case of Amyloidosis with Renal Predominance
Session Information
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Kalaw, Gerome Owen Manguiat, Makati Medical Center, Makati, NCR, Philippines
- Sarmiento, Josierina, Makati Medical Center, Makati, NCR, Philippines
Introduction
Systemic immunoglobulin light chain (AL) amyloidosis is a rare plasma cell disorder increasingly recognized in the Philippines but often underdiagnosed due to overlap with common causes of chronic kidney disease such as diabetes and hypertension. It typically presents with nephrotic-range proteinuria and progressive renal dysfunction, while cardiac involvement markedly worsens prognosis. Kidney biopsy remains the gold standard for diagnosis. We report a rare case of stage IV systemic AL amyloidosis with renal and cardiac involvement in a Filipino patient.
Case Description
A 48-year-old male with heart failure with preserved ejection fraction, chronic kidney disease, type 2 diabetes mellitus, chronic hepatitis B infection, and liver cirrhosis presented with progressive edema, frothy urine, and fatigue for one year.
Discussion
Initial evaluation showed nephrotic-range proteinuria, worsening kidney function, and IgG lambda monoclonal gammopathy. The patient was initially lost to follow-up but later returned with worsening edema and fatigue. Repeat work-up revealed persistent renal dysfunction and massive proteinuria of 17.56 g/day. Kidney biopsy demonstrated Congo red-positive amyloid deposits with lambda light chain restriction, confirming AL amyloidosis, along with chronic glomerulosclerosis and tubulointerstitial damage. Further evaluation showed findings suggestive of cardiac amyloidosis, while bone marrow biopsy revealed 19.2% plasmacytosis, establishing the diagnosis of stage IV systemic AL amyloidosis with renal and cardiac involvement. The patient was started on Dara-CyBorD therapy, resulting in marked reduction in proteinuria from 17.56 g/day to 3.9 g/day, resolution of edema with significant overall clinical improvement. This case highlights the importance of early kidney biopsy in patients with unexplained nephrotic-range proteinuria and multisystem disease.
Congo red stain