Rowland, TA;
Gillmore, J;
Bhandari, S;
(2019)
Regression of chronic kidney disease in a patient with AL amyloidosis: A case report.
Clinical Nephrology
, 91
(5)
pp. 317-320.
10.5414/CN109676.
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Abstract
Background: AL amyloidosis is a disease that causes significant end-organ damage via deposition of insoluble amyloid fibrils, which cause disruption of normal tissue architecture and function. The mainstay of current treatment employs various chemotherapy regimens, all of which aim to suppress the underlying plasma cell dyscrasia and reduce the production of amyloidogenic precursor proteins. Renal disease is found in between 50 and 80% of sufferers and is often both progressive and irreversible, ultimately leading to end-stage renal failure and death. Recovery from advanced chronic kidney disease is uncommon, however, this case demonstrates that it is possible with ongoing therapy. Case presentation: We report the case of a 53-year-old lady with amyloid-induced nephrotic syndrome who underwent intensive chemotherapy. Initially, this lady experienced the gradual decline in renal function typical of the natural history of this disease; her renal function indicated an impending need for dialysis. However, soon after, evidence of recovery became apparent on serum amyloid protein component scintigraphy, measurement of serum free light chains, and measurement of renal parameters. Ultimately, her renal disease was completely resolved. Conclusion: This case shows that renal recovery in AL amyloidosis may be possible and also highlights the importance of continued therapy in order to achieve clonal response and recovery of organ function.
| Type: | Article |
|---|---|
| Title: | Regression of chronic kidney disease in a patient with AL amyloidosis: A case report |
| DOI: | 10.5414/CN109676 |
| Publisher version: | https://dx.doi.org/10.5414/CN109676 |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. |
| Keywords: | Science & Technology, Life Sciences & Biomedicine, Urology & Nephrology, AL amyloidosis, renal amyloidosis, amyloidosis, chronic kidney disease (CKD), nephrotic syndrome, amyloid-induced nephrotic syndrome |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10114813 |
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