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Renal-limited AL amyloidosis – a diagnostic and management dilemma

Authors
  • Fuah, Kar Wah1
  • Lim, Christopher Thiam Seong2
  • 1 Hospital Tengku Ampuan Afzan, Department of Medicine, Kuantan, Malaysia , Kuantan (Malaysia)
  • 2 Universiti Putra Malaysia, Unit of Nephrology, Department of Medicine, Faculty of Medicine and Health Sciences, Serdang, 43400, Malaysia , Serdang (Malaysia)
Type
Published Article
Journal
BMC Nephrology
Publisher
Springer (Biomed Central Ltd.)
Publication Date
Nov 06, 2018
Volume
19
Issue
1
Identifiers
DOI: 10.1186/s12882-018-1118-8
Source
Springer Nature
Keywords
License
Green

Abstract

BackgroundAmyloidosis is a disorder caused by extracellular tissue deposition of insoluble fibrils which may result in a wide spectrum of symptoms depending upon their types, sites and amount of deposition. Amyloidosis can be divided into either systemic or localized disease.Case presentationWe present a case of a middle-aged gentleman who presented with persistent nephrotic syndrome with worsening renal function. Repeated renal biopsies showed the presence of renal-limited AL amyloidosis. Systemic amyloidosis workup was unremarkable apart from a slightly raised band of IgG lambda level with no associated immunoparesis. The nephrotic syndrome and renal histology did not improve over a 3-year period despite being given two courses of chemotherapies.ConclusionWe hope that early recognition of this unusual localised presentation of renal- limited AL Amyloidosis and its poor response to conventional treatment can alert the nephrologist to the potential existence of this rare condition.

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