Renal-limited AL amyloidosis – a diagnostic and management dilemma

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.

CD38 and Anti-CD38 Monoclonal Antibodies in AL Amyloidosis: Targeting Plasma Cells and beyond

“…Renal involvement, as identified by the detection of decreased estimated glomerular filtration rate (eGFR) or the presence of proteinuria, is found in approximately 70% of patients [7][8][9][10][11][12][13]. The risk of dialysis at two years is 11%-25% in patients with eitherdecreased eGFR orproteinuria, and up to 60%-75% in patients with both decreased eGFR and proteinuria [14,15].…”

Kidney-limited AL amyloidosis: a case report and review of the literature

“…In our review of the available literature, renallimited immunoglobulin light chain (AL) amyloidosis was only discussed in one other case report [3]. Thus, epidemiologic and demographic data is very limited.…”

“…She had no constitutional symptoms and no cardiac, respiratory, abdominal, or integumentary complaints. She also had no findings concerning for other system involvement, including heart failure, neuropathy, hepatic disease, or bleeding [3]. The diagnosis of AL amyloidosis, either local or systemic, is based on the clinical findings of the affected organs and positive histologic findings.…”

“…Kidney involvement is typical in systemic AL; however, other organs are almost always affected as well, and it is rare for the kidney to be the only organ in which amyloid is deposited. Kidney involvement in amyloidosis is associated with an increase in morbidity and mortality [1][2][3][4]. We report a patient who initially presented with significant bilateral lower extremity edema and proteinuria and was subsequently diagnosed with renal-limited amyloidosis.…”

A rare case of amyloid light-chain amyloidosis with bilateral perirenal hematoma shortly after initiation of peritoneal dialysis

“…Amyloid light-chain (AL) amyloidosis is a heterogeneous group of diseases characterized by extracellular deposition of immunoglobulin light chains [1]. These amyloid fibrils can deposit in various locations, including the heart and kidneys, and cause organ failure [2]. Amyloid-related kidney disease is a rare condition and a cause of end-stage renal disease (ESRD), the incidence of which appears to be rising in recent years [3].…”