Unit of Nephrology, Department of Medicine, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia, 43400, Serdang, Malaysia.
Published: November 2018
AI Article Synopsis
Article Abstract
Background: Amyloidosis 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 Presentation: We 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.
Conclusion: We 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.
Pathologic evaluation of the non-neoplastic renal parenchyma in tumor nephrectomy specimens is critical and can detect both renal-limited and systemic pathologies. We report the case of a 69-year-old Punjabi male who underwent cytoreductive nephrectomy for advanced renal cell carcinoma after immunotherapy. We detected clinically unexpected leukocyte chemotactic factor 2 (LECT2) amyloidosis during pathologic analysis of the surrounding non-neoplastic renal parenchyma, which was confirmed by mass spectrometry.
Amyloidosis involves the deposition of abnormal proteins in various tissues and results in progressive organ dysfunction, commonly affecting multiple organs. Two types of systemic amyloidosis are AA and AL; the former is associated with acute phase reactions and the latter is composed of light chain immunoglobulins. This disease commonly affects the kidneys and is evidenced by massive proteinuria.
- Kidney involvement is common in immunoglobulin-related amyloidosis (AIg), showing significant effects on quality of life, though it's generally less fatal than cardiac amyloidosis.
- The causes and mechanisms of kidney issues in AIg are still not fully understood, but there are models to help predict outcomes and responses to treatment.
- Patients with severe kidney damage face a poor prognosis, highlighting the need for improved therapies, while those with end-stage renal disease may benefit from kidney transplantation.
Background: Amyloidosis 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 Presentation: We present a case of a middle-aged gentleman who presented with persistent nephrotic syndrome with worsening renal function.
Gelsolin amyloidosis is a rare type of amyloidosis typically involving the cranial and peripheral nerves, but rarely the kidney. Here we report the clinical, kidney biopsy, and mass spectrometry findings in 12 cases of renal gelsolin amyloidosis. Of the 12 patients, five were men and seven were women with mean age at diagnosis of 63.
