Incidence and clinical outcome of renal amyloidosis: a retrospective study.

Saudi J Kidney Dis Transpl

Department of Nephrology, Theodor Bilharz Research Institute, Cairo, Egypt.

Published: September 2013

The kidneys are affected in almost all patients with amyloid A in secondary amyloidosis (AA) amyloidosis but less frequently in immunoglobulin light chains in primary systemic amyloidosis (AL) amyloidosis. In this study, we present the incidence, etiology, clinical manifestations, biochemical features and clinical course of renal amyloidosis. We conducted a retrospective study on a group of 40 cases with renal biopsy-proven amyloidosis. They constituted 2.5% of the total cases of renal biopsies performed in the Theodor Bilharz Research Institute, Cairo, Egypt, during the period from February 2003 to May 2009. The mean age (30 males, ten females) was 36.51 ± 10.32 years. Thirty-two of the cases had secondary AA amyloidosis and eight cases had primary AL amyloidosis. The causes of secondary amyloidosis were as follows: 12 (30%) familial Mediterranean fever (FMF), eight (20%) pulmonary tuberculosis, four (10%) chronic osteomyelitis, four (10%) bronchiectasis, three (7%) rheumatoid arthritis and one (2%) rheumatic heart disease. The eight cases of primary AL amyloidosis comprised of five cases that were associated with myloma (13%) and three (8%) cases that were idiopathic. Among the 23 patients with AA amyloidosis, after six months of treatment with colchicine, the proteinuria improved, serum albumin level increased and edema disappeared in 13 patients. In four cases of AA amyloidosis who were clinically and biochemically normal after cholchicine therapy, a second renal biopsy disclosed decreased amyloid deposition compared with the first biopsy. In the three renal transplanted patients who had amyloidosis secondary to FMF and were treated with colchicines, AA amyloidosis did not recur in the transplanted kidney. It might be possible that in AL amyloidosis, treatment with methotrexate, melphalan and prednisolone may improve survival. The incidence of renal amyloidosis is increasing and colchicine can be used in secondary amyloidosis as it may have an effect on reducing the production of the amyloid precursor proteins and in reducing proteinuria.

Download full-text PDF

Source
http://dx.doi.org/10.4103/1319-2442.118094DOI Listing

Publication Analysis

Top Keywords

amyloidosis
18
secondary amyloidosis
16
renal amyloidosis
12
retrospective study
8
amyloidosis amyloidosis
8
cases
8
cases renal
8
cases primary
8
primary amyloidosis
8
amyloidosis secondary
8

Similar Publications

Background: The ongoing post-COVID-19 syndrome (PCS) epidemic, causing complications of diverse etiology, necessitates the search for new diagnostic markers and the development of widely accessible methods for their detection. This would enable the prognosis of PCS progression and faster implementation of targeted treatments. One potential marker is neutrophil elastase (NE), whose elevated levels in the blood during PCS may result from organ damage caused by increased secretion of severe inflammatory mediators or amyloidosis resulting from the interaction of NE with SARS-CoV-2.

View Article and Find Full Text PDF

Transthyretin cardiac amyloidosis (ATTR-CA) involves the buildup of transthyretin protein in the heart muscle in the form of amyloid fibrils, which can affect heart structure and function. Common ECG findings of ATTR-CA include low QRS voltage and a pseudo-myocardial infarction (MI) pattern, defined as pathological Q waves or QS complexes in two consecutive leads without a history of MI or echocardiographic evidence of akinetic areas. Here, we present a case of ATTR-CA in a very elderly patient, in whom pathological Q waves on ECG were true indicators of a prior inferior MI.

View Article and Find Full Text PDF

Objective: This study aimed to assess the diagnostic accuracy of a novel semiautomated method to calculate relative myocardial wall uptake (RCU) of 99mTechnetium-pyrophosphate SPECT/CT in suspected cardiac transthyretin amyloidosis (ATTR).

Methods: Prospective analysis of 108 participants with suspected ATTR. Using novel software, we quantified radiopharmaceutical uptake in the wall of each chamber of the heart at 3 h, calculating RCU for combined ventricular (RCU-V) and whole-heart (RCU-W) measures as a ratio over physiological blood-pool activity.

View Article and Find Full Text PDF

Characterization of left atrial strain in left ventricular hypertrophy: A study of Fabry disease, sarcomeric hypertrophic cardiomyopathy and cardiac amyloidosis.

Arch Cardiovasc Dis

February 2025

Bordeaux University Hospital, 33000 Bordeaux, France; University of Bordeaux, 33000 Bordeaux, France; CIC-P 1401, 33600 Bordeaux-Pessac, France; Inserm 1045, 33600 Pessac, France.

Background: Patients with left ventricular hypertrophy (LVH) often maintain preserved left ventricular ejection fraction in the early stages of the disease. There is a need to identify simple and reliable variables beyond left ventricular ejection fraction to recognize those at risk of developing adverse clinical outcomes.

Aims: To examine left atrial (LA) strain in patients with hypertrophic cardiomyopathy (HCM), cardiac amyloidosis (CA) and Fabry disease (FD), pathologies known to cause LVH, and the relationship between LA strain and adverse clinical outcomes.

View Article and Find Full Text PDF

The transthyretin (TTR) tetramer, assembled as a dimer of dimers, transports thyroxine and retinol binding protein in blood plasma and cerebrospinal fluid. Aggregation of wild type (WT) or pathogenic variant TTR leads to transthyretin amyloidosis, which is associated with neurodegenerative and cardiac disease. The trigger for TTR aggregation under physiological conditions is unknown.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!