Pattern of renal amyloidosis in South Africa

BACKGROUND: Amyloidosis is a heterogeneous group of diseases with multi-organ involvement and different clinical manifestations; one of them is nephrotic syndrome. Amyloidosis results from the extracellular deposit of fibrillar proteins that form the amyloid substance; amyloidogenic proteins resistant to proteolysis infiltrate tissues extracellularly and are capable of severely altering their structure and function. Amyloid deposits in the renal parenchyma can be observed in the mesangium, capillary wall, interstitium, and arterial vessels. Its diagnosis requires a biopsy of the comprised organs. CASE REPORT: 53-year-old male patient, presented with the incidental finding of microalbuminuria. The only clinical manifestations were mild lower limbs edema and foamy urine. Laboratory tests showed: hypoalbuminemia, hypercholesterolemia, hypertriglyceridemia and nephrotic- range proteinuria. With pure nephrotic syndrome diagnosis a renal biopsy was performed, leading to the histopathological diagnosis of amyloidosis. EVOLUTION: Nephroprotective measures were instituted and the extent of systemic involvement was studied, we diagnosed congestive heart failure and amyloid deposition in the digestive system. The patient initially received treatment based on Cyclophosphamide, Bortezomib and dexamethasone, with no renal or haematological response after four cycles of treatment, so treatment was changed to Bortezomib and Bendamustine. CONCLUSION: It is vitally important to have a high index of suspicion and to make an early diagnosis of the disease through a biopsy with Congo red staining. The treatment depends on the type of amyloidosis, its opportune start allows to increase survival rate, as well as, to avoid the deposit of amyloid in other organs.

show abstract

“…La infección por VIH se observó en sólo dos (10%) de los 20 casos de AA. La mediana de supervivencia después del diagnóstico fue de 2 meses [7].…”

Section: Discussionunclassified

Caso Clínico: Síndrome nefrótico como presentación de amiloidosis AL, una asociación poco frecuente en nuestro medio

Ordoñez¹,

Cabrera²,

Montero³

2022

Rev Med HJCA

View full text Add to dashboard Cite

show abstract

“…AL amyloidosis is the most frequent subtype of renal amyloidosis worldwide, however, epidemiological differences are observed among different countries. AL amyloidosis is more prevalent in developed countries, while AA amyloidosis prevails in developing countries due to the high prevalence of infectious diseases ( 15 ). In a Brazilian study including 110 cases of biopsy-proven systemic amyloidosis, 60 patients had renal involvement and immunoglobulin-related amyloidosis was predominant ( 16 ).…”

Section: Renal Amyloidosismentioning

confidence: 99%

“…The distribution of AA amyloidosis varies worldwide and, together with AL, constitute the main causes of renal amyloidosis in developing countries ( 15 ). A reduction in AA incidence has been observed in recent decades, reflecting the therapeutic advances and improvements in treating chronic inflammatory diseases ( 13 , 15 ). AA patients usually have inadequately controlled chronic inflammatory diseases, with high levels of C-reactive protein and SAA.…”

Section: Types Of Renal Amyloidosesmentioning

confidence: 99%

Renal amyloidosis: a new time for a complete diagnosis

Feitosa

Neves

Jorge

et al. 2022

Braz J Med Biol Res

View full text Add to dashboard Cite

Amyloidoses are a group of disorders in which soluble proteins aggregate and deposit extracellularly in tissues as insoluble fibrils, causing organ dysfunction. Clinical management depends on the subtype of the protein deposited and the affected organs. Systemic amyloidosis may stem from anomalous proteins, such as immunoglobulin light chains or serum amyloid proteins in chronic inflammation or may arise from hereditary disorders. Hereditary amyloidosis consists of a group of rare conditions that do not respond to chemotherapy, hence the identification of the amyloid subtype is essential for diagnosis, prognosis, and treatment. The kidney is the organ most frequently involved in systemic amyloidosis. Renal amyloidosis is characterized by acellular pathologic Congo red-positive deposition of amyloid fibrils in glomeruli, vessels, and/or interstitium. This disease manifests with heavy proteinuria, nephrotic syndrome, and progression to end-stage kidney failure. In some situations, it is not possible to identify the amyloid subtype using immunodetection methods, so the diagnosis remains indeterminate. In cases where hereditary amyloidosis is suspected or cannot be excluded, genetic testing should be considered. Of note, laser microdissection/mass spectrometry is currently the gold standard for accurate diagnosis of amyloidosis, especially in inconclusive cases. This article reviews the clinical manifestations and the current diagnostic landscape of renal amyloidosis.

show abstract

“…Very few viruses have been associated with AA amyloidosis. Human immunodeficiency virus (HIV) is the most frequently reported 42,65,70‐76 . Therefore, amyloidosis can be responsible for up to 10% of kidney diseases in HIV, 77 but whether this association is secondary to infections induced by immunodeficiency or coinfections or to the virus that is responsible for the chronic inflammation remains to be determined.…”

Section: Infections Associated With Aa Amyloidosismentioning

confidence: 99%

Infections and AA amyloidosis: An overview

et al. 2021

View full text Add to dashboard Cite

Background Amyloidoses are a heterogeneous group of systemic diseases characterised by extracellular accumulation of insoluble amyloid fibrils derived from unfolded proteins. Inflammatory (AA) amyloidosis can complicate various inflammatory disorders that are associated with a sustained acute phase response and serum amyloid A (SAA) protein overproduction. Chronic infections were the first recognised cause of amyloidoses. However, with the better management of underlying diseases, the frequency of AA amyloidosis is decreasing. Purpose The aim of this overview was to discuss the several infections associated with AA amyloidosis and the relative frequency of infections as aetiological factors. Methods A search of the literature was performed using the PubMed database using the MeSH terms “Amyloidosis” and “Infections,” from inception to December 31st, 2019. Articles written in other languages than English or French were excluded. Results The frequency of AA amyloidosis secondary to infections decreased from more than 50% to less than 20% after the 2000s, with a parallel increase in the frequency of AA amyloidosis secondary to inflammatory diseases and to an unknown cause. Conclusion Whereas new antibiotics have been developed and sanitary conditions are better, infections still represent 5%‐30% of the causes of AA amyloidosis, including in developed countries. These data argue for better screening of chronic infections to prevent AA amyloidosis and the development of new strategies to manage recurrent infections.

show abstract

Pattern of renal amyloidosis in South Africa

Cited by 12 publications

References 35 publications

Caso Clínico: Síndrome nefrótico como presentación de amiloidosis AL, una asociación poco frecuente en nuestro medio

Caso Clínico: Síndrome nefrótico como presentación de amiloidosis AL, una asociación poco frecuente en nuestro medio

Renal amyloidosis: a new time for a complete diagnosis

Infections and AA amyloidosis: An overview

Contact Info

Product

Resources

About