Clinical Perspectives on Amyloidosis in India: A Systematic Literature Review.

Abstract

Background: Amyloidosis is a condition characterized by deposition of insoluble protein fibrils in tissues, leading to diverse clinical manifestations. There is limited data addressing amyloidosis from India.

Objective: This study aims to systematically review the available clinical data on amyloidosis in India. By synthesizing existing knowledge, the review seeks to identify research gaps that require further exploration.

Methods: A systematic review was conducted using the PubMed database to identify English-language articles on amyloidosis from India published between 1959-2023. Additionally, abstracts from international conferences were analyzed. Data extracted were-type of amyloidosis, demographics, clinical presentation, diagnostic methods, and outcomes.

Results: The median age at presentation in Indian patients was approximately 10 years younger (50 years) compared to their counterparts in Western countries (60 years), with males comprising 70% of the cases. Renal involvement was the most common, with AA being more prevalent than AL amyloidosis, often secondary to tuberculosis. Cardiac involvement was second most common, affecting 40%-50% of patients. In patients with paraproteinemic neuropathies, AL amyloidosis accounts for the cause in 4% cases. Treatment of AL amyloidosis primarily involved chemotherapy and supportive care, with autologous transplantation underutilized due to resource limitations. Amongst localized amyloidosis, skin was the most common site (68% of all cases).

Conclusion: Amyloidosis in India remains a significant and often underdiagnosed condition, with varied presentations and causes. Most data come from retrospective studies, highlighting variability in presentation and outcomes. This review underscores the importance of understanding the disease burden and advancing research to improve outcomes in India.