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Systemic AA Amyloidosis Secondary to Chronic Bronchiectasis and Primary Immunodeficiency: A Case Report

Year: 2025
Type: article
Study design: Case Report
Abstract: A 37-year-old ex-teacher, having suffered severe bronchiectasis for over a decade and not diagnosed as having primary specific antibody deficiency, later developed systemic AA amyloidosis and end-stage renal disease. Persistent respiratory symptoms developed at age 26, and subsequent investigations revealed cylindrical bronchiectasis and impaired specific antibody responses to Streptococcus pneumoniae and Haemophilus influenzae. Lack of compliance with the treatment and follow-up despite continuous specialist treatment led to a gradual decline in lung function and frequent Pseudomonas aeruginosa and Streptococcus pneumoniae-induced infections. The patient has progressively developed extreme systemic complications with severe renal dysfunction and hypoalbuminemia. In May 2024, a biopsy of the kidney indicated that the amyloid A was deposited all over the kidneys, and the condition was identified as amyloidosis caused by an underlying chronic respiratory disease. By May 2025, his renal functions had deteriorated significantly, leading to renal replacement therapy, as well as extreme malnutrition and frequent infections. The given case demonstrates the importance of prompt and thorough treatment of chronic inflammatory diseases and their etiologic alterations in immune disorders to prevent severe complications, such as AA amyloidosis.
Source: Cureus
DOI: 10.7759/cureus.88790
Authors: Juhi Jain, Muhammad Junaid Akram, Gerald Langman, Rahul Mukherjee
Language: English
Open Access status: diamond
OpenAlex ID: W4412663290
FWCI:
Cites: 8
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Related to: 10