ArticleRheumatology (Oxford, England)2024
Characteristics and course of patients with AA amyloidosis: single centre experience with 174 patients from Turkey.
Article in Rheumatology (Oxford, England), 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed.
- AA amyloidosis in the four historical monogenic autoinflammatory diseases: Clinical burden and insights from a retrospective referral-centre study.Journal of internal medicine · 2026Article
- Long-term clinical course of pediatric familial Mediterranean fever with heterozygous pathogenic MEFV exon 10 variants.European journal of pediatrics · 2026Article
- Evaluation of echocardiographic features and cardiac biomarkers in patients with AA amyloidosis: a retrospective comparative study with patients with al amyloidosis and hypertrophic cardiomyopathy.Rheumatology international · 2026Observational
- Amyloidosis awareness and colchicine adherence in patients with familial Mediterranean fever: a cross-sectional study in an endemic region.Clinical rheumatology · 2026Article
- Clinical Association of Pan-Immune-Inflammation Value with MEFV Mutation Burden and Amyloidosis in Adults with Familial Mediterranean Fever: A Retrospective Cohort Study.Journal of clinical medicine · 2026Article
- Amyloidosis secondary to familial Mediterranean fever: machine learning-based prediction models.Rheumatology (Oxford, England) · 2026Article
- [An unusual cause for kidney transplantation].Innere Medizin (Heidelberg, Germany) · 2026Article
- Pathogenesis and kidney prognosis of renal amyloidosis.Cellular and molecular life sciences : CMLS · 2026Review
- Evaluation of pediatric FMF patients with biallelic pathogenic exon 10 variants: focus on chest pain.European journal of pediatrics · 2026Article
- Cardiac amyloidosis risk across ethnoracial and clinical subgroups: a five-year national study.American journal of cardiovascular disease · 2026Article
- Re-evaluation of MEFV carriers previously diagnosed with FMF: a colchicine discontinuation study.Rheumatology (Oxford, England) · 2025Article
- From ulcerative colitis to amyloid storm: a case-based review.Rheumatology international · 2025Review
- Comparative Histopathological Characteristics of Duodenal Involvement in Different Types of Amyloidosis.Biomedicines · 2025Article
- Scrotal Pain in Familial Mediterranean Fever: A Series of Three Case Reports and Literature Review.Mediterranean journal of rheumatology · 2025Article
- Canakinumab Treatment in Familial Mediterranean Fever Patients: With/Without Colchicine.International journal of rheumatic diseases · 2025Article
- AA Amyloidosis: A Contemporary View.Current rheumatology reports · 2024Review
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13 authors.
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Abstract
objectivesThis study aimed to evaluate the clinical, laboratory and genetic characteristics and outcomes of patients with AA amyloidosis.
methodsPatients followed up in a tertiary referral centre in Turkey with the diagnosis of inflammatory rheumatic diseases and immunohistologically proven AA amyloidosis were included in the study and retrospectively analysed.
resultsAmong 184 patients with the diagnosis of AA amyloidosis, 174 (83 female, 91 male) were included in the analysis. The most common cause of AA amyloidosis was FMF (78.7%), and 91% of FMF-AA amyloidosis patients were carrying the p.M694V variant (74.1% homozygous). AA amyloidosis was identified earlier in patients with homozygous or compound heterozygous MEFV exon 10 variants compared with the heterozygous patients (27, 30 and 41 years, respectively). Patients with an estimated glomerular filtration rate <60 ml/min at admission had a higher frequency of progression to end-stage renal disease (P < 0.001). The overall mortality rate was 15.3% and it increased gradually in association with the amyloid burden (10% in patients with renal, 15% in renal + gastrointestinal and 43% in those with additional cardiac involvement). Renal findings responded completely to treatment in 31% of the patients, a partial response was observed in 4%, a stable course in 23.6% and progression in 38.5%. Amyloid storm was identified in nine patients and was found to be associated with increased mortality within 1 year.
conclusionFMF patients still constitute the majority of AA amyloidosis patients in Turkey. The MEFV genotype and associated inflammatory load may affect the age of onset of AA amyloidosis, and earlier diagnosis and stricter follow-up and treatment may delay progression of the disease.
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