Autoinflammatory & rare systemic diseases
Characteristics and course of patients with AA amyloidosis: single centre experience with 174 patients from Turkey
Authors' conclusion
FMF patients still constitute the majority of AA amyloidosis patients in Turkey.
Study context
- Population
- Patients with inflammatory rheumatic diseases and immunohistologically proven AA amyloidosis, tertiary referral centre in Turkey83 female, 91 male
- Participants
- 174 patients
- Outcome
- Clinical, genetic characteristics and outcomes of AA amyloidosis
Methodological detailssingle-centre, tertiary referral centre
- Single-centre
- Tertiary referral centre
Main finding
As reported in the abstract
- FMF as most common cause of AA amyloidosis
- 78.7%
- FMF-AA amyloidosis patients carrying p.M694V variant
- 91%
- p.M694V homozygous
- 74.1%
- Age at AA amyloidosis identification, homozygous MEFV exon 10 variants (years)
- 27
- Age at AA amyloidosis identification, compound heterozygous MEFV exon 10 variants (years)
- 30
- Age at AA amyloidosis identification, heterozygous (years)
- 41
- Overall mortality rate
- 15.3%
- Mortality, renal involvement
- 10%
- Mortality, renal + gastrointestinal involvement
- 15%
- Mortality, additional cardiac involvement
- 43%
Observational data: associations, not effects.
Further findings
Progression to end-stage renal disease, eGFR <60 ml/min at admission
- Higher frequency of progression to end-stage renal disease with eGFR <60 ml/min at admission
- P < 0.001
Renal response to treatment
- Complete response
- 31%
- Partial response
- 4%
- Stable course
- 23.6%
- Progression
- 38.5%
Amyloid storm · 1 year
- Patients with amyloid storm
- 9
- Associated with increased mortality within 1 year
Original publication
Characteristics and course of patients with AA amyloidosis: single centre experience with 174 patients from Turkey. Rheumatology (Oxford). Published 20 September 2023. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →