Autoinflammatory & rare systemic diseases
Predictors of proteinuria, amyloidosis and kidney failure in familial Mediterranean fever: data from the International AIDA Network Registry
Authors' conclusion
FMF patients with older age at diagnosis, longer disease duration, anaemia, leucocytosis, thrombocytosis, elevated ESR and homozygous M694V mutation are at higher risk of kidney complications.
Study context
- Population
- FMF patients in the International AIDA Network registry for monogenic autoinflammatory diseases
- Participants
- 598 patients
- Outcome
- Risk factors for proteinuria, amyloidosis and kidney failure
Methodological detailsinternational multicentre registry, multivariate regression
- International multicentre registry
- Multivariate regression
Main finding
As reported in the abstract
Proteinuria (n=80), multivariate predictors
- Out-of-flares thrombocytosis: OR 4.78 (95% CI 1.54-14.8), P = 0.007
- Increased out-of-flares ESR: OR 2.7 (95% CI 1.3-5.6), P = 0.008
- Homozygous M694V mutation: OR 2.27 (95% CI 1.1-4.66), P = 0.025
- Heterozygous M694V mutation: OR 0.29 (95% CI 0.09-0.86), P = 0.026
Observational data: associations, not effects.
Further findings
Amyloidosis (n=61), multivariate predictors
- Disease duration: OR 1.034 (95% CI 1.004-1.065), P = 0.027
- During-flares anaemia: OR 2.9 (95% CI 1.18-7.19), P = 0.021
- Out-of-flares leucocytosis: OR 7.47 (95% CI 1.6-34.7), P = 0.01
- Increased out-of-flares ESR: OR 3.6 (95% CI 1.48-8.81), P = 0.005
- Heterozygous M694V mutation: OR 0.18 (95% CI 0.035-0.9), P = 0.04
Kidney failure (n=25), multivariate predictors
- Age at diagnosis: OR 1.04 (95% CI 1.0003-1.19), P = 0.048
- Disease duration in years: OR 1.07 (95% CI 1.02-1.12), P = 0.005
- Attack frequency per year: OR 1.04 (95% CI 1.007-1.076), P = 0.019
- Anaemia out-of-flares: OR 4.7 (95% CI 1.004-22.1), P = 0.049
- Out-of-flares leucocytosis: OR 25.8 (95% CI 2.75-242), P = 0.004
Intraclass correlation coefficient related to ethnicity and country of residence
- Proteinuria and kidney failure
- 0%
- Amyloidosis: ethnicity 6.7%, country of residence 6.8%
Original publication
Predictors of proteinuria, amyloidosis and kidney failure in familial Mediterranean fever: data from the International AIDA Network Registry. Rheumatology (Oxford). Published 2 April 2025. doi:10.
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