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Familial Mediterranean fever and acute rheumatic fever: a pathogenetic relationship?
M Tekin1, F Yalçinkaya, N Tümer
1Ankara University School of Medicine, Turkey. mtekin@hsc.vcu.edu
Abstract:
The frequency of acute rheumatic fever (ARF) in patients with familial Mediterranean fever (FMF) was documented and the effects of preceding streptococcal infections on the exacerbation of FMF were determined. In the first part of the study, 162 individuals with FMF were investigated for a history of ARF in a retrospective study. In the second part of the study, antistreptolysin-O (ASO) titres were measured in 130 individuals with FMF. Thirty-six patients had an arthritic attack (group A1), 55 patients had a typical FMF attack without arthritis (group A2) and 39 patients were in the attack-free interval (group B) during the investigation. Nine patients with FMF (5.5%) were considered to have ARF and three of them (1.85%) also had rheumatic heart disease. This prevalence of rheumatic heart disease in FMF is higher than that of the normal population (0.65%) reported in Turkey. Elevated ASO titres were found in 75%, 42% and 38% of the patients in groups A1, A2 and B, respectively. These percentages were found to be significantly higher in group A1 than in both groups A2 (p<0.01) and B (p<0.01). We concluded that patients with FMF might be more prone to the late complications of streptococcal infections.
Insights
Familial Mediterranean Fever (FMF) patients show a higher incidence of acute rheumatic fever (ARF) and rheumatic heart disease. Streptococcal infections may increase the risk of FMF complications, particularly arthritis.
Area of Science:
- Rheumatology
- Immunology
- Genetics
Background:
- Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder.
- The relationship between FMF, acute rheumatic fever (ARF), and streptococcal infections is not fully understood.
- Previous studies suggest potential links, but further investigation is needed.
Purpose of the Study:
- To determine the frequency of ARF in FMF patients.
- To investigate the impact of preceding streptococcal infections on FMF exacerbations.
- To assess the prevalence of rheumatic heart disease in FMF patients.
Main Methods:
- Retrospective study of 162 FMF patients for ARF history.
- Measurement of antistreptolysin-O (ASO) titres in 130 FMF patients during different attack phases.
- Comparison of ASO titres between patients with arthritic attacks, non-arthritic attacks, and attack-free intervals.
Main Results:
- Nine FMF patients (5.5%) had ARF, with three (1.85%) having rheumatic heart disease, a higher prevalence than the general population.
- Elevated ASO titres were observed in 75% of patients with arthritic attacks (Group A1), significantly higher than in other groups.
- ASO titres were elevated in 42% of patients with non-arthritic attacks (Group A2) and 38% in the attack-free interval (Group B).
Conclusions:
- FMF patients may have an increased susceptibility to late complications following streptococcal infections.
- The findings suggest a potential link between streptococcal infections and FMF exacerbations, particularly arthritic attacks.
- Further research is warranted to elucidate the mechanisms underlying this association and its clinical implications.