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Comparison of clinical features in FMF patients according to severity scores: An analysis with the ISSF scoring
N Şule Yaşar Bilge1, Erdal Bodakçi1, Muzaffer Bilgin2
1Division of Rheumatology, Department of Internal Medicine, Eskişehir Osmangazi University, Eskişehir, Turkey.
Objective:
Familial Mediterranean fever (FMF), is an auto-inflammatory disease characterized by attacks of fever and serositis. Some scoring systems have been developed to evaluate the severity of the disease, however, predicting the severity of FMF is not possible with current knowledge. Our aim in this study was to evaluate the factors affecting disease severity in FMF.
Methods:
This study included 150 FMF patients. The medical data of the patients were collected retrospectively and the International severity scoring system for Familial Mediterranean fever (ISSF) was used to evaluate disease severity.
Results:
Patients were sorted into 3 groups based on the ISSF scores; ≤2=mild (Group 1; n: 61), 3-5=moderate (Group 2; n: 70), and ≥6=severe (Group 3; n: 19). Age at the onset of disease and age at diagnosis was younger in patients with severe disease (p: 0.009 and p: 0.031, respectively). Fever, peritonitis, and vasculitis were similar in all 3 groups. Pleuritis, erysipelas-like erythema (ELE), arthritis, myalgia, amyloidosis, and chronic kidney disease (CKD) were more common in Group 3.
Conclusion:
FMF patients with early onset and early diagnosis, having more frequent pleuritis, ELE, arthritis, and myalgia tended to manifest a more severe form of the disease. Close monitoring of such patients may prevent the development of amyloidosis and CKD and improve the long-term prognosis of the disease.
Insights
Familial Mediterranean fever (FMF) patients with early onset and diagnosis show more severe disease, indicated by pleuritis, arthritis, and myalgia. Monitoring these individuals can prevent complications like amyloidosis and chronic kidney disease.
Area of Science:
- Rheumatology
- Genetics
- Internal Medicine
Background:
- Familial Mediterranean fever (FMF) is a chronic autoinflammatory disorder.
- Characterized by recurrent episodes of fever and serositis.
- Predicting FMF disease severity remains challenging.
Purpose of the Study:
- To identify factors influencing disease severity in Familial Mediterranean fever.
- To evaluate the correlation between clinical manifestations and disease severity using the ISSF score.
Main Methods:
- Retrospective analysis of medical data from 150 FMF patients.
- Utilized the International severity scoring system for Familial Mediterranean fever (ISSF) to categorize severity.
- Grouped patients into mild (ISSF ≤2), moderate (ISSF 3-5), and severe (ISSF ≥6) categories.
Main Results:
- Younger age at disease onset and diagnosis correlated with increased FMF severity.
- Severe FMF cases exhibited higher prevalence of pleuritis, erysipelas-like erythema (ELE), arthritis, and myalgia.
- Fever, peritonitis, and vasculitis were similarly distributed across severity groups.
Conclusions:
- Early onset and diagnosis in FMF patients are associated with more severe disease phenotypes.
- Increased monitoring of FMF patients with early onset/diagnosis is crucial.
- Proactive management may mitigate risks of amyloidosis and chronic kidney disease, improving long-term outcomes.
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