Sacroiliitis in children and adolescents with familial Mediterranean fever
Hülya Kaçmaz1, Esin Aldemir2, Ayşe Tanatar3
1Department of Pediatric Rheumatology, University of Health Sciences, Kanuni Sultan Süleyman Research and Training Hospital, Istanbul, Turkey.
Background:
Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by recurrent episodes of fever and serositis. Sacroiliitis can be observed in some FMF patients. This study aimed to compare the demographic, clinical, and laboratory findings, and treatment in children with FMF and sacroiliitis, and children with juvenile spondyloarthropathy (JSpA).
Methods:
In total, 1687 pediatric FMF patients that were followed-up between May 2010 and June 2020 were evaluated retrospectively. Among them, those with sacroiliitis (n = 63) were included in the study and compared to patients with JSpA (n = 102).
Results:
The study included 63 FMF patients with sacroiliitis (38 males [60.3%] and 25 females [39.7%]) with a mean age of 15.2 ± 4.1 years. Mean age at symptom onset was 7.2 ± 5.05 years and mean age at diagnosis was 9.74 ± 4.67 years. The most common mutation in the FMF patients was M694V/M694V (n = 22). Patients were diagnosed with sacroiliitis with a mean of 12 months (range: 6-36 months) after the diagnosis of FMF. Among the FMF patients, 28 (44.4%) had enthesitis, 23 (36.5%) had heel pain, and 11 (17.4%) had low back pain. The study also included 102 JSpA patients (90 males [88.2%] and 12 females [11.8%]). Mean age of patients with JSpA was 16.1 ± 2.8 years. As compared to 102 JSpA patients, patients with FMF and sacroiliitis had higher acute phase reactants, whereas HLA-B27 positivity rate was lower. In addition, axial involvement rate was higher in the JSpA patients.
Conclusion:
Sacroiliitis is a common co-morbidity in FMF patients. The phenotypic features of these patients are different from patients with JSpA.
Insights
Familial Mediterranean fever (FMF) with sacroiliitis presents distinct features compared to juvenile spondyloarthropathy (JSpA). FMF patients with sacroiliitis showed higher acute phase reactants and lower HLA-B27 rates than JSpA patients.
Area of Science:
- Pediatric Rheumatology
- Autoinflammatory Diseases
- Spondyloarthropathies
Background:
- Familial Mediterranean fever (FMF) is an autoinflammatory condition causing recurrent fever and serositis.
- Sacroiliitis, inflammation of the sacroiliac joints, can occur in FMF patients.
- Distinguishing FMF-associated sacroiliitis from juvenile spondyloarthropathy (JSpA) is clinically important.
Purpose of the Study:
- To compare demographic, clinical, and laboratory findings in pediatric FMF patients with sacroiliitis versus those with JSpA.
- To analyze treatment differences between these two groups.
- To elucidate the distinct characteristics of sacroiliitis in FMF.
Main Methods:
- Retrospective analysis of 1687 pediatric FMF patients from May 2010 to June 2020.
- Inclusion of 63 FMF patients with sacroiliitis and 102 JSpA patients for comparison.
- Evaluation of demographic data, clinical symptoms, laboratory results, and treatment regimens.
Main Results:
- FMF patients with sacroiliitis (n=63) had a mean age of 15.2 years; JSpA patients (n=102) had a mean age of 16.1 years.
- FMF patients exhibited higher acute phase reactants and lower HLA-B27 positivity rates compared to JSpA patients.
- Axial involvement was more prevalent in JSpA patients.
Conclusions:
- Sacroiliitis is a notable comorbidity in pediatric FMF.
- Phenotypic presentations of sacroiliitis in FMF differ significantly from those in JSpA.
- Understanding these differences aids in accurate diagnosis and management.
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