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Evaluation of familial Mediterranean fever patients concomitant with juvenile spondyloarthropathy
Ayşenur Paç Kısaarslan1, Nihal Şahin1, Sümeyra Özdemir Çiçek1
1Department of Pediatric Rheumatology, Faculty of Medicine, Erciyes University, Kayseri, Turkey.
Objectives:
Familial Mediterranean fever (FMF) may present with various concomitant diseases. This study aims to evaluate the clinical characteristics of patients with FMF with Juvenile Spondyloarthropathy (jSpA).
Method:
Thirty-two patients diagnosed with FMF/jSpA, sixty-four with FMF, and fifty-four with jSpA were included in this retrospective study. Three patient groups were compared in terms of clinical and laboratory features.
Results:
The mean ages of patients in the FMF/jSpA, FMF and jSpA groups were 15.75(11.50-19.83), 15,41(6.83-21.50), and 16(9-22) years, respectively. Chronic arthritis (OR: 0.11, p = .049), erythrocyte sedimentation rate values (OR:1.07, p = .011), and C-reactive protein values (OR:1,08, p: .039) of the patients in remission period were found higher, the international severity scores for FMF (ISSF) before and after colchicine treatment (OR: 1.16, p: .021, OR: 2,21, p: .012) were higher in the FMF/jSpA group compared to FMF. Plantar fasciitis was more common and HLA-B27 positivity rate was lower in the FMF/jSpA group (OR:0.08, p = .024), (OR:4.71, p = .002) compared to jSpA. FMF/jSpA patients were divided as previous diagnosed FMF and jSpA.The diagnosis of jSpA was at a younger age(p = .002), Juvenile arthritis damage index-articular(p = 0.022) and extraarticular(p = .026), and the rate of biologic drug usage(p = .015) were higher in the previous jSpA group. The number of FMF attacks before colchicine was lower in the previous jSpA group(p = .02).
Conclusion:
Our findings suggest that both classical FMF and jSpA findings were lower in patients with FMF/jSpA. Patients who were diagnosed with jSpA at an early age and who had enthesitis and plantar fasciitis should also be evaluated in terms of FMF.
Insights
Familial Mediterranean fever (FMF) and juvenile spondyloarthropathy (jSpA) can co-occur. Patients with both conditions showed distinct clinical and laboratory features compared to those with only one disease, suggesting FMF evaluation for early-onset jSpA with specific symptoms.
Area of Science:
- Rheumatology
- Genetics
- Immunology
Background:
- Familial Mediterranean fever (FMF) is a genetic autoinflammatory disorder.
- Juvenile spondyloarthropathy (jSpA) is a group of inflammatory arthritic conditions affecting children.
- Concomitant occurrence of FMF and jSpA presents unique clinical challenges.
Purpose of the Study:
- To investigate the clinical and laboratory characteristics of patients with coexisting FMF and jSpA.
- To compare these features with patients having only FMF or only jSpA.
- To identify potential diagnostic indicators for FMF in jSpA patients.
Main Methods:
- Retrospective study including 32 FMF/jSpA patients, 64 FMF patients, and 54 jSpA patients.
- Comparison of clinical and laboratory features across the three groups.
- Analysis of disease severity scores and specific symptoms like enthesitis and plantar fasciitis.
Main Results:
- FMF/jSpA patients exhibited higher erythrocyte sedimentation rate and C-reactive protein values during remission.
- International Severity Scores for FMF (ISSF) were higher in the FMF/jSpA group.
- Plantar fasciitis was more prevalent, and HLA-B27 positivity was lower in FMF/jSpA patients compared to jSpA alone.
Conclusions:
- The study highlights distinct clinical and laboratory profiles for patients with coexisting FMF and jSpA.
- Early-onset jSpA with enthesitis and plantar fasciitis warrants evaluation for FMF.
- Recognizing these overlapping features can improve diagnosis and management of these complex cases.
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