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Inflammatory comorbidities ın the largest pediatric Familial Mediterranean fever cohort: a multicenter retrospective study of Pediatric Rheumatology Academy (PeRA)-Research Group (RG)

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Abstract

Aim

The aim of this study was to investigate the frequency and type of FMF-associated inflammatory diseases in a large FMF pediatric patients and to compare them to those FMF patients without concomitant inflammatory diseases.

Materials and methods

Familial Mediterranean fever patients enrolled in the Pediatric Rheumatology Academy (PeRA)-Research Group (RG) were included. The patients were divided into two groups according to concomitant inflammatory disease as FMF patients who had a concomitant inflammatory disease (group 1) and FMF patients who did not have a concomitant inflammatory disease (group 1). The clinical findings and treatments were compared between the two groups.

Results

The study group comprised 3475 patients with FMF. There were 294 patients (8.5%) in group 1 and 3181 patients (91.5%) in group 2. Juvenile idiopathic arthritis (n = 136) was the most common accompanying inflammatory disease. Arthritis, M694V homozygosity, and the need for biological therapy were more frequently observed in Group 1 (p < 0.05). Fever and abdominal pain were more frequently detected in Group 2 (p < 0.05). FMF patients with concomitant inflammatory diseas more frequently demonstrated colchicine resistance. There were no significant differences in the median attack frequency, chest pain, amyloidosis, erysipelas-like erythema, or family history of FMF between the two patient groups.

Conclusion

To the best of our knowledge, this is the largest pediatric cohort reviewed to date. FMF patients may have different clinical profiles and colchicine responses if they have with concomitant inflammatory diseases.

Key points

• FMF is associated with some inflammatory comorbidities diseases.

• To the best of our knowledge, this is the largest cohort evlauated pediatric FMF associated inflammatory comorbidities diseases reviewed to date

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Authors and Affiliations

  1. Department of Pediatric Rheumatology, Dr Sami Ulus Maternity and Child Health and Diseases Training and Research Hospital, Ankara, Turkey

    Semanur Ozdel, Elif Arslanoglu Aydın, Esra Bağlan & Mehmet Bülbül

  2. Department of Pediatric Rheumatology, Etlik City Hospital, Ankara, Turkey

    Semanur Ozdel

  3. Department of Pediatric Rheumatology, University of Health Sciences, Umraniye Research and Training Hospital, Istanbul, Turkey

    Taner Coşkuner, Mustafa Çakan & Betül Sözeri

  4. Department of Pediatric Rheumatology, Istanbul University, Faculty of Medicine, Istanbul, Turkey

    Fatmagül Demirkan, Şerife Gül Karadağ & Nuray Aktay Ayaz

  5. Department of Pediatric Rheumatology, Faculty of Medicine, Dokuz Eylül University, Izmir, Turkey

    Rüya Torun, Balahan Bora Makay & Şevket Erbil Ünsal

  6. Department of Pediatric Rheumatology, Medicalpark Hospital, Gaziantep, Turkey

    Gülçin Otar Yener

  7. Department of Pediatric Rheumatology, Istanbul Medeniyet University, Göztepe Prof. Dr. Süleyman Yalçın City Hospital, Istanbul, Turkey

    Kübra Öztürk

  8. Department of Pediatric Rheumatology, Acıbadem Healthcare Group, Istanbul, Turkey

    Ferhat Demir

  9. Department of Pediatric Rheumatology, Kocaeli University, Faculty of Medicine, Kocaeli, Turkey

    Hafize Emine Sönmez

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  1. Semanur Ozdel
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Ozdel, S., Coşkuner, T., Demirkan, F. et al. Inflammatory comorbidities ın the largest pediatric Familial Mediterranean fever cohort: a multicenter retrospective study of Pediatric Rheumatology Academy (PeRA)-Research Group (RG). Clin Rheumatol 43, 407–413 (2024). https://doi.org/10.1007/s10067-023-06802-6

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