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Erschienen in: Clinical Rheumatology 5/2022

28.01.2022 | Original Article

The expanded spectrum of arthritis in children with familial Mediterranean fever

verfasst von: Pinar Ozge Avar-Aydın, Zeynep Birsin Ozcakar, Fatma Aydın, Hatice Dilara Karakas, Nilgun Cakar, Fatos Yalcınkaya

Erschienen in: Clinical Rheumatology | Ausgabe 5/2022

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Abstract

Introduction

Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease that can present with various forms of arthritis. This retrospective study aims to evaluate the characteristics of patients with arthritis in a large pediatric cohort of FMF patients.

Methods

The demographic and clinical data were extracted from electronic medical records. Patients with arthritis were grouped as arthritis of FMF and arthritis of FMF-associated diseases.

Results

A total of 541 patients were followed with a diagnosis of FMF in the last 5 years. Acute arthritis of FMF (n: 138) was the most common cause. It showed a recurrent course in the majority with a longer duration than other attack symptoms. Significantly higher frequencies of biallelic exon 10 and M694V mutations, erysipelas-like erythema, and protracted febrile myalgia were detected in these patients, particularly in those older than 2 years of age. Sacroiliitis of FMF was the second most common cause (n: 19). Patients with acute arthritis and sacroiliitis of FMF needed higher doses of colchicine. One patient with neonatal-onset FMF and M694V homozygosity was diagnosed with protracted arthritis. Arthritides of FMF-associated diseases including IgA vasculitis (n: 10), juvenile idiopathic arthritis (n: 9), chronic nonbacterial osteomyelitis (n: 5), and inflammatory bowel disease (n: 2) were detected in 26 patients.

Conclusions

Arthritis is an important clinical finding of FMF mostly associated with M694V mutations. The frequency of protracted arthritis is declined, whereas sacroiliitis of FMF and arthritis of associated diseases expand the spectrum of arthritis. This study represents the changing face and current perspectives of arthritis in FMF.
Key Points
• Arthritis is an important clinical finding of familial Mediterranean fever (FMF) that can present in various forms
• Arthritis is most likely associated with M694V mutations
• The frequency of protracted arthritis is declined whereas sacroiliitis of FMF and arthritis of associated diseases expand the spectrum of arthritis in FMF
Literatur
3.
Zurück zum Zitat Ince E, Cakar N, Tekin M et al (2002) Arthritis in children with familial Mediterranean fever. Rheumatol Int 21:213–217 CrossRef Ince E, Cakar N, Tekin M et al (2002) Arthritis in children with familial Mediterranean fever. Rheumatol Int 21:213–217 CrossRef
7.
Zurück zum Zitat Petty RE, Southwood TR, Manners P et al (2004) International League of Associations for Rheumatology Classification of Juvenile Idiopathic Arthritis: Second Revision, Edmonton, 2001. J Rheumatol 31:390–392 PubMed Petty RE, Southwood TR, Manners P et al (2004) International League of Associations for Rheumatology Classification of Juvenile Idiopathic Arthritis: Second Revision, Edmonton, 2001. J Rheumatol 31:390–392 PubMed
16.
Zurück zum Zitat Tanatar A, Karadağ ŞG, Çakan M et al (2021) Age of onset as an influencing factor for disease severity in children with familial Mediterranean fever. Mod Rheumatol 31:219–222 CrossRef Tanatar A, Karadağ ŞG, Çakan M et al (2021) Age of onset as an influencing factor for disease severity in children with familial Mediterranean fever. Mod Rheumatol 31:219–222 CrossRef
19.
Zurück zum Zitat Kargin Cakici E, Kurt Sukur ED, Ozlu SG et al (2019) MEFV gene mutations in children with Henoch-Schönlein purpura and their correlations—do mutations matter? Clin Rheumatol 38:1947–1952 CrossRef Kargin Cakici E, Kurt Sukur ED, Ozlu SG et al (2019) MEFV gene mutations in children with Henoch-Schönlein purpura and their correlations—do mutations matter? Clin Rheumatol 38:1947–1952 CrossRef
23.
Zurück zum Zitat Avar Aydin PO, Özçakar ZB, Çakar N et al (2020) Chronic non-bacterial osteomyelitis: another disease associated with MEFV gene mutations. Clin Exp Rheumatol 38:S112–S117 Avar Aydin PO, Özçakar ZB, Çakar N et al (2020) Chronic non-bacterial osteomyelitis: another disease associated with MEFV gene mutations. Clin Exp Rheumatol 38:S112–S117
Metadaten
Titel
The expanded spectrum of arthritis in children with familial Mediterranean fever
verfasst von
Pinar Ozge Avar-Aydın
Zeynep Birsin Ozcakar
Fatma Aydın
Hatice Dilara Karakas
Nilgun Cakar
Fatos Yalcınkaya
Publikationsdatum
28.01.2022
Verlag
Springer International Publishing
Erschienen in
Clinical Rheumatology / Ausgabe 5/2022
Print ISSN: 0770-3198
Elektronische ISSN: 1434-9949
DOI
https://doi.org/10.1007/s10067-022-06082-6

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