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Not easy-peasy to diagnose: familial Mediterranean fever unaccompanied by fever

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Abstract

Classical attacks of familial Mediterranean fever (FMF) are often accompanied by fever, but some of the patients have attacks without fever. This study aimed to compare the characteristics of FMF patients with and without fever during their attacks and draw attention to the different clinical presentations of FMF in children. Medical files of patients aged 0–18 years who were followed up with the diagnosis of FMF in two reference pediatric rheumatology centers were reviewed retrospectively. The patients were divided into two groups: children who had had no fever in any of their attacks were assigned as group 1, and those who had fever during their attacks were classified as group 2. Out of 2003 patients evaluated, 191 (9.53%) patients had attacks not accompanied by fever and their median age at onset of symptoms (7.0 vs. 4.0 years, p < 0.001) and the median age at diagnosis (8.6 vs. 6.0 years, p < 0.001) were significantly higher; however, group 2 had a delay in diagnosis. The annual number of attacks and abdominal attacks were more common in group 2; arthritis, arthralgia, erysipelas-like rash, exercise-induced leg pain, and myalgia were more common in group 1.

    Conclusion: The data from the assessment of children with FMF attacks not accompanied with fever were presented for the first time. Children with late age onset of FMF and dominance of musculoskeletal features may display attacks not accompanied with fever.

What is Known:

• Familial Mediterranean fever (FMF) is the most common inherited auto-inflammatory disease, characterized by recurrent attacks of fever, serositis, and musculoskeletal symptoms.

• Although fever is the most common symptom, few studies have reported attacks without fever.

What is New:

• The aim of this study was to identify patients with FMF but without fever during attacks and to demonstrate their distinctive presentations.

• We found that 7% of our patients had afebrile attacks with predominant musculoskeletal symptoms and were diagnosed earlier than patients with febrile attacks, probably due to early referral to pediatric rheumatology clinics.

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Authors and Affiliations

  1. Department of Pediatric Rheumatology, İstanbul School of Medicine, İstanbul University, İstanbul, Turkey

    Selen Duygu Arık, Gülşah Kavrul Kayaalp, Vafa Guliyeva, Fatma Gül Demirkan, Ayşe Tanatar, Özlem Akgün, Şerife Gül Karadağ & Nuray Aktay Ayaz

  2. Department of Pediatric Rheumatology, Umraniye Training and Research Center, University of Health Sciences, İstanbul, Turkey

    Şengül Çağlayan, Kadir Ulu, Taner Coşkuner & Betul Sözeri

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  1. Selen Duygu Arık
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Contributions

SDA, GKK, and NAA designed the form of the study. SDA, FGD, GKK, VG, and NAA prepared the initial draft of the article. AT, ÖA, ŞÇ, KU, TC, ŞGK, and BS revised it critically for important intellectual content. All authors contributed to data collection, analysis and interpretation of data, providing comments to the draft article and final approval of the article.

Corresponding author

Correspondence to Nuray Aktay Ayaz.

Ethics declarations

Ethics approval

This study was performed in line with the principles of the Declaration of Helsinki. Ethics committee approval (No: 816470) was obtained from Istanbul University, Faculty of Medicine.

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Because of the retrospective nature of the study, obtaining written informed consent from the patients was not required.

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Not applicable.

Conflict of interest

The authors declare no competing interests.

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Communicated by Peter de Winter

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Arık, S.D., Kayaalp, G.K., Guliyeva, V. et al. Not easy-peasy to diagnose: familial Mediterranean fever unaccompanied by fever. Eur J Pediatr 182, 3983–3988 (2023). https://doi.org/10.1007/s00431-023-05061-6

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