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Background: Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by recurrent attacks of fever, serositis, and musculoskeletal symptoms [1, 2]. Arthritis is the most common musculoskeletal symptom of attacks and also included in diagnostic criteria of FMF [3]. If it is specifically queried, myalgia may be detected in up to 20-25% of the patients with FMF. [4, 5].
Objectives: In our study, we aim to assess the localization of attack-related myalgia and associated parameters in patients with FMF.
Methods: A total of 349 consecutive patients followed by FMF in our clinic were enrolled in the study and asked for attack-induced myalgia and if present, localization of muscle groups on the mannequin body parts diagram.
Attack frequency, duration, and disease activity were evaluated with the AutoInflammatory Diseases Activity Index (AIDAI) scoring system [6]. Patients were also asked for work/study day loss during attacks and patient acceptable symptom state (PASS) status [7].
Results: 126 patients showed attack myalgia (36%); attack duration, frequency, severity were significantly higher in patients with attack-myalgia (p<0,005). Most common muscle groups were calves, lower back, and latissimus dorsi muscles in order. Myalgia was most commonly accompanied by arthritis (p<0,002). Patients with myalgia have a higher frequency of colchicine resistance and work/study day loss due to attacks.
Conclusion: Our results conclude that myalgia is an important domain of attacks and causes absenteeism and uncontrolled disease activity.
REFERENCES:
[1]El-Shanti H, Majeed HA, El-Khateeb M. Familial mediterranean fever in Arabs.Lancet. 2006;367(9515):1016–24.
[2]Majeed HA, Al-Qudah AK, Qubain H, Shahin HM. The clinical patterns of myalgia in children with familial Mediterranean fever. Semin Arthritis Rheum. 2000;30(2):138–43.
[3]Gattorno M, Hofer M, Federici S, Vanoni F et al. Eurofever Registry and the Paediatric Rheumatology International Trials Organisation (PRINTO). Classification criteria for autoinflammatory recurrent fevers. Ann Rheum Dis. 2019 Aug;78(8):1025-1032. doi: 10.1136/annrheumdis-2019-215048.
[4]Zemer D. Muscle pains in familial Mediterranean fever. Harefuah 1984; 106: 232-233.
[5]Majeed HA. Differential diagnosis of fever of unknown origin in children. Curr Opin Rheumatol 2000; 12: 439-444.
[6]Piram M, Frenkel J, Gattorno M et al. EUROFEVER and EUROTRAPS networks. A preliminary score for the assessment of disease activity in hereditary recurrent fevers: results from the AIDAI (Auto-Inflammatory Diseases Activity Index) Consensus Conference. Ann Rheum Dis. 2011 Feb;70(2):309-14. doi: 10.1136/ard.2010.132613.
[7]Salaffi F, Carotti M, Gutierrez M, Di Carlo M, De Angelis R. (2015) Patient Acceptable Symptom State in Self-Report Questionnaires and Composite Clinical Disease Index for Assessing Rheumatoid Arthritis Activity: Identification of Cut-Off Points for Routine Care. Biomed Res Int. 2015:930756. doi: 10.1155/2015/930756.
Comparison of clinical and laboratory parameters between patients with /without myalgia attacks
| Patients with myalgia attack | Patients without myalgia attack | p value | |
|---|---|---|---|
| Age (years) | 36,33 ±10, 68 | 37,9±11, 48 | >0,05 |
| Sex (female/male) | 78/43 | 138/87 | >0,05 |
| Follow-up time (years) | 16,1 | 17,6 | >0,05 |
| Dominant attack(number) | |||
| Peritonitis | 42 | 55 | >0,05 |
| Arthritis | 11 | 39 | 0,012 |
| Pleuritis | 13 | 10 | >0,05 |
| Only fever | 4 | 7 | >0,05 |
| AIDAI score (mean, 0-175) | 127,40 | 80,68 | <0,05 |
| VAS score for pain (median score, during attack. 0-10) | 8 | 5 | <0,05 |
| Colchicine resistance (number of patients) | 69 | 25 | 0,003 |
| Colchicine-resistantColchicine-sensitive | 29 | 84 | 0,002 |
| PASS status (number) | 41 | 6 | 0,016 |
| Need additional treatment Satisfied from treatment | 34 | 74 | 0,010 |
| Work/study day loss (number) | 48 | 6 | 0,003 |
Acknowledgements: No financial funding is received for this report. Informed consents for participating and publishing were obtained from all participants
Disclosure of Interests: None declared