Protracted febrile myalgia as a challenging manifestation of familial Mediterranean fever: case-based review

Yıldırım, Deniz Gezgin; Bakkaloğlu, Sevcan A.; Buyan, Necla

doi:10.1007/s00296-018-4163-5

Cited by 11 publications

(3 citation statements)

References 19 publications

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“…A high signal intensity distributed around myofascicles in the inflamed muscles can be detected using MRI [79]. It requires high-dosage cortisone treatment or anti-Il1 agents [80].…”

Section: Musculoskeletal Symptomsmentioning

confidence: 99%

An Update on Familial Mediterranean Fever

Lancieri

Bustaffa

Palmeri

et al. 2023

IJMS

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(1) Background: Familial Mediterranean Fever (FMF) is the prototypal autoinflammatory disease, characterized by recurrent bursts of neutrophilic inflammation. (2) Methods: In this study we look at the most recent literature on this condition and integrate it with novel information on treatment resistance and compliance. (3) Results: The canonical clinical presentation of FMF is in children with self-limited episodes of fever and polyserositis, associated with severe long-term complications, such as renal amyloidosis. It has been described anecdotally since ancient times, however only recently it has been characterized more accurately. We propose an updated overview on the main aspects of pathophysiology, genetics, diagnosis and treatment of this intriguing disease. (4) Conclusions: Overall, this review presents the all the main aspects, including real life outcome of the latest recommendation on treatment resistance of FMF, a disease, that not only helped understanding the pathophysiology of the auto inflammatory process but also the functioning of the innate immune system itself.

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“…A high signal intensity distributed around myofascicles in the inflamed muscles can be detected using MRI [79]. It requires high-dosage cortisone treatment or anti-Il1 agents [80].…”

Section: Musculoskeletal Symptomsmentioning

confidence: 99%

An Update on Familial Mediterranean Fever

Lancieri

Bustaffa

Palmeri

et al. 2023

IJMS

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“…118,119 However, these experiences are limited to adult FMF patients and are relatively dated, except for 1 small case series including 5 FMF children with protracted febrile myalgia who received clinical benefit from the use of a short course of prednisolone (1-2 mg/kg), in addition to naproxen (15 mg/kg), anyway. 120 Other drugs, such as thalidomide, interferon-α and dapsone, were used in very few case reports or small case series describing adults with FMF, but there are no significant experiences in paediatrics. 118…”

Section: Other Pharmacological Resourcesmentioning

confidence: 99%

“…Some authors have suggested that steroid infusions (methylprednisolone, 40 mg) may be tried in colchicine‐resistant cases, especially if severe abdominal pain or other unusual inflammatory manifestations are present 118,119 . However, these experiences are limited to adult FMF patients and are relatively dated, except for 1 small case series including 5 FMF children with protracted febrile myalgia who received clinical benefit from the use of a short course of prednisolone (1–2 mg/kg), in addition to naproxen (15 mg/kg), anyway 120 …”

Section: Pharmacological Treatments For Pediatric Familial Mediterran...mentioning

confidence: 99%

Conventional and novel therapeutic options in children with familial Mediterranean fever: A rare autoinflammatory disease

Poddighe

Romano

Garcia‐Bournissen

et al. 2021

Brit J Clinical Pharma

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Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease and is usually diagnosed in childhood, especially in the first decade of life. Paediatric FMF is characterized by a protean clinical expression and a variable therapeutic response, which can make its medical management very challenging. However, even if long-term complications of untreated FMF (e.g. amyloidosis and related organ damage) are less frequent in children compared to adults, they are not uncommon. Colchicine is the mainstay of the therapy in paediatric FMF; however, if children develop colchicine intolerance and/or resistance, biologics, particularly interleukin-1 antagonists, must be considered. Other conventional or biological therapeutic options do not currently have appropriate evidence-based support, except for some specific clinical presentations (e.g., arthritis). In this review, we discuss the biological basis and the clinical evidence for the current pharmacological treatment options available for paediatric FMF.

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Protracted febrile myalgia syndrome as the first manifestation of familial Mediterranean fever in children: case-based review

Öztürk

Çakan

2020

Rheumatol Int

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Protracted febrile myalgia as a challenging manifestation of familial Mediterranean fever: case-based review

Cited by 11 publications

References 19 publications

An Update on Familial Mediterranean Fever

An Update on Familial Mediterranean Fever

Conventional and novel therapeutic options in children with familial Mediterranean fever: A rare autoinflammatory disease

Protracted febrile myalgia syndrome as the first manifestation of familial Mediterranean fever in children: case-based review

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