Protracted Febrile Myalgia Syndrome in a Japanese Patient with Fasciitis Detected on MRI

Fujikawa, Keita; Migita, Kiyoshi; Takahashi, Toshiaki; Kawakami, Atsushi; Eguchi, Katsumi

doi:10.2169/internalmedicine.53.2871

Cited by 13 publications

(5 citation statements)

References 18 publications

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“…Aseptic meningitis [32] also has been described in Japanese patients with FMF. Myalgia particularly affects the legs, and protracted febrile myalgia is considered to be one of the muscular manifestations of FMF [33]. It can be resolved with prednisolone treatment.…”

Section: Clinical Manifestations Of Japanese Fmf Patientsmentioning

confidence: 99%

Familial Mediterranean fever: overview of pathogenesis, clinical features and management

Migita

Asano

Sato

et al. 2018

Immunological Medicine

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Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease, and is characterized by recurrent attacks of fever and polyserositis. It is associated with mutations in the MEFV gene encoding pyrin, which result in inflammasome activation and the uncontrolled production of IL-1b. FMF mainly affects individuals originating from the Mediterranean basin; however, a Japanese nationwide survey demonstrated that FMF is not uncommon in Japan. The survey also indicated that Japanese FMF patients are clinically or genetically distinct from Mediterranean FMF patients, suggesting a genotype-phenotype correlation. In Japanese patients with FMF, MEFV exon 10 mutations are associated with the more typical FMF phenotype. Conversely, Japanese FMF patients with mutations in MEFV exons 2 or 3 present with an atypical FMF phenotype. Colchicine is the mainstay of FMF treatment, and its regular use prevents febrile attacks and decreases the long-term risk of AA amyloidosis. However, a minority of FMF patients are colchicine-resistant, and anti-IL-1 treatment has proven beneficial in suppressing inflammation in these patients. Although Japanese FMF patients may develop less severe disease compared with Mediterranean FMF patients, they should nevertheless be treated early to prevent recurrent attacks and the subsequent development of AA amyloidosis.

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Section: Clinical Manifestations Of Japanese Fmf Patientsmentioning

confidence: 99%

Familial Mediterranean fever: overview of pathogenesis, clinical features and management

Migita

Asano

Sato

et al. 2018

Immunological Medicine

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“…A recent case report from Japan described a 22-year-old man with PFMS who showed thickening of the fascia on short-time inversion recovery (STIR) images of plain MRI [ 7 ]. The authors suggested that inflammation in the setting of PFMS occurs in the fascia rather than the muscle fiber, and that the myalgia experienced by affected patients is caused by fasciitis.…”

Section: Discussionmentioning

confidence: 99%

“…In 1988, Schapira et al [ 6 ] evaluated the ultrastructural muscle tissue features of PFMS and noted a large deposition of collagen fibers during the acute stage. More recently, Fujikawa et al [ 7 ] described a finding of fasciitis on magnetic resonance imaging (MRI) in a patient with PFMS.…”

Section: Introductionmentioning

confidence: 99%

Magnetic resonance findings may aid in diagnosis of protracted febrile myalgia syndrome: a retrospective, multicenter study

Aviran

Amarilyo

Lakovsky

et al. 2022

Orphanet J Rare Dis

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Background Protracted febrile myalgia syndrome (PFMS) is a rare complication of Familial Mediterranean fever (FMF). The diagnosis is based on clinical symptoms and is often challenging, especially when PFMS is the initial manifestation of FMF. The aim of this report was to describe the magnetic resonance imaging (MRI) findings in pediatric patients with PFMS. Results There were three girls and two boys ranging in age from 6 months to 16 years, all of Mediterranean ancestry. Three had high-grade fever, and all had elevated inflammatory markers. MRI of the extremities yielded findings suggestive of myositis, which together with the clinical picture, normal CPK levels, and supporting family history of FMF, suggested the diagnosis of PFMS. Out of most common MEFV mutations tested, one patient was homozygous for M694V mutation, three were heterozygous for M694V mutation, and one was compound heterozygous for the M694V and V726A mutations. Conclusions MRI may serve as an auxiliary diagnostic tool in PFMS.

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“…Monogenic interferonopathies, such as proteasome-associated autoinflammatory syndromes and stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy syndrome, can mimic JDM 78 , 79 . Protracted febrile myalgia is a rare manifestation of familial Mediterranean fever characterized by prolonged severe and symmetric muscle pain, fever and elevated inflammatory markers that can also mimic JIIM 80 .…”

Section: Clinical Phenotypesmentioning

confidence: 99%

Juvenile idiopathic inflammatory myositis: an update on pathophysiology and clinical care

et al. 2023

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The childhood-onset or juvenile idiopathic inflammatory myopathies (JIIMs) are a heterogenous group of rare and serious autoimmune diseases of children and young people that predominantly affect the muscles and skin but can also involve other organs, including the lungs, gut, joints, heart and central nervous system. Different myositis-specific autoantibodies have been identified that are associated with different muscle biopsy features, as well as with different clinical characteristics, prognoses and treatment responses. Thus, myositis-specific autoantibodies can be used to subset JIIMs into sub-phenotypes; some of these sub-phenotypes parallel disease seen in adults, whereas others are distinct from adult-onset idiopathic inflammatory myopathies. Although treatments and management have much improved over the past decade, evidence is still lacking for many of the current treatments and few validated prognostic biomarkers are available with which to predict response to treatment, comorbidities (such as calcinosis) or outcome. Emerging data on the pathogenesis of the JIIMs are leading to proposals for new trials and tools for monitoring disease.

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Protracted Febrile Myalgia Syndrome in a Japanese Patient with Fasciitis Detected on MRI

Cited by 13 publications

References 18 publications

Familial Mediterranean fever: overview of pathogenesis, clinical features and management

Familial Mediterranean fever: overview of pathogenesis, clinical features and management

Magnetic resonance findings may aid in diagnosis of protracted febrile myalgia syndrome: a retrospective, multicenter study

Juvenile idiopathic inflammatory myositis: an update on pathophysiology and clinical care

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