Myositis in a patient with familial Mediterranean fever and spondyloarthritis successfully treated with anakinra

Estublier, Charline; Stojanovic, Katia Stankovic; Bergerot, Jean-François; Broussolle, C.; Sève, P.

doi:10.1016/j.jbspin.2013.03.004

Cited by 25 publications

(13 citation statements)

References 15 publications

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“…PFMS is a very rare manifestation of FMF and is defined as severe, disabling myalgia of at least 5 days duration (108, 112). It is associated with fever, the presence of at least one M694V mutation, and elevated inflammatory markers while creatine kinase levels are usually normal (131, 132).…”

Section: Treatmentmentioning

confidence: 99%

Familial Mediterranean Fever: Recent Developments in Pathogenesis and New Recommendations for Management

2017

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Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease (AID) affecting mainly the ethnic groups originating from Mediterranean basin. The disease is characterized by self-limited inflammatory attacks of fever and polyserositis along with elevated acute phase reactants. FMF is inherited autosomal recessively; however, a significant proportion of heterozygotes also express the phenotype. FMF is caused by mutations in the MEFV gene coding for pyrin, which is a component of inflammasome functioning in inflammatory response and production of interleukin-1β (IL-1β). Recent studies have shown that pyrin recognizes bacterial modifications in Rho GTPases, which results in inflammasome activation and increase in IL-1β. Pyrin does not directly recognize Rho modification but probably affected by Rho effector kinase, which is a downstream event in the actin cytoskeleton pathway. Recently, an international group of experts has published the recommendations for the management of FMF. Colchicine is the mainstay of FMF treatment, and its regular use prevents attacks and controls subclinical inflammation in the majority of patients. Furthermore, it decreases the long-term risk of amyloidosis. However, a minority of FMF patients fail to response or tolerate colchicine treatment. Anti-interleukin-1 drugs could be considered in these patients. One should keep in mind the possibility of non-compliance in colchicine-non-responders. Although FMF is a relatively well-described AID and almost 20 years has passed since the discovery of the MEFV gene, there are still a number of unsolved problems about it such as the exact mechanism of the disease, symptomatic heterozygotes and their treatment, and the optimal management of colchicine resistance.

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Section: Treatmentmentioning

confidence: 99%

Familial Mediterranean Fever: Recent Developments in Pathogenesis and New Recommendations for Management

2017

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“…Blockade of IL-1 signalling using anakinra has also resulted in clinical responses in myositis patients [109][110][111][112]. In a number of these patients IL-17 signalling was targeted indirectly through the reduction of IL-1-dependent Th17 differentiation [109].…”

Section: Therapeutic Implicationsmentioning

confidence: 99%

Cytokines in immune-mediated inflammatory myopathies: cellular sources, multiple actions and therapeutic implications

Moran

Mastaglia

2014

Clinical and Experimental Immunology

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SummaryThe idiopathic inflammatory myopathies are a heterogeneous group of disorders characterised by diffuse muscle weakness and inflammation. A common immunopathogenic mechanism is the cytokine-driven infiltration of immune cells into the muscle tissue. Recent studies have further dissected the inflammatory cell types and associated cytokines involved in the immune-mediated myopathies and other chronic inflammatory and autoimmune disorders. In this review we outline the current knowledge of cytokine expression profiles and cellular sources in the major forms of inflammatory myopathy and detail the known mechanistic functions of these cytokines in the context of inflammatory myositis. Furthermore, we discuss how the application of this knowledge may lead to new therapeutic strategies for the treatment of the inflammatory myopathies, in particular for cases resistant to conventional forms of therapy.

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“…The muscle biopsy showed no inflammatory infiltrates, but an immunohistochemical analysis revealed a high deposition of immunoglobulin G (IgG) and major histocompatibility complex 1 (MHC1) with sarcolemmal staining, which are signs of an inflammatory component with single muscle fiber degeneration. The other case was described by Estublier et al 8 : a 39-year-old Jewish man with FMF presented with myalgia with the M694I homozygous mutation in the MEFV gene. Similar to the former case, a muscle biopsy showed only nonspecific inflammation of the muscle.…”

mentioning

confidence: 91%

“…After the introduction of TNF inhibitors, the FMF attacks disappeared in seven patients, and the frequency of attacks decreased in the other three patients. The anakinra, an interleukin (IL)-1 receptor antagonist, was also reported to be effective for 28 colchicine-resis-tant FMF patients; complete remission was achieved by 21 patients and partial remission was observed in the other seven patients; 8 however, anakinra is not yet approved in Japan.…”

mentioning

confidence: 99%

Tocilizumab is effective in a familial Mediterranean fever patient complicated with histologically proven recurrent fasciitis and myositis

Umeda

Aramaki

Fujikawa³

et al. 2015

Int J of Rheum Dis

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Myositis in a patient with familial Mediterranean fever and spondyloarthritis successfully treated with anakinra

Cited by 25 publications

References 15 publications

Familial Mediterranean Fever: Recent Developments in Pathogenesis and New Recommendations for Management

Familial Mediterranean Fever: Recent Developments in Pathogenesis and New Recommendations for Management

Cytokines in immune-mediated inflammatory myopathies: cellular sources, multiple actions and therapeutic implications

Tocilizumab is effective in a familial Mediterranean fever patient complicated with histologically proven recurrent fasciitis and myositis

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