Familial Mediterranean fever associated with optic neuritis, successfully treated with anti-interleukin 1 agents

Başaran, Özge; Kavuncu, Sevim; Güven, Alev; Uncu, Nermin; Acar-Çelikel, Banu; Çakar, Nilgün

doi:10.24953/turkjped.2016.03.018

Cited by 10 publications

(12 citation statements)

References 12 publications

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“…Data on previous immunosuppressive therapy were available for 100 patients. [13][14][15][16][17][18][19][20][21] Forty-four patients used Anakinra prior to initiation of Canakinumab therapy. Of them, 26 had an intolerance to Anakinra and in 16 patients Anakinra was ineffective in preventing attacks.…”

Section: Indications For Canakinumabmentioning

confidence: 99%

“…The most common dose of Canakinumab was 2mg/kg in patients <40kg, or 150mg in those above. 13,17,18,[20][21][22][23][24][25][26][27][28] In case of inadequate disease control, an increase in dose to 3-4mg/kg in those below 40kg or 300mg in those over was used in 8 studies. 10,14,15,19,23,[29][30][31] In 8 studies, 10,19,22,[25][26][27][28]31 Canakinumab was given at 4-weekly intervals, in 3 studies 18,21,24 it was given at 8-weekly intervals, whereas the remaining studies had dosing frequencies tailored to clinical needs and/or protocols, with the medication being given every 4, 6 or 8 weeks.…”

Section: Dosing Of Canakinumabmentioning

confidence: 99%

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<p>The Efficacy, Safety and Tolerability of Canakinumab in the Treatment of Familial Mediterranean Fever: A Systematic Review of the Literature</p>

Kačar¹,

Savic²,

Hilst³

2020

JIR

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Familial Mediterranean Fever (FMF) is the most prevalent genetic autoinflammatory disorder. In most patients, treatment with colchicine can prevent attacks of fever and inflammation. However, 5%-10% of patients are resistant to colchicine treatment, while a similar percentage cannot tolerate colchicine in doses needed to prevent attacks. For these patients, Canakinumab, a full human antibody against IL-1β, has been approved recently by the FDA and EMA. In this article, we present a systematic review of the long-term efficacy, safety, and tolerability of Canakinumab in FMF patients who cannot tolerate colchicine or who are resistant to colchicine treatment.

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Section: Indications For Canakinumabmentioning

confidence: 99%

Section: Dosing Of Canakinumabmentioning

confidence: 99%

<p>The Efficacy, Safety and Tolerability of Canakinumab in the Treatment of Familial Mediterranean Fever: A Systematic Review of the Literature</p>

Kačar¹,

Savic²,

Hilst³

2020

JIR

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“…MEFV gene encodes the protein pyrin. The gain of function mu-tations in the gene leads to activation of pyrin inflammasome and overproduction of interleukin-1, causing inflammation [4]. FMF is most commonly seen in the countries of the Mediterranean basin and the highest prevalence is observed in Turks, Armenians, Sephardic Jews and Arabs [1].…”

Section: Discussionmentioning

confidence: 99%

Corticosteroid-resistant anakinra-responsive protracted febrile myalgia syndrome as the first manifestation of familial Mediterranean fever

Çakan

Karadağ

Ayaz

2019

North Clin Istanbul

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Familial Mediterranean fever (FMF) is the most common type of monogenic periodic fever syndromes and characterized by recurrent self-limited attacks of fever and polyserositis. Musculoskeletal signs and symptoms are not uncommon and manifested as arthritis and myalgia. Myalgia may be spontaneous or exercise-induced that mostly affects lower limbs and spontaneously resolves in 2-3 days. Protracted febrile myalgia syndrome (PFMS) is another form of rare and severe muscle involvement in FMF. PFMS affects all muscle groups and lasts for several weeks. Herein we present a pediatric case of PFMS that presented as the first manifestation of FMF, not responded to prednisolone at all but showed dramatic improvement with anakinra. Our case has a few distinctive points. She did not have a diagnosis of FMF and also she did not have any previous complaints compatible with FMF. Thus, PFMS was the first sign of FMF in this patient. Most of the cases of PFMS show dramatic response to corticosteroids, but our case did not respond at all to high-dose corticosteroids and anakinra resulted in rapid resolution of the symptoms. Protracted febrile myalgia syndrome may be the first manifestation of FMF. It should be suspected in cases with prolonged and unexplained fever, severe myalgia, and high acute phase reactants.

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“…Although it has been reported that uveitis, retinal ischemia, cataract, glaucoma and retinal detachment are related to FMF, optic nerve involvement has been rarely reported in the literature. 5 Alim et al evaluated the thickness of the peripapillary retinal nerve fiber layer (RNFL) and retinal ganglion cell-inner plexiform layer (GCIPL) in 42 adult-onset FMF patients by OCT but no relation was detected. 6 The inflammatory process mediated by IL-1β can be responsible for FMF-related optic nerve involvement.…”

Section: Discussionmentioning

confidence: 99%

“…6 The inflammatory process mediated by IL-1β can be responsible for FMF-related optic nerve involvement. 5 The diagnosis of case 1 was established as OPN related to adult-onset FMF. To the best of our knowledge this is the first case of OPN in medical literature associated with FMF.…”

Section: Discussionmentioning

confidence: 99%

Optic Perineuritis: How Does it Differ from Optic Neuritis?

Ateş¹,

Şahin²,

Çınar³

et al. 2019

Turkiye Klinikleri J Case Rep

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ptic perineuritis (OPN) and acute optic neuritis (ON) are clinically similar disorders. Sometimes, ocular motility disturbances, ptosis, and chemosis can accompany OPN. While ON is common in multiple sclerosis, OPN can be related to vascular, toxic, infectious, metabolic, neoplastic and inflammatory etiologic factors. Unlike demyelinating ON, central vision is spared in OPN, and it responds well to corticosteroids but recurrence is more likely to occur. Differential diagnosis of these disorders is usually dependent on radiologic features. 1,2 Fat-suppressed, contrasted magnetic resonance imaging (MRI) sequences can aid in recognizing the distinction between the two disorders. 3 The differential diagnosis is very important because the prognosis and the response to treatment are different. 1,2 In this article, we report three rare cases with clinical, ophthalmologic and radiologic findings. CASE REPORTS CASE 1 A seventy-five-year-old man was referred to our outpatient clinic complaining of the sudden onset of blurring, redness, and pain in the left eye for two days. Neurologic examination showed restricted abduction of the left eye in a conjugate horizontal gaze with a mildly dilated left pupil. Edema of Optic Perineuritis: How Does it Differ from Optic Neuritis? A AB BS S T TR RA AC CT T Sometimes, a diagnostic error between optic neuritis (ON) and optic perineuritis (OPN) can occur. The differential diagnosis of these two entities is very important because the prognosis and response to treatment are different from each other. We describe here three unusual OPN cases that presented with the symptom of sudden onset blurred vision and are associated with different etiologies (Familial Mediterranean fever, Lyme disease and possible Behcet's disease). Ophthalmological examination and optical coherence tomography can not clearly discriminate ON from OPN, however, magnetic resonance imaging of the optic nerve can help form a diagnosis through the typical appearance of optic nerve enhancement and myeline sheath detachment. These cases are discussed in detail in this article.

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Familial Mediterranean fever associated with optic neuritis, successfully treated with anti-interleukin 1 agents

Cited by 10 publications

References 12 publications

<p>The Efficacy, Safety and Tolerability of Canakinumab in the Treatment of Familial Mediterranean Fever: A Systematic Review of the Literature</p>

<p>The Efficacy, Safety and Tolerability of Canakinumab in the Treatment of Familial Mediterranean Fever: A Systematic Review of the Literature</p>

Corticosteroid-resistant anakinra-responsive protracted febrile myalgia syndrome as the first manifestation of familial Mediterranean fever

Optic Perineuritis: How Does it Differ from Optic Neuritis?

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