THU0268 The frequency of juvenile spondyloarthropathies in childhood famillial mediterranean fever

Özer, Emre; Seker, Demet; Taner, Hasan Emir; Adroviç, Amra; Şahin, Sezgin; Köker, Oya; Barut, Kenan; Kasapçopur, Özgür

doi:10.1136/annrheumdis-2018-eular.3419

Cited by 14 publications

(18 citation statements)

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“…An erythematous rash can be observed over the involved joint, and this unique sign is termed as “red arthritis” ( 24 ). In addition, seronegative sacroiliitis has been reported in patients with FMF ( 33 , 35 ). The most common skin finding in FMF is erysipelas-like erythema that is typically noted on the dorsum of the foot (24) ( Figure 2A ).…”

Section: Familial Mediterranean Fevermentioning

confidence: 99%

Autoinflammatory Diseases in Childhood

Yıldız¹,

Haşlak²,

Adroviç³

et al. 2020

Balkan Med J

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Autoinflammatory diseases are characterized by recurrent fevers and clinical findings of impaired natural immunity and can involve various organ systems. The concept of autoinflammatory disease emerged after the definition of familial Mediterranean fever and tumor necrosis factor receptor-associated periodic syndrome. This new disease group was considered to differ from the standard concept of autoimmune diseases, which is relatively better known in terms of basic features, such as defects in innate immunity and the absence of antibodies. A better understanding has been achieved regarding the genetic and pathogenetic mechanisms of this relatively new disease group over the past 20 years since they were first diagnosed, which have led to some changes in the concept of autoinflammatory diseases. The recent definition classifies autoinflammatory disease to be a wide range of diseases with different clinical features, mainly accompanied by changes in innate immune and rarely in humoral immunity. The spectrum of autoinflammatory diseases is rapidly expanding owing to recent developments in molecular sciences and genetics. This review article discusses the clinical features, classification criteria, treatment options, and long-term prognosis of periodic fever, aphthous stomatitis, pharyngitis, adenitis syndrome, and other common autoinflammatory diseases in the light of current literature.

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Section: Familial Mediterranean Fevermentioning

confidence: 99%

Autoinflammatory Diseases in Childhood

Yıldız¹,

Haşlak²,

Adroviç³

et al. 2020

Balkan Med J

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“…8 In our cohort of patients with FMF, 10.2% of children also fulfilled classification criteria for JSpA. 8 Moreover, chronic recurrent multifocal osteomyelitis (CRMO) was suggested to be an unusual form of SpA by several authors and Vittecoq et al 9 showed that some of the patients with…”

Section: O R R E S P O N D E N C Ementioning

confidence: 63%

“…Therefore, enthesitis‐related arthritis can be seen as an early presentation of JSpA. In addition to mentioned diseases, it is also shown that clinical features of JSpA can be seen in the course of familial Mediterranean fever (FMF) and Mediterranean fever gene mutation may be a susceptibility factor for enthesitis‐related arthritis especially in regions endemic for FMF 8 . In our cohort of patients with FMF, 10.2% of children also fulfilled classification criteria for JSpA 8 .…”

mentioning

confidence: 58%

“…In addition to mentioned diseases, it is also shown that clinical features of JSpA can be seen in the course of familial Mediterranean fever (FMF) and Mediterranean fever gene mutation may be a susceptibility factor for enthesitis‐related arthritis especially in regions endemic for FMF 8 . In our cohort of patients with FMF, 10.2% of children also fulfilled classification criteria for JSpA 8 . Moreover, chronic recurrent multifocal osteomyelitis (CRMO) was suggested to be an unusual form of SpA by several authors and Vittecoq et al 9 showed that some of the patients with CRMO developed SpA.…”

mentioning

confidence: 99%

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Comment on: The conundrum of juvenile spondyloarthritis classification: Many names for a single disease? Lesson learned from an instructive clinical case

et al. 2020

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“…In the study conducted by Gülhan et al ( 23 ), on the other hand, it was reported that the frequency of MEFV mutations was increased in patients with HLA B27-negative ERA, and this might be one of the genetic factors. In addition, it was stated that FMF, which is observed commonly in our country, might overlap with juvenile spondyloarthropathy at the same time ( 20 ). In our study, there were seven patients who were diagnosed as having both FMF and ERA, and possessed significant mutations.…”

Section: Discussionmentioning

confidence: 93%

Türkiye İç Batı Anadolu bölgesinde jüvenil idiyopatik artrit

Yener¹

2019

Turk Pediatri Ars

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Aim: To demonstrate the demographic data, subgroup distributions, responses to treatment and outcomes of long-term follow-up in patients who were followed up and treated in our clinics with a diagnosis of juvenile idiopathic arthritis, and to compare these data with national and international data. Material and Methods: The files of 116 patients who had been diagnosed as having juvenile idiopathic arthritis, were initiated on treatment and presented for regular follow-up visits between January 2012 and January 2018, were examined. Their demographic findings, treatments, active/inactive disease states (on-medication and off-medication) and treatment response states were evaluated. Results: According to the International League of Associations for Rheumatology criteria, the subtypes were specified as enthesitis-related arthritis (n=38), oligoarticular (n=37), rheumatoid factor (-) polyarticular (n=17), systemic (n=15), rheumatoid factor (+) polyarticular (n=5), and psoriatic juvenile idiopathic arthritis (n=4). In total, the female/male ratio was found to be 1.5. The mean delay time between the first complaint and the diagnosis was found to be 5.7±5.2 months. The patients with systemic type were diagnosed at the earliest, while the patients with polyarticular and enthesitis-related subtypes were diagnosed at the latest. Thirty-two percent of the patients were treated with methotrexate alone, and 38% were given additional biologic drugs. In both treatment groups, the time to achieve inactive disease was the shortest in the oligoarticular group and the longest in the enthesitis-related arthritis group. In the study period, 38 patients were in remission off-medication (the highest rate (53.3%) was observed in the systemic group) and 71 patients were in remission on-medication (the highest rate (70.2%) was observed in the oligoarticular group). Remission was obtained in 94% of the patients. Conclusion: Enthesitis which is the remarkable finding of enthesitis-related arthritis, should not be overlooked in routine physical examination. Awareness of enthesitis can contribute to the prevention of diagnostic delay in children with enthesitis-related arthritis.

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THU0268 The frequency of juvenile spondyloarthropathies in childhood famillial mediterranean fever

Cited by 14 publications

References 0 publications

Autoinflammatory Diseases in Childhood

Autoinflammatory Diseases in Childhood

Comment on: The conundrum of juvenile spondyloarthritis classification: Many names for a single disease? Lesson learned from an instructive clinical case

Türkiye İç Batı Anadolu bölgesinde jüvenil idiyopatik artrit

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