ReviewEuropean journal of pediatrics2003
Familial mediterranean fever: revisiting an ancient disease.
Review in European journal of pediatrics, 2003. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 78 citations in OpenAlex.
- Children and Adults with PFAPA Syndrome: Similarities and Divergences in a Real-Life Clinical Setting.Advances in therapy · 2021Article
- Management of Monogenic IL-1 Mediated Autoinflammatory Diseases in Childhood.Frontiers in immunology · 2021Review
- OLT1177, a β-sulfonyl nitrile compound, safe in humans, inhibits the NLRP3 inflammasome and reverses the metabolic cost of inflammation.Proceedings of the National Academy of Sciences of the United States of America · 2018Article
- Investigation of the arterial stiffness and associated factors in patients with familial Mediterranean fever.Anatolian journal of cardiology · 2017Article
- FMF Genotype-phenotype correlation in Iranian Azeri Turks: Association between M694V/R761H mutation and amyloidosis.Iranian journal of basic medical sciences · 2015Article
- MEFV Gene Profile in Northwest of Iran, Twelve Common MEFV Gene Mutations Analysis in 216 Patients with Familial Mediterranean Fever.Iranian journal of medical sciences · 2015Article
- Treating inflammation by blocking interleukin-1 in humans.Seminars in immunology · 2013Review
- The factors considered as trigger for the attacks in patients with familial Mediterranean fever.Rheumatology international · 2013Article
- Application of the new pediatric criteria and Tel Hashomer criteria in heterozygous patients with clinical features of FMF.European journal of pediatrics · 2011Article
- The Eurofever Project: towards better care for autoinflammatory diseases.European journal of pediatrics · 2011Review
- Mediterranean fever (MEFV) gene mutation frequency is not increased in adults with rheumatic heart disease.Clinical rheumatology · 2011Article
- The association of inflammatory bowel disease and Mediterranean fever gene (MEFV) mutations in Turkish children.Digestive diseases and sciences · 2010Article
- Anti-IL-1 treatment for secondary amyloidosis in an adolescent with FMF and Behçet's disease.Clinical rheumatology · 2010Article
- Antibodies directed to cyclic citrullinated peptides in familial Mediterranean fever.Rheumatology international · 2010Article
- MEFV mutations in patients with Familial Mediterranean Fever from the Aegean region of Turkey.Molecular biology reports · 2010Article
- Recurrent febrile syndromes: what a rheumatologist needs to know.Nature reviews. Rheumatology · 2009Review
- Co-existence of Hashimoto's thyroiditis with familial Mediterranean fever: is there a pathophysiological association between the two diseases?Clinical and experimental immunology · 2009Article
- Decreased bone mineral density in adult familial Mediterranean fever patients: a pilot study.Clinical rheumatology · 2008Article
- A very frequent mutation and remarkable association of R761H with M694V mutations in Turkish familial Mediterranean fever patients.Clinical rheumatology · 2008Article
- The efficacy of anakinra in an adolescent with colchicine-resistant familial Mediterranean fever.European journal of pediatrics · 2008Article
Corrections and comments
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Authors and funding
1 author at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
unlabelledFamilial Mediterranean fever (FMF) is an auto-inflammatory disease characterised by periodic attacks of fever and serositis. Recent genetic and epidemiological research have highlighted the importance of this disease. FMF is the most frequent periodic fever syndrome and is transmitted in an autosomal recessive fashion. The disease is caused by mutations in the gene on the short arm of chromosome 16, coding for the protein "pyrin". Pyrin is mainly expressed in neutrophils and monocytes and is among the proteins involved in the interleukin-1 inflammatory pathway. The recurrent attacks of fever are accompanied by severe abdominal pain, arthritis and/or chest pain along with a marked increase in acute phase reactants. Among these, serum amyloid A protein is especially important since it is the precursor of the amyloid A fibrils deposited in secondary renal amyloidosis. Renal amyloidosis has a grave prognosis. Differential diagnosis from other periodic fever syndromes is especially important in western European countries. Among these hyper IgD syndrome is common in Netherlands and the tumour necrosis factor receptor-associated periodic syndrome is especially common among Scottish and Irish families. Mutation analysis of the gene may be helpful in diagnosing FMF; however, if this is not possible, a trial of colchicine is a helpful diagnostic tool. The indications for life-long colchicine treatment should be discussed with the family.
conclusionFamilial mediterranean fever and other auto-inflammatory syndromes should be suspected in children with recurrent febrile attacks. Early diagnosis will save the child from unnecessary work-up and kidney involvement.
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