Attacks of pericarditis as a manifestation of familial Mediterranean fever (FMF)

Kees, Salim; Langevitz, Pnina; Zemer, Deborah; Padeh, Shai; Pras, Mordechai; Livneh, Avi

doi:10.1093/qjmed/90.10.643

Cited by 118 publications

(59 citation statements)

References 16 publications

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“…Recurrent pericarditis episodes are seen in 0.5% of the patients with FMF and it causes retrosternal pain and ST segment abnormality on ECG. Pericardial episodes rarely cause tamponade and constructive pericarditis (38,(43)(44).…”

Section: (C) Pleural Episodementioning

confidence: 99%

Familial Mediterranean Fever

Küçük

Gezer²,

Uçar

et al. 2014

Acta Med. (Hradec Kralove, Czech Repub.)

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Familial Mediterranean Fever is an autosomal recessive inherited disease with a course of autoinflammation, which is characterized by the episodes of fever and serositis. It affects the populations from Mediterranean basin. Genetic mutation of the disease is on MEFV gene located on short arm of Chromosome 16. The disease is diagnosed based on clinical evaluation. Amyloidosis is the most important complication. The only agent that decreases the development of amyloidosis and the frequency and severity of the episodes is colchicine, which has been used for about 40 years. In this review, we aimed to discuss especially the most recent advances about Familial Mediterranean Fever which is commonly seen in our population.

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Section: (C) Pleural Episodementioning

confidence: 99%

Familial Mediterranean Fever

Küçük

Gezer²,

Uçar

et al. 2014

Acta Med. (Hradec Kralove, Czech Repub.)

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“…[1][2][3] More recently, the drug has been used successfully for the prophylaxis of familial Mediterranean fever attacks 4,5 and the treatment of recurrent pericarditis. 6 -13 …”

mentioning

confidence: 99%

Colchicine in Addition to Conventional Therapy for Acute Pericarditis

et al. 2005

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Background-Colchicine is effective and safe for the treatment and prevention of recurrent pericarditis and might ultimately serve as the initial mode of treatment, especially in idiopathic cases. The aim of this work was to verify the safety and efficacy of colchicine as an adjunct to conventional therapy for the treatment of the first episode of acute pericarditis. Methods and Results-A prospective, randomized, open-label design was used. A total of 120 patients (mean age 56.9Ϯ18.8 years, 54 males) with a first episode of acute pericarditis (idiopathic, viral, postpericardiotomy syndromes, and connective tissue diseases) were randomly assigned to conventional treatment with aspirin (group I) or conventional treatment plus colchicine 1.0 to 2.0 mg for the first day and then 0.5 to 1.0 mg/d for 3 months (group II). Corticosteroid therapy was restricted to patients with aspirin contraindications or intolerance.

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“…Pleurite recorrente causando dor torácica ocorre em 39% dos pacientes, enquanto que pericardite é raramente descrita (1 a 2,4%) 27,30 . A manifestação cutânea mais comum e específica da FFM é o eritema erisipeloide (7 a 40% dos casos) 16,19 , caracterizado por lesões eritematosas, vesiculares e bolhosas, principalmente em membros inferiores 16 .…”

Section: Duraçãounclassified

Pediatric hereditary autoinflammatory syndromes

et al. 2010

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Objective: To describe the most prevalent pediatric hereditary autoinflammatory syndromes. Sources:A review of the literature including relevant references from the PubMed and SciELO was carried out using the keywords autoinflammatory syndromes and child. Summary of the findings:The hereditary autoinflammatory syndromes are caused by monogenic defects of innate immunity and are classified as primary immunodeficiencies. These syndromes are characterized by recurrent or persistent systemic inflammatory symptoms and must be distinguished from infectious diseases, autoimmune diseases, and other primary immunodeficiencies. This review describes the epidemiological, clinical and laboratory features, prognosis, and treatment of the main autoinflammatory syndromes, namely: familial Mediterranean fever; TNF receptor associated periodic syndrome; the cryopyrinopathies; mevalonate kinase deficiency; pediatric granulomatous arthritis; pyogenic arthritis, pyoderma gangrenosum and acne syndrome; Majeed syndrome; and deficiency of interleukin 1 receptor antagonist. The cryopyrinopathies discussed include neonatalonset multisystem inflammatory disease (also known as chronic infantile neurologic, cutaneous and articular syndrome) Muckle-Wells syndrome, and familial cold autoinflammatory syndrome. Conclusions:Pediatricians must recognize the clinical features of the most prevalent autoinflammatory syndromes. Early referral to a pediatric rheumatologist may allow early diagnosis and institution of treatment, with improvement in the quality of life of these patients. J Pediatr (Rio J). 2010;86(5):353-366:Autoinflammatory syndromes, child, familial Mediterranean fever, cryopyrinopathies, TRAPS, NOMID. ResumoObjetivo: Descrever as principais síndromes autoinflamatórias hereditárias na faixa etária pediátrica. Fontes dos dados:Foi realizada uma revisão da literatura nas bases de dados PubMed e SciELO, utilizando as palavras-chave "síndro-mes autoinflamatórias" e "criança", e incluindo referências bibliográficas relevantes. Síntese dos dados:As principais síndromes autoinflamatórias são causadas por defeitos monogênicos em proteínas da imunidade inata, sendo consideradas imunodeficiências primárias. Elas são caracterizadas clinicamente por sintomas inflamatórios sistêmicos recorrentes ou contínuos e devem ser diferenciadas das doenças infecciosas, autoimunes e outras imunodeficiências primárias. Nesta revisão, foram enfatizadas características epidemiológicas, manifestações clínicas, alterações laboratoriais, prognóstico e terapia das principais síndromes autoinflamatórias: febre familiar do Mediterrâneo; síndrome periódica associada ao receptor de fator de necrose tumoral; criopirinopatias; deficiência de mevalonato-quinase; artrite granulomatosa pediátrica; síndrome de pioderma gangrenoso, artrite piogênica e acne; síndrome de Majeed; e deficiência do antagonista do receptor de interleucina-1. As criopirinopatias discutidas foram: doença inflamatória multissistêmica de início neonatal ou síndrome neurológica, cutânea e articu...

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Attacks of pericarditis as a manifestation of familial Mediterranean fever (FMF)

Cited by 118 publications

References 16 publications

Familial Mediterranean Fever

Familial Mediterranean Fever

Colchicine in Addition to Conventional Therapy for Acute Pericarditis

Pediatric hereditary autoinflammatory syndromes

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