Isolated Recurrent Pleuritis Revealing Familial Mediterranean Fever in Adulthood | Zendy

JeanChristophe Lega | Zendy; C. Khouatra | Zendy; Vincent Cottin | Zendy; J.F. Cordier | Zendy

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Isolated Recurrent Pleuritis Revealing Familial Mediterranean Fever in Adulthood

Author(s) -

JeanChristophe Lega,

C. Khouatra,

Vincent Cottin,

J.F. Cordier

Publication year - 2009

Publication title -

respiration

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 1.264

H-Index - 81

eISSN - 1423-0356

pISSN - 0025-7931

DOI - 10.1159/000272314

Subject(s) - familial mediterranean fever , medicine , mefv , colchicine , pyrin domain , peritonitis , fever of unknown origin , arthritis , pleurisy , disease , immunology , pediatrics , mutation , gene mutation , gene , pleural effusion , genetics , inflammation , inflammasome , biology

Familial Mediterranean fever (FMF) is a genetic autoinflammatory disease especially affecting populations of Mediterranean origin with an autosomal recessive inheritance. The cardinal manifestations consist of short febrile and painful attacks of peritonitis, arthritis and pleuritis developing during childhood. We report the case of a 26-year-old man of Tunisian descent who had febrile episodes of right-sided pleuritis without any extrathoracic complaints. Disappearance of attacks with one dose of colchicine (1 mg/day) strengthened the presumptive diagnosis of atypical FMF, which was further confirmed by genetic testing identifying the homozygous mutation M694I/M694I of the MEFV gene.

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