Familial Mediterranean Fever (recurrent Hereditary Polyserositis) in Children: Analysis of 88 Cases

Overview

Journal Eur J Pediatr

Specialty Pediatrics

Date 1989 Jun 1

PMID 2663516

Citations 13

Authors

H A Majeed

M Barakat

Affiliations

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Abstract

The clinical profile, course and complications of familial Mediterranean fever (recurrent hereditary polyserositis) seen in 88 children over a period of 11 years are presented. Forty eight children (55%) started their illness below the age of 5 years, and the mean age of onset was 4.9 years. Peritonitis occurred in 85% of children, arthritis in 50%, pleuritis in 33% and erysipelas-like lesions in 16%. Two children developed renal amyloidosis, and one third of the children were subjected to unnecessary operative surgery, reflecting the diagnostic difficulties. The arthritis was mono-articular in 80% and polyarticular in 20% of children with arthritis, and was seronegative (rheumatoid factor and antinuclear antibodies). Human leucocyte antigen (HLA) typing for the B-27 antigen carried out in ten children with arthritis was negative. The synovial attack showed a wide variation in the clinical presentation, course and duration of arthritis, causing diagnostic difficulties. The difficulties in the differentiation of recurrent hereditary polyserositis (familial Mediterranean fever) arthritis from the common causes of acute and chronic juvenile arthritis and the seronegative spondyloarthropathies are discussed. Of 45 children treated with colchicine, 42 children (93%) achieved a therapeutic response.

Citing Articles

Treat-to-target strategies for the management of familial Mediterranean Fever in children.

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Interleukin-6 inhibition in the treatment of autoinflammatory diseases.

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Decreased exercise capacity, strength, physical activity level and quality of life in adult patients with familial Mediterranean fever.

Tore N, Sari F, Sarac D, Bayram S, Satis H, Karadeniz H Turk J Med Sci. 2021; 51(4):1712-1718.

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[Results of the systematic literature search as basis for the "Evidence-based treatment recommendations for familial Mediterranean fever patients with insufficient response or intolerability to colchicine" of the Society for Pediatric and Adolescent...].

Sahr T, Kiltz U, Weseloh C, Kallinich T, Braun J Z Rheumatol. 2020; 79(9):943-951.

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An open-label continuation trial of tocilizumab for familial Mediterranean fever with colchicine ineffective or intolerance: Study protocol for investigator-initiated, multicenter, open-label trial.

Koga T, Hagimori N, Sato S, Morimoto S, Hosogaya N, Fukushima C Medicine (Baltimore). 2020; 99(1):e18328.

PMID: 31895769 PMC: 6946311. DOI: 10.1097/MD.0000000000018328.

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