Amyloid goitre in familial Mediterranean fever: report on three patients and review of the literature

Familial Mediterranean fever (FMF) is a hereditary disease, the most threatening complication of which is systemic amyloidosis. The thyroid gland may be asymptomatically involved in most patients with systemic amyloidosis secondary to FMF. However, clinically detectable thyroid goitre is quite rare,...

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Bibliographic Details
Published in:Clinical rheumatology 2002-11, Vol.21 (6), p.497-500
Main Authors: Altiparmak, M R, Pamuk, O N, Pamuk, G E, Apaydin, S, Ataman, R, Serdengeçti, K
Format: Article
Language:English
Subjects:
Adult
Amyloid - metabolism
Amyloidosis - complications
Amyloidosis - metabolism
Amyloidosis - pathology
Familial Mediterranean Fever - complications
Familial Mediterranean Fever - metabolism
Familial Mediterranean Fever - pathology
Goiter - etiology
Goiter - metabolism
Goiter - pathology
Humans
Male
Radionuclide Imaging
Thyroid gland
Thyroid Gland - diagnostic imaging
Thyroid Gland - metabolism
Ultrasonography
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Summary:Familial Mediterranean fever (FMF) is a hereditary disease, the most threatening complication of which is systemic amyloidosis. The thyroid gland may be asymptomatically involved in most patients with systemic amyloidosis secondary to FMF. However, clinically detectable thyroid goitre is quite rare, and until now only nine cases of thyroid goitre secondary to amyloid deposition in FMF have been reported. Of 1,100 FMF patients regularly followed up at our centre, thyroid goitre due to the accumulation of amyloid substance could be detected in only three (0.27%). In this report, we summarise the clinical and laboratory features of these patients. All three patients were euthyroid. Total thyroidectomy was performed for compressive symptoms in one patient and for aesthetic purposes in the other two. In countries with a high prevalence of FMF, such as Turkey, secondary amyloidosis of the thyroid gland should be borne in mind in long-standing FMF patients.
ISSN:0770-3198
1434-9949
DOI:10.1007/s100670200122