Amyloid goiter in familial Mediterranean fever (IMF): A clinicopathologic study of 10 cases

FMF Amyloidosis is an important etiological factor of end stage renal disea se (ESRD) in Mediterranean Countries. Apart from major target organs as car diovascular, respiratory and gastrointestinal system, endocrine organs can also be involved. We planned to investigate the thyroid involvement in our amyloidosis group. The aim of this study was to determine clinical characte ristics of amyloid goiter in FMF patients and the abnormalities of thyroid function, as well as to identify pathologic characteristics. Twenty-two hem odialysis patients (mean age 34.1 +/- 14 years, range 17-68) whose ESRD sec ondary to FMF amyloidosis were evaluated with physical examination, serum l evels of thyroid hormones, ultrasound examination of thyroid glands, thyroi d syntigraphic studies. Goiter was found in 10 patients (4 male, 6 female) having enlarged neck mass (mean age 35 +/- 14 years, range 23-64). The seru m levels of thyroid hormones and TSH were normal in 4 patients. Other four cases had euthyroid sick syndrome. Only one patient developed tender enlarg ed neck mass with subacute thyroiditis symptoms and one had primary hyperth yroidism. Ultrasound examination showed; hypoecoic nodules in 6 patients di ffuse multinodular enlargement in 4 patients. Thyroid syntigraphic studies revealed hypoactive nodules in 7 patients and hyperactive nodules in 3 pati ents. After the laboratory tests were completed, in 10 patients diagnosis w ere made with fine needle aspiration biopsy. Of 10 patients 5 underwent sub total thyroidectomy. Immunohistochemical evaluation demonstrated the presen ce of amyloid AA immunoreactivity in all cases. In conclusion fine needle a spiration from the thyroid when enlarged is useful in the diagnosis of susp ected amyloidosis, especially since it is a safe, easily performed procedur e. With the help of amyloid goiter diagnosis the patient's prognosis on hem odialysis and with renal transplantation can be predicted. Amyloid goiter m ust be searched in hemodialysis patients especially in Mediterranean Countr ies.