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Amyloid goitre in familial Mediterranean fever
Abstract:
Three patients known to suffer from familial Mediterranean fever (FMF), systemic amyloidosis and chronic renal failure developed large amyloid goitres. Amyloid goitre is an extremely rare complication of systemic amyloidosis not previously described in FMF. The clinical and pathological features of these three cases were similar to those previously described in amyloid goitre. In two of the patients abnormalities in thyroid function were consistent with those documented in chronic renal failure. There was evidence of hypothyroidism in a third patient. There was no evidence of amyloid induced dysfunction of other endocrine organs.
Insights
Familial Mediterranean fever (FMF) patients with chronic renal failure can develop rare amyloid goiters. This study describes three such cases, noting thyroid function abnormalities but no other endocrine organ dysfunction.
Area of Science:
- Nephrology
- Endocrinology
- Genetics
Background:
- Familial Mediterranean fever (FMF) is a genetic autoinflammatory disorder.
- Systemic amyloidosis is a known complication of FMF.
- Amyloid goiter is an exceptionally rare manifestation of systemic amyloidosis.
Purpose of the Study:
- To report on three cases of amyloid goiter in patients with FMF and chronic renal failure.
- To describe the clinical and pathological features of these rare cases.
- To investigate thyroid function in these patients.
Main Methods:
- Case series reporting.
- Clinical and pathological assessment of three FMF patients with amyloid goiter.
- Thyroid function tests.
Main Results:
- Three patients with FMF, systemic amyloidosis, and chronic renal failure developed large amyloid goiters.
- Clinical and pathological findings were consistent with previously described amyloid goiters.
- Two patients showed thyroid function abnormalities related to chronic renal failure; one had hypothyroidism.
- No amyloid-induced dysfunction of other endocrine organs was observed.
Conclusions:
- Amyloid goiter is a potential, albeit rare, complication in FMF patients with systemic amyloidosis and chronic renal failure.
- Thyroid function should be monitored in these patients, as abnormalities may relate to renal failure or hypothyroidism.
- Amyloid deposition in the thyroid does not appear to affect other endocrine organs in these cases.