Evaluation of the structure, autoimmunity, and functions of the thyroid gland in familial Mediterranean fever patients

ABSTRACT Objective Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder that is frequently seen in the eastern Mediterranean region. The thyroid gland can be affected in FMF patients through autoimmunity or amyloidosis. Here, we aimed to evaluate the structure and functions of the thyroid gland in addition to possible autoimmunity in FMF patients. Subjects and methods The study was conducted by the Endocrinology and Metabolism and Internal Medicine Departments. Thirty FMF patients and 30 age and gender-matched healthy controls were enrolled in the study. Free thyroxin (fT4), free triiodothyronine (fT3), thyroid-stimulating hormone (TSH), and anti-thyroid peroxidase (anti-TPO) autoantibodies were investigated. Detailed thyroid grayscale and Doppler Ultrasonography examinations and shear-wave elastosonography (SWE) were performed in the patient and control groups. Results Anti-TPO was detected in 24% (n = 7) of the patients. On the grayscale US, mean thyroid volumes were similar between the FMF and the control groups (p > 0.05). By Doppler US, thyroid vascularity observed was detected in 10.3% (n = 3) of the patients. SWE revealed that the mean velocity value of right vs. left lobe in the patient group was 1.77 ± 0.45 m/s and 1.95 ± 0.51 m/s, respectively. Compared to the control group, the mean velocity values were significantly higher in the right (p = 0.004) and left (p = 0.01) lobes of the patient group. The mean stiffness value in the patient group was also significantly higher in the right and left lobes [10.13 ± 5.65 kPa (p = 0.005) and 12.24 ± 6.17 kPa (p = 0.02), respectively]. Conclusion Recognizing the complications of FMF early in the course of the disease is as important as the early diagnosis of the disorder. Based on this, thyroid functions and changes in its structure should be evaluated carefully for early diagnosis of a possible coexisting thyroid disorder. Arch Endocrinol Metab. 2020;64(1):66-70

Síndromes autoinflamatorios / Autoinflammatory syndromes

Los síndromes autoinflamatorios (SAI) se caracterizan por periodos recurrentes de inflamación no mediada por anticuerpos ni linfocitos T y sin desencadenantes conocidos. Investigaciones acerca de alteraciones en la regulación del inflamasoma, la producción anómala de interleukina-1 beta (IL - 1B) y el rol del factor de necrosis tumoral alfa (TNF-a) que ocurre en algunas de estas enfermedades, han permitido ampliar el conocimiento sobre los mecanismos de activación de la inmunidad innata y el uso de terapias biológicas como alternativas de tratamiento.

Autoinflammatory syndromes (AIS) are characterized by recurrent periods of inflammation, not mediated by antibody and T lymphocytes and without triggers known. Investigations about alterations in regulation of inflammasome, abnormal production of interleukin-1 beta (IL - 1b) and the role of tumor necrosis factor alpha (TNF-a) that occurs in some of these diseases, has improved knowledge about the mechanisms of activation of innate immunity and the use of biological therapies such as treatment options.

Phagocytic activity in familial Mediterranean fever

Familial Mediterranean fever (FMF) is an autosomal recessive disease. Although the possibility of multiple immunologic mechanisms have been studied, the actual mechanism is still unresolved. Forty-one patients with FMF (24 males and 17 females with a mean age and disease duration of 17.8 +/- 4.1 and 4.7 +/- 2.3 years, respectively) and 14 healthy controls (10 males and 4 females with a mean age 23.2 +/- 5.1) were involved in the study. A phagotest was studied in both the patients and control groups with a FACScalibur Flow. All patients were in the acute stages of the disease and had not undergone colchicine treatment for 2 months. The percentage blood phagocytic activity of both granulocytes and monocytes were 84.23 +/- 8.76 and 67.28 +/- 10.15 in the patient group and 94.68 +/- 3.24 and 76.23 +/- 5.7 in the control group, respectively. There was no statistically significant difference in the percentage of phagocytic activity of the granulocytes and monocytes between the FMF patients and healthy controls (p > 0.05 and p > 0.05, respectively).