A new syndrome of amenorrhea in association with hypergonadotropism and apparently normal ovarian follicular apparatus

Adrenal and ovarian autoimmunity

“…Two distinct entities are identifiable: (i) autoimmune oophoritis and (ii) resistent ovary syndrome, also called Savage's syndrome (84).…”

“…These data indicate that the absence of lymphocytic oophoritis cannot be regarded as conclusive evidence against an autoimmune aetiology, as this could equally be due to immunological mechanisms, such as blocking antibodies hypothesized for Savage's syndrome. This syndrome is characterized by amenorrhea in association with hypergonadotropism and apparently normal ovarian follicular apparatus with immature follicles (84).…”

Current evaluation of amenorrhea

“…In premature ovarian failure (POF), amenorrhea, persistent estrogen deficiency, and elevated FSH levels occur prior to the age of 40, and this condition affects 1% to 5% of women (22,23). Iatrogenic causes of POF, such as chemotherapy and radiation therapy for malignancy, have a potential for recovery.…”

Premature Ovarian Failure

“…In 1969, Jones and de Moraes-Ruehsen were the first to report on three female patients with ROS; based on the first patient's surname, they called it "Savage syndrome" [19]. Other authors have also described a similar combination of symptoms, such as numerous primordial follicles present in the ovary, hypergonadotropic hypoestrogenism, and decreased sensitivity even to high-dose gonadotropin used in patients both with primary and secondary lack of menses, in order to induce ovulation [20,21].…”

“…Autoreactive T lymphocytes cause damage to the gland tissue. Patients with autoimmune thyroid diseases (Graves' disease and Hashimoto's disease) have been shown to have increased amounts of macrophage cells and NK cells with MHC class II molecules (histocompatibility complex class II) in the thyroid gland [19,20], and increased IgG levels in blood [35,36].…”