Satoyoshi's Syndrome in an Adult: A Review of the Literature of Adult Onset Cases.

Síndrome de Satoyoshi: Enfermedad multisistémica con respuesta exitosa a tratamiento esteroidal

“…El retraso puberal y la presencia de amenorrea ocurre casi en la totalidad de las pacientes 2,5,6,[8][9][10][11]13,14 . En nuestro caso, el retraso puberal presentaba niveles elevados de gonadotrofinas basales con bajos niveles de estradiol, lo que indica un hipogonadismo hipergonadotropo, planteándose la posibilidad de una ooforitis autoinmune como mecanismo responsable de estos trastornos endocrinológicos.…”

“…La edad de inicio de la enfermedad fluctúa entre los 5 y 19 años 6,11 , existiendo también casos de presentación en adultos 12,13 . La gran mayoría han sido descritos en Japón, afectando a las mujeres en una proporción casi el doble que los hombres 11 .…”

Adult-onset Satoyoshi Syndrome with Prominent Laterality of Clinical Features

“…Painful muscle spasms and alopecia are the most frequent features of Satoyoshi syndrome (1), usually followed by diarrhea (1). Patients who develop this syndrome before 12 years of age often exhibit skeletal abnormalities and growth retardation (2)(3)(4). Other clinical findings include low levels of serum protein and total cholesterol (2), and endocrine disturbances, such as amenorrhea with a tendency toward the development of a hypoplastic uterus and ovaries (1).…”

“…Patients who develop this syndrome before 12 years of age often exhibit skeletal abnormalities and growth retardation (2)(3)(4). Other clinical findings include low levels of serum protein and total cholesterol (2), and endocrine disturbances, such as amenorrhea with a tendency toward the development of a hypoplastic uterus and ovaries (1). Complications of autoimmune diseases, such as myasthenia gravis (5) as well as the presence of autoantibodies (auto-Abs) (2,(6)(7)(8)(9)(10)(11) and responsiveness to treatment with corticosteroids (9,(11)(12)(13) and intravenous immunoglobulin (IVIG), have been reported in patients with Satoyoshi syndrome (7,14), indicating that an autoimmune mechanism may play a role in the onset of this syndrome, although the precise underlying mechanism remain unknown.…”

“…Other clinical findings include low levels of serum protein and total cholesterol (2), and endocrine disturbances, such as amenorrhea with a tendency toward the development of a hypoplastic uterus and ovaries (1). Complications of autoimmune diseases, such as myasthenia gravis (5) as well as the presence of autoantibodies (auto-Abs) (2,(6)(7)(8)(9)(10)(11) and responsiveness to treatment with corticosteroids (9,(11)(12)(13) and intravenous immunoglobulin (IVIG), have been reported in patients with Satoyoshi syndrome (7,14), indicating that an autoimmune mechanism may play a role in the onset of this syndrome, although the precise underlying mechanism remain unknown. The age of onset of Satoyoshi syndrome ranges from 5 to 19 years, with a mean age of 11 years (2, 15); however, adult-onset Satoyoshi syndrome is rare, having so far been reported in only a few patients (2,7,(15)(16)(17).…”