Primary ovarian insufficiency due to steroidogenic cell autoimmunity is associated with preserved pool of functioning follicles

Context: Primary ovarian insufficiency (POI) is defined as hypergonadotropic amenorrhea before the age of 40 yr. In 4–5% of patients with POI, an ovarian autoimmune process is present. Design: Serum concentrations of antimu¨ llerian hormone (AMH) have been determined in 26 women with POI due to steroidogenic cell autoimmunity (SCA-POI), 66 with nonautoimmune idiopathic POI (iPOI), 40 postmenopausal women (PMW), and 44 healthy fertile women (HW). SCA-POI was diagnosed according to presence of steroidogenic enzyme autoantibodies (17alpha-hydroxylase, side chain cleavage, and 21-hydroxylase autoantibodies). Results: AMH concentrations were significantly higher in women with SCA-POI than women with iPOI (P=0.018) orPMW(P=0.03) but significantly lower than HW(P<0.0001).AMH was detected in 11 of 26 women with SCA-POI (42%) and seven of 66 with iPOI (11%) (P = 0.002). Serum concentrations above the fifth percentile of the normal range (0.6 ng/ml) were detected in nine of 26 women with SCA-POI (35%) and four of 66 with iPOI (6%) (P = 0.001). Eight of 12 women with SCA-POI with less than 5 yr (67%) and one of 14 with longer disease duration (7%) had AMH concentrations within the normal range (P=0.003). AMH concentrations correlated inversely with disease duration in women with SCA-POI (rho=-0.563, P=0.003) but not women with iPOI. AMH correlated inversely with FSH serum concentrations in HW(rho=-0.584, P=0.001) but not PMW or women with POI. Conclusions: Two thirds of women with recent-onset SCA-POI had normal AMH concentrations. Women with SCA-POI, differently from those with iPOI, present a preserved ovarian follicle pool for several years after diagnosis of ovarian insufficiency.