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2014 ; 176
(3
): 373-9
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Gonadal function in males with autoimmune Addison s disease and autoantibodies to
steroidogenic enzymes
#MMPMID24666377
Dalla Costa M
; Bonanni G
; Masiero S
; Faggian D
; Chen S
; Furmaniak J
; Rees Smith B
; Perniola R
; Radetti G
; Garelli S
; Chiarelli S
; Albergoni MP
; Plebani M
; Betterle C
Clin Exp Immunol
2014[Jun]; 176
(3
): 373-9
PMID24666377
show ga
Steroidogenic enzyme autoantibodies (SEAbs) are frequently present and are
markers of autoimmune premature ovarian failure (POF) in females with autoimmune
Addison's disease (AAD). The prevalence and significance of SEAbs in males with
AAD have not yet been defined. We studied the prevalence of SEAbs in a large
cohort of males with AAD and assessed the relationship between SEAbs positivity
and testicular function. A total of 154 males with AAD (mean age 34 years) were
studied. SEAbs included autoantibodies to steroid-producing cells (StCA),
detected by immunofluorescence, and steroid 17?-hydroxylase (17?-OHAbs) and side
chain cleavage enzyme (SCCAbs) measured by immunoprecipitation assays. Gonadal
function was evaluated by measuring follicle-stimulating hormone (FSH),
luteinizing hormone (LH), total testosterone (TT), sex hormone-binding globulin
(SHGB), anti-müllerian hormone (AMH) and inhibin-B (I-B). Twenty-six males, 10
SEAbs((+)) and 16 SEAbs((-)), were followed-up for a mean period of 7·6 years to
assess the behaviour of SEAbs and testicular function. SEAbs were found in 24·7%
of males with AAD, with the highest frequency in patients with autoimmune
polyendocrine syndrome type 1 (APS-1). The levels of reproductive hormones in 30
SEAbs((+)) males were in the normal range according to age and were not
significantly different compared to 55 SEAbs((-)) males (P?>?0·05). During
follow-up, both SEAbs((+)) and SEAbs((-)) patients maintained normal testicular
function. SEAbs were found with high frequency in males with AAD; however, they
were not associated with testicular failure. This study suggests that the
diagnostic value of SEAbs in males with AAD differs compared to females, and this
may be related to the immunoprivileged status of the testis.