Hirsutism, Hyperandrogenism, PCOS, Amenorrhea Flashcards
hodgepotch of syndromes with hirsutism and amenorrhea
a progestin withdrawal test does what
induces mentrual bleeding in pts with amenorrhea if there’s not enough estrogen to prime the endometrium. They are given medoxyprogestrone for 5-10 days.
causes of hirsutism in women
PCOS
idiopathic hirsutism
non classic 21 hydroxylase deficiency
androgen secreting ovarian tumor,
ovarian hyperthecosis
Cushings syndrome
features of PCOS
and labs
oligomenorrhea,
hyperandrogenism,
obesity associated with DM2,
dyslipidemia,
obesity
and HTN
LABS: total testosterone will not be <150 or and DHEA-S is <1000
features of idiopathic hirsutism
normal menstruation normal serum androgens
non classic 21 hydroxylase deficiency clinical features
similar to PCOS elevated serum 17 hydroxyprogesterone
androgen secreting ovarian tumors and ovarian hyperthecosis clinical features
more common in post menopausal women and rapidly progressive hirsutism with virilization very high serum androgens
seen in women >30 years
see total testosterone >150-200 DHEA-S level >2000
Cushing’s syndrome
obesity (see face, neck, trunk abdomen) increased libido, virilization, irregular menses
hirsutism should be differentiated from
hypertrichosis - increased growth of light unpigmented hair in nonsexual areas that can be due to systemic conditions like hypothyroidism, medications (phenytoin).
Can see increased facial hair that is seen in certain ethnicities (Hispanic) which can be normal variant.
All women with hirsutism should get these labs:
serum total testosterone level to evalute underlying androgen disorder.
- may need 17 hydroxyprogesterone level depending if women has virilization and irregular menses.
when do we get a pelvic ultrasound in the evaluation of hirsutism in women?
Very high testosterone >150 should get pelvic ultrasound to rule out androgen secreting ovarian tumor.
hirsutism is
excess terminal hair growth in androgen dependent areas
(chin upper lip, upper abdomen, chest and back)
causes of hirsutism in women are:
PCOS idiopathic hirsutism non classic 21 hydroxylase deficiency androgen secreting ovarian tumor, ovarian hypothecosis Cushing’s syndrome
clinical features of PCOS?
oligomenorrhea,
hyperandrogenism,
obesity associated with DM2, HLD, and HTN
clinical features of idiopathic hirsutism?
normal menstruation normal serum androgens see body hair
clinical features of nonclassical 21 hydroxylase deficiency
similar to PCOS elevated serum 17 hydroxyprogesterone
clinical features of androgen secreting ovarian tumor, ovarian hyperthecosis
more common in post menopausal women rapidly progressive hirsutism with virilization very high serum androgens
clinical features of cushings dx
obesity (usually of the face, neck, trunk and abdomen) increased libido, virilization irregular menses
virilization in women where is it seen?
frontal balding, voice deepening and clitoromegaly seen in androgen producing tumors of ovary or adrenal and non classical (late onset) congenital adrenal hyperplasia (CAH)
triad of obesity, oligomenorrhea, hirsutism: think:
PCOS but other causes of hirsutisim and virilization should be ruled out by getting LH, FSH and prolactin and 17 hydroxyprogesterone level (to rule out androgen producing tumor or congenital adrenal hyperplasia)
non classical (late onset) congenital adrenal hyperplasia (CAH) presentation
see deficiency in 21 hydroxylase enzyme deficiency. Pts have androgen excess with premature pubarche, acne, accelerated bone age, hirsuitism, mentrual irregularities in childhood and early adulthood. Usually no cortisol deficiency (unlike classic early onset CAH).
who gets non classical (late onset) congenital adrenal hyperplasia (CAH)
Seen in younger pts teens and early adulthood Commonly seen in Ashkenazi Jewish, Italian, Slavic and hispanic pts.
diagnosis of non classical (late onset) congenital adrenal hyperplasia (CAH)
elevated 17 hydroxyprogesterone basal levels >200 and confirmed by significant increase in 17 hydroxyprogesterone to >1000 after high dose ACTH (250 ug) administration
when do we suspect androgen secreting tumors and what do we order to screen for it?
androgen secreting tumors are post menopausal women who get them will see elevated Testosterone level get a pelvic ultrasound.
androgen excess, oligoovulation or anovulation obesity polycystic ovaries on U/S
clinical features of PCOS
female pt with irregular menses to anovulation, who has acne, male patterned baldness, and mustasche hair, and is obese, should suspect:
PCOS - not rapid onset of androgenic hirsutism so don’t think of androgen secreting tumor.
Rather PCOS is a diagnosis of exclusion.
pathophysiology behind PCOS is
See GnRH pulses and so see more LH being made which decreases FSH production. See excess LH and not enough FSH which results in excessive ovarian androgen production and ovulatory dysfunction. High androgens causes hirsutism. Also see insulin resistance which also increases estrogen production and prevents ovulation.
Labs: total testosterone will be elevated but <150. DHEAS will be elevated. LH:FSH 2:1 or imbalanced.
When treating PCOS, OCPs decrease free or bioavailable testosterone levels (via increased sex hormone binding globulin) and suppresses LH secretion resulting in reduced ovarian testosterone production
what other medical conditions are often seen with PCOS
metabolic syndrome (diabetes, HTN)
OSA
NASH
Endometrial hyperplasia and cancer
Treatment options for PCOS
for hirsutism
for anovulation
weight loss (First line)
OCP’s for menstrual regulation progestins for endometrial protection and will help with hirsutism (can take up to 6 months)
metformin can help with induction of ovulation (NOT used for treating hirsutism)
clomiphene citrate for ovulation induction
If no improvement in hirsutism with OCPs and it’s been >6 months, can try spironolactone.
Treatment goal is to improve hyperandrogenism symptoms, prevent associated comorbidities, and attempt ovulation induction in women who want to get pregnant.
What is the minimum criteria to diagnose PCOS empirically (Rotterdam criteria)
irregular or absent ovulation (irregular menses, anovulation)
hyperandrogenism
—clinical based on signs of hirsuitism (acne or hirsutism)
—biochemical (elevated testosterone levels) of hyperandrogenism
Polycystic ovaries on ultrasound or in surgery
only need 2/3 to say they have PCOS
what is seen on physical exam with these pts PCOS?
see abdominal striae
metabolic syndrome
hirsutism
management of PCOS involves
improving hirsutism restoring reproductive function managing comorbidities
what is the preferred treatment for oligomenorrhea and hirsutism in pts who do not want pregnancy and have PCOS
OCPs
OCPs i_ncrease sex binding protein levels_ and results decreased free androgen levels They also reduce circulating LH levels and this decreases ovarian androgen production.
If pt with PCOS can’t take OCP what to offer next for treatment
Spironolactone
if pt with PCOS is on OCP and they don’t have an improvement what to do next?
if 6 weeks passes and no improvement in symptoms give spironolactone
what are features that would point to a androgen secreting tumor?
someone who has rapidly progressive hyperandrogenism with marked virilization and see clitoromegaly, voice deepening
what to order for a suspected androgen secreting tumor?
dehydroepiandrosterone sulfate or DHEA-S >150 but >700 ug/dl is highly suggestive. Note: PCOS pts may have modestly elevated DHEA-S level
infertility evaluation should be started after 6 months of unprotected intercourse for a woman who is
woman >35 yrs
infertility evaluation be started after 1 yr of unprotected intercourse in woman
<35 yrs
mid luteal phase serum progesterone level obtained 1 week before menses will show
progesterone >3 is evidence of recent ovulation
Turner’s syndrome
loss of all an X so it’s 45 XO.
diagnosed after 12 years and present with primary amenorrhea and gonadal dysgenesis.
See short stature, neck webbing, hearing loss, aortic coarctation and bicuspid aortic valve.
diagnosis of Turner’s syndrome
karotype analysis.
functional hypothalamic amenorrhea (FHA) is what
this is secondary amenorrhea due to relative caloric deficiency (excessive exercise, weight loss, decreased intake) or stress lack of hypothalamic response results in low FSH level this causes lack of estrogen deficiency and so see thin endometrium and see lack of withdrawal bleeding during medroxyprogesterone actate challenge
long term complications of functional hypothalamic amenorrhea (FHA)
stress fractures early onset osteoporosis secondary to low bone density
Treatment of functional hypothalamic amenorrhea (FHA)
lifestyle changes increased caloric intake and decreased exercise stress reduction and weight gain normalization of estrogen will improve bone density combined OCPs is second line if pts are unwilling to or unable to make lifestyle changes.
evaluation of secondary amenorrhea
Follow the chart.
what to order for someone who develops rapid onset hirsutism and develops irregular menses. See total testosterone >150.
Needs U/S of ovaries to look for androgen secreting tumor.
What is a longterm complication of PCOS?
increased risk for endometrial cancer
but no routine endometrial thickness not recommended.
can we use progestin releasing IUD for long term contraception for women with PCOS?
yes. It provides good endometrial protection. IUDs that don’t have estrogen can’t suppress ovarian testosterone and won’t treat the hirsutism.
how long does it take before women see a difference in PCOS therapy while on OCPs?
can take up to 6 months to see a difference and improvement in hair growth. if fails to reduce needs to get spironolactone therapy.
metformin is no longer a treatment for hirsutism. only can be used for concurrent DM2 and possibly for ovulation induction.
what cancer are PCOS pts at highest risk for?
high risk for endometrial cancer but no screening needed unless they have symptoms
only person who is asymptomatic that needs endometrial cancer screening is Lynch syndrome.
Emergency contraception
PCOS pt who is 36 and has vaginal bleeding and spotting after sex should get:
need to get endometrial cancer screening if >35 yrs with endometrial biopsy if having symptoms
they are at greater risk for endometrial cancer
no screening if no symptoms.
Treatment of hyperanderogenism
mechanical hair removal (threading depilatories, elctrolysis laser, may be adequate for cosmesis in women with idiopathic hirsutism.
1st line pharmacology is: hirsutism is combined hormonal estrogen-progestin oral OCPS and agents that suppress gonadotropin secretion and ovarian androgen production and increase SHBG (sex hormone binding globulin) levels.
Spironolactone can be added and need to add contraception to prevent male fetuses from getting teratogenesis.
Topical eflornithine is also used for unwanted hair growth.