Haley Minnehan MD Adrenal Insufficiency Definition A disease state that is caused by lack of glucocorticoidsGC andor mineralcorticoids MC by interruption at any level of the hypothalamuspituitaryadrenal axis ID: 545953
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Slide1
Adrenal Insufficiency
Haley
Minnehan
, MDSlide2
Adrenal Insufficiency Definition
A disease state that is caused by
lack of glucocorticoids(GC) and/or
mineralcorticoids
(MC)
by interruption at any level of the hypothalamus-pituitary-adrenal axis.Slide3
Hypothalamus-Pituitary-Adrenal AxisSlide4
Adrenal Insufficiency
Primary?
Secondary?
Tertiary?Slide5
Primary(
Addisons
)
Destruction
Of the Adrenal
CortexSlide6
Primary=Primary Gland Failure = No cortisol or MC
1. Autoimmune
>Most Common
>Lymphocytic infiltration destroys entire adrenal cortex
>Antibodies are detectable
2. Infectious
TB
CMV
Histoplasmosis
3. Drugs
Ketoconazole
Rifampin
EtomidateSlide7
Primary Causes continued…..
4.Deposition Diseases
Sarcoidosis
Amyloidosis
Hemochromatosis
5. Metastatic Disease
6. Congenital Adrenal Hyperplasia
7. Adrenal Surgery
8. Bilateral adrenal gland hemorrhageSlide8
Secondary= Lack of ACTH
=Pituitary ProblemSlide9
Secondary Causes
Autoimmune -
Lymphocytic
hypophysitis
Postpartum hemorrhage-Sheehan’s syndrome
Head Trauma
Tumor
Infiltrative Process
Pituitary surgerySlide10
Tertiary Causes
1
. Exogenous
Glucocorticoids
Prolonged
therapy that is withdrawn
2.
Megestrol
Has
some GC
properties
so will affect the axis
3. Opiates
Affects
the axis at the CRH level.Slide11
What does cortisol do?
MAINTAINS GLYCOGEN (cause of hypoglycemia)
REGULATES IMMUNE FUNCTION (more infections)
PART OF HEPATIC NEOGENESIS (gut absorption, nausea, diarrhea, vomiting)
VASCULAR TONE (cause of hypotension)Slide12
What does MC =Aldosterone do?
Regulates Na and K at the level of the kidney/ renin-angiotensin system
(Very little aldosterone secretion comes from ACTH stim)
Lack of aldosterone as in Primary AI
a) renal wasting of Na
b) retention of K
c)volume loss >>severe intravascular depletion>>>
hyptotension
and shockSlide13
Renin-Angiotensin-Aldosterone LoopSlide14
What does AI feel like?Slide15
Chronic AI Symptoms
GI
Unexplained abdominal pain
Weight loss
Chronic Nausea
Constipation
Vomiting
CNS
Headache
Cognitive clouding
Hypersomnia
Depression
Anxiety
OTHER
Fatigue
Weakness
Chills
Recurrent
infxns
Tan skin (Primary)
HypotensionSlide16
Normal vs SAI Cortisol PatternSlide17
JFK hadAddisons
Hyperpigmented
Chronically ill
How sick was JFK?Slide18
Acute AdrenalCrisis
Always be in your differential of SHOCK
Look
foR
:
1.Hypoglycemia
2. Acidosis
3. Hyponatremia
4. Hyperkalemia
Primary=
low NA, High KSlide19
ACUTE ADRENAL CRISIS CAUSES
#1 IS
gi
FROM VOMITING/DIARRHEA
SURGERY
HEAT
EMOTIONAL DISTRESS
TRAUMA
PREGNANCY
INFECTIONSlide20
ACUTE ADRENAL CRISIS TREATMENT
STEROIDS AND FLUIDS
100 mg IV Hydrocortisone stat then Q8 hours x 24 hours
Normal Saline
Correct hypoglycemia D5 with 100 mg IV HC if neededSlide21
Laboratory Diagnosis 8-9 am cortisol with ACTH
Cortisol Level:
>18 mcg/dl excludes AI
<3 is virtually diagnostic
< or =10 is suggestive and should start therapy before get further testing
11-18 hold therapy and obtain ACTH stim test (
cosyntropin
)
Sepsis workup without AI baseline cortisol < or = to 10 think AI
ACTH Level:
>or = to 22 is virtually diagnostic of primary AISlide22
ACTH STIM TEST (Cosynotropin)
Give 250 mcg IV
Cosynotropin
Measure serial cortisol serum levels at 30 and 60 minutes
Cortisol < 5 = Adrenal Failure
Cortisol >20 = Normal
Cortisol 5-20 = Pituitary FailureSlide23
Goals of Treatment
#1
Physiological
Replacement
Of GC/MC
#2
Improve QOL
#3
Prevent
Adrenal
CrisisSlide24
Glucocorticoid Replacement Options
Hydrocortisone
Prednisone
dexamethasoneSlide25
Glucocorticoids are not all created equal
None are ideal in avoidance of risks of over or under replacement
Most physiological =
hc
Prednisone potency= 4xHC
Dexamethasone=no effect on vascular tone last 24-36
hrsSlide26
Chronic AI Treatment
Primary
Hydrocortisone 20-30 mg/day
Most physiologic
Divide doses up to TID
10/5/5=7am/12 pm/5 pm
Fludocortisone
0.05-0.1 mg/day
Secondary
Hydrocortisone 10-25 mg/day
Divide doses up to TID
7.5/5/2.5 (all combinations)
No need for
fludocortisone
because aldosterone not under ACTH influenceSlide27
Chronic AI Treatment cont…
Tertiary
Removal of the offending drug with a taper if possible (reversible)
Treat with maintenance HC if not able to d/c drug
As little as 20 mg/day prednisone for 7 days can cause AI
Be suspicious in asthmatics, COPD, rheumatology ptsSlide28
Hydrocortisone
GOOD
Restores physiology and relieves some symptoms
Nausea, weakness, headache, abdominal pain, hypotension
Allowed longer life span of AI patients
Prior to GC pts lived max 2 years after diagnosis
BAD
Cannot replace the physiology-circadian rhythm
ie
TIMING
Side effects: depression, irritability, insomnia, bone metabolism
Short acting- peaks at 1-2 hours then rapid decline until next dose at
hr
4-5
Absorption rate variableSlide29
Treatment is Challenging
Physiological demands change throughout the day depending on the day.
No objective measurement of cortisol that is “normal” for that individual.
Ideal world =
fingerstick
rapid test so with symptoms know what to treat
Symptoms checklist:
bp
, fluids, low blood sugar, stress not
accouneted
for, infection, sleep deprivedSlide30
Treatment is often trial and error
Despite treatment morbidity is high and life expectancy is reduced
WHY? Non-physiological nature of replacement therapy
Compliance with twice-three times daily dosing is difficult
Overexposure to GC> 30 mg per day=Cardiovascular Complications, Osteoporosis, Infections, Glucose Intolerance, Insomnia, Obesity
Underexposure= Infections, adrenal crisis, feel “post call” all the time, hypersomnia, significant impairment in physical, emotional and cognitive functioning affecting work, family, social Slide31
Stress Dosing!
Ideal to “anticipate” the upcoming stressful event
Travel, holidays, call, prolonged exercise (football game), emotional distress, illness, surgery, pregnancy
Double or Triple the maintenance dose for 3 days then taper for 3 days until reach maintenance doses
Challenging- again
How much?
How long?Slide32
Stress dose for prolonged exerciseSlide33
Patient and Family Education on Management
Life threatening disease that requires lifelong replacement
Educate and Reiterate the importance of stress dosing and how to avoid v adrenal crisis
ID Emergency bracelet>>”Adrenal Failure- Need steroids”
Emergency kit with 100 mg HC vials, needles, syringes for vomiting/diarrhea or other illness and cannot keep oral down
Give
Prevnar
and Pneumovax to prevent recurrent sinusitis/bronchitisSlide34
Despite treatment….
Patients report poor QOL
Worldwide survey of 1245 pts:
64%
reported compromised health status
40%
missed school or work in last 3 months
38%
hospitalized in last
yr
25%
DisabilitySlide35
Patients need support
Support groups
NADF (National adrenal disease foundation)
Website:
www.nadf.org
provides information on emergency kits, patient information, newsletter and online support group- inspire.com
Quality of Life Discussions:
stress, exercise, routine, dosing compliance, sleep (no dosing after 6 pm), relationships, family life, work
Journaling
dosing times,
bp
, hours of sleep, stress
Slide36
What is in the Pipeline?
Cortisol pumps
Long acting/IR CortisolSlide37
Adrenal Insufficiency after 4 years of TreatmentSlide38
Thank you IAFP.