Download Adrenal Insufficiency - Idaho Academy Of Family Physician

Adrenal Insufficiency
HALEY MINNEHAN, MD
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-pituitaryadrenal axis.
Hypothalamus-Pituitary-Adrenal Axis
Adrenal Insufficiency
 Primary?
 Secondary?
 Tertiary?
Primary
(Addisons)
Destruction
Of the
Adrenal
Cortex
Primary=Primary Gland Failure
= No cortisol or MC
1. Autoimmune
2. Infectious
3. Drugs
>Most Common
TB
Ketoconazole
>Lymphocytic
infiltration destroys
entire adrenal cortex
CMV
Rifampin
Histoplasmosis
Etomidate
>Antibodies are
detectable
Primary Causes continued…..
4.Deposition
Diseases
5. Metastatic
Disease
Sarcoidosis
6. Congenital
Adrenal
Hyperplasia
Amyloidosis
Hemochromatosis
7. Adrenal Surgery
8. Bilateral adrenal
gland hemorrhage
Secondary= Lack of ACTH
=Pituitary Problem
Secondary Causes

Autoimmune - Lymphocytic hypophysitis

Postpartum hemorrhage-Sheehan’s syndrome

Head Trauma

Tumor

Infiltrative Process

Pituitary surgery
Tertiary Causes
1. Exogenous
2. Megestrol
3. Opiates
Has some GC
properties so will affect
the axis
Affects the axis at the
CRH level.
Glucocorticoids
Prolonged therapy that
is withdrawn
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)
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 shock
Renin-Angiotensin-Aldosterone Loop
What does AI feel like?
Chronic AI Symptoms
GI

Unexplained
abdominal pain

Weight loss

Chronic Nausea

Constipation

Vomiting
CNS
OTHER

Headache
Fatigue

Cognitive clouding
Weakness

Hypersomnia
Chills

Depression
Recurrent infxns

Anxiety
Tan skin (Primary)
Hypotension
Normal vs SAI Cortisol Pattern
JFK had
Addisons
Hyperpigmented
Chronically ill
How sick was JFK?
ALWAYS BE IN YOUR
DIFFERENTIAL OF SHOCK
LOOK FOR:
Acute Adrenal
Crisis
1.HYPOGLYCEMIA
2. ACIDOSIS
3. HYPONATREMIA
4. HYPERKALEMIA
PRIMARY=LOW NA,
HIGH K
#1 IS GI FROM
VOMITING/DIARRHEA
SURGERY
ACUTE ADRENAL
CRISIS CAUSES
HEAT
EMOTIONAL DISTRESS
TRAUMA
PREGNANCY
INFECTION
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 needed
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 AI
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 Failure
Goals of Treatment
#1
#2
#3
Physiological
Improve QOL
Prevent
Adrenal
Replacement
Of GC/MC
Crisis
Glucocorticoid
Replacement
Options
1)
HYDROCORTISONE
2)
PREDNISONE
3)
DEXAMETHASONE
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 HRS
Chronic AI Treatment
Primary

Hydrocortisone 20-30 mg/day
Secondary

Hydrocortisone 10-25 mg/day

Most physiologic

Divide doses up to TID

Divide doses up to TID

7.5/5/2.5 (all combinations)

10/5/5=7am/12 pm/5 pm


Fludocortisone 0.05-0.1
mg/day
No need for fludocortisone
because aldosterone not
under ACTH influence
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 pts
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 physiologycircadian 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 variable
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 deprived
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
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?
Stress dose for prolonged exercise
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/bronchitis
PATIENTS REPORT POOR QOL
WORLDWIDE SURVEY OF
1245 PTS:
Despite
treatment….
64% REPORTED
COMPROMISED HEALTH
STATUS
40% MISSED SCHOOL OR
WORK IN LAST 3 MONTHS
38% HOSPITALIZED IN
LAST YR
25% DISABILITY
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
What is in the Pipeline?
Cortisol pumps
Long acting/IR Cortisol
Adrenal Insufficiency after 4 years of
Treatment
Thank you IAFP.