PENYAKIT ADDISONS

Dr. dr. Shahrul Rahman, Sp.PD, FINAS

Departemen Ilmu Penyakit Dalam
Fakultas Kedokteran
Universitas Muhammadiyah Sumatera Utara

Diseases of the Adrenals

Adrenocortical insufficiency
Addison's
Hypopituitarism
Iatrogenic ACTH deficiency
Cushing's Syndrome
Cushing's Disease (cortical hyperplasia)
Pituitary tumor
Adrenal adenoma or carcinoma
Ectopic ACTH syndrome (from tumors)
Virilization
Adrenal adenoma or carcinoma
Congenital adrenal hyperplasia (CAH)
Adrenal-mediated hypertension syndromes
Primary hyperaldosteronism (adenoma vs. hyperplasia), Cushing's
syndrome, Pheochromocytomas

Pendahuluan
1855, Inggris, Dr. Thomas Addisons Gambaran
klinis & Patologis kel. Adrenal, TBC >
Kel. Adrenal sepasang, piramid, diatas ginjal.
Cortex (luar) kortisol & aldosteron.
Medula ( dalam ) katekolamin.
Fungsi Kortisol :
metabolisme karbohidrat,prot, fat; fgs sistim
imun, sintesa dan efek katekolamin, kontraktilitas
jantung, penyembuhan luka, adanya stress,
integritas endotel, gula darah normal

Aldosteron tek.darah normal & keseimbangan Na+,air

Sekresi hormon korteks adrenal tak memadai karena :
1. Penyakit Primer atau insufisiensi korteks adrenal.
2. Kekurangan hormon ACTH.
Penyakit Addisons / insufisiensi adrenokortikal adalah :
suatu kumpulan gejala yg disebabkan defisiensi berat
atau total dari sekresi kelenjar korteks adrenal.
Insiden
10 / 1 juta penduduk ( negara barat ), 5-6/1 juta ( UK ).
Etiologi Tabel 1.

Tabel 1. Causes of Primary and secondary adrenal Insufficiency

Primary adrenal Insufficiency.

Secondary adrenal Insufficiency.

Slow Onset

Autoimmune Adrenalitis
Pituitary or metastatic tumor
Infection (TBC,fungal,bacterial,etc)
Craniopharyngioma
Adrenomyeloneuropathy
Pituitary surgery or radiation
Thrombosis
Sepsis
Hemorrhage
Lymphoma
Cancer
Long term glucocorticoid th/
Drug
Hypothalamic tumor
Abrupt Onset
Adrenal hemorrhage, necrosis, or
Sheehans Syndrome,
thrombosis or other kinds of sepsis,
Necrosis or bleeding into pituitary
in Coagulation disorders, or in
Anti phospolipid syndrome.

macroadenoma
Head trauma or lesion of pituitary stalk
Pituitary or adrenal surgery

Fisiologi :
Kortisol me pagi hari dan stres, me siang & malam hari
Signal (sitokin,tissue injury,hipotensi, sakit,hipoglikemia )
SSP Hipotalamus CRH Pituitary Ant. ACTH
adrenal kortisol,aldosteron dan androgen.

Patogenesa :
Insufisiensi adrenal primer lesi lokal / proses penyakit
rusak / disfungsi kel. Adrenal.
Insufisiensi adrenal sekunder sekresi kel.Pituitary ACTH .

Gejala & Tanda :

Tabel 2. Manifestasi klinis mayor Penyakit Addisons
Symptoms
Fatigue,weakness
Anorexia
GIT Symptoms
Nausea
Vomitting
Constipation
Abdominal pain
Diarrhea
Salt craving
Postural dizziness
Muscle or joint pain

(%)
100
100
92
86
75
33
31
16
16
12
6-13

Sign
Weight Loss
Hyperpigmentasi
Hypotension
Vitiligo

(%)
100
94
88-94
10-20

Laboratory
Electrolite disturbanc
Hyponatremia
Hyperkalemia
Hypercalcemia
Azotemia (Hipovol )
Anemia
Eosinofilia

(%)
92
88
64
6
55
40
17

Diagnosa :
- Gejala dan tanda klinis, hiperpigmentasi, Lab
(Kortisol,Kortikotropin,aldosteron tabel 3 ),
Auto antibodi, MRI, Biopsi, Radiolabelled
Recombinant human 21 Hydroxylase.
- Uji Stimulasi ACTH
- Uji Hipoglikemia yang distimulasi oleh Insulin.

Table 3. Hormonal Function Test For Adrenal Insufficiency.

Reason for Test
Rule out adrenal
Insufficiency

Hormon Test

Normal Range

Measurement of basal
plasma cortisol between
8 and 9 a.m.

Plasma cortisol
6 24 g / dl

Interpretation Result
If plasma cortisol 3 g / dl
adrenal insuff. Confirmed; if
19 g / dl adrenal insufficiency
rule out.

Conventional corticotropin test

Low dose corticotro
pin
Primary adrenal
Insufficiency
suspected

Conventional cortico
tropin test

Measurement of basal
plasma cortisol and
corticotropin

Basal or post corticotropin plas

ma cortisol 20 g / dl
Basal or post cortico
tropin plasma corti
son 18 g / dl
Basal or post cortico
tropin plasma cortisol
20 g / dl.

Insufficient increase in plasma

cortisol in most cases of
adrenal insufficiency.
Probably insufficient increase
in plasama cortisol in all case
of adrenal insufficiency.
No increase in plasma cortisol
in primary adrenal insuffi Ciency.

Plasma cortisol 6-24 Plasma cortisol low or in the low

g / dl, Plasmai corti- normal range, but plasma cortic
cotropinl 6-45 pg / ml otropin always 100 pg/ml in
primary adrenal insufficiency.

Reason for test

Hormone test

Normal Range

Secondary adrenal
Insufficiency sus Pected.

Insulin induced
hypoglycemia

Plasma Glucose
<40 mg/dl, plas Ma cortisol 20

Little or no increse in plas

ma cortisol in secondary
adrenal insufficiency.

Short metyra pone test

Plasma 11 deoxy
cortisol at 8 hours
7 g / dl,plasma

Insufficient increase in pla

ma corticotropin very sen-

corticotropin >150pg/ml

Corticotropin
releasing hormon
adr-

test

Depends on dose,
time of administra
tion and spesies
human or bovine of
CRH

Low dose cor

ticotropin test

Interpretation Result

sitive and 11 deoxycortisol in

secondary adrenal insuff.

Insufficient increase in
plasma corticotropin and
cortisol in secondary
enal insufficiency.

Basal or corticotro
pin stimulated plas
ma cortisol 18

Probably insufficient stimulation all cases of

secondary adrenal insuffi

g / dl

ciency.

Reason for test

Hormone Test

Secondary adrenal Insulin Induced

Insufficiency due to hypoglicemia
Hyphothalamus
Disease suspected

Corticotropin
releasing hor
mon test on
different day

Normal Range

Interpretation Result

Plasma Gucose Little or no increase in

< 40 mg/dl; plas plasma cortisol in se ma cortisol 20 condary adrenal insuf.
g / dl
due to hypothalamic
Disease.
Transient increa
se in plasma cor
ticotropin and
cortisol

Prolonged, exaggerat
ed plasma corticotro pin response, weak
plasma cortisol
respons in hypothala
mic disease.

Suspected Adrenal Insufficiency

Basal Plasma ACTH , Cortisol
short ACTH Stimulation Test
Normal

No adrenal
insufficiency

Abnormal
ACTH Low
Secondary adrenal
insufficiency

Abnormal
ACTH Low
Primary adrenal
insufficiency

48 hours ACTH Stimulation Test

Cortisol respons
Secondary adrenal
insufficiency
CRH Stimulation Test
Absent or Subnormal ACTH Response
Gambar 1.

Secondary Adrenal insufficiency

No cortisol respons
Primary adrenal
insufficiency

Pengobatan :
- Mengganti hormon yg tidak dihasilkan kel. Adrenal :
Glukokortikoid sintetik (hidrokortison) 25-37,5 mg/12 jam.
Mineralkortikoid (Fludokortison asetat) 0,05-0,2mg.
- Mengobati penyakit dasar.
- Suportif : dehidrasi, hipotensi, gangguan elektrolit,KGD normal

Adrenal Krisis :
- Hidrokortison IV : Bolus 100 mg 100-200 mg / 6 jam.
- Hipovolemia dan hiponatremia Isotonic saline.
- Suplemen glukosa.

Kesimpulan
- Penyakit Addison adalah penyakit yang sangat jarang.
- Penyebab terbanyak autoimun disease.
- Penyakit ini timbul karena kerusakan / disfungsi

kelenjar adrenal.
- Diagnosa berdasdarkan gejala dan tanda klinis, lab.
- Prinsip pengobatan mengganti hormon, mengatasi
- Penyakit dasar dan suportive.