What is this?

This rare endocrine disease is related to insufficiency of the cortical-adrenal glands. It is characterized by deep fatigue with arterial hypotension and bronzed skin tone. Lumbar pains and gastric disorders are commonly associated with bronchial disease.

Causes and risk factors

In Addison's disease, it is usually the chronic destruction of the two cortical-adrenal glands. Two causes dominate

Signs of the disease

Other causes can destroy the adrenal cortex: metastasis of cancer, syphilis, hemochromatosis, amyloidosis, atherosclerosis, histoplasmosis, blastomycosis, traumatic or surgical removal of the adrenal glands ...

Additional examinations and analyzes

Sometimes no cause is found, it is called Addison "idiopathic".

Differential Diagnosis

Processing

In all cases, the deficit being initially adrenal, there is a response of the pituitary that secretes ACTH to try to make the adrenal secrete. This hypersecretion of ACTH causes melanoderma (bronzed skin tone).

The beginning is insidious. The patient is very tired, losing weight and complaining of digestive and genital disorders.

The brownish coloration of the skin (melanoderma) evokes the diagnosis. The pigmentation begins with the folds of flexion discovered and in the level of the scars. Then, it gradually spreads over all the skin and the mucous membranes giving darker spots on a dirty brown background and others lighter (pseudo-vitiligo). Palms and plants are often spared.

The asthenia is major, both physical (fatigability to the effort evoking a myopathy with muscular pains) and psychic (depression). Sexual impotence is common.

The blood pressure is low: the pulse is weak, the patient feels palpitations and dizziness. (Increased hypotension in standing position, known as "orthostatic" hypotension).

Digestive disorders are constant: inappetence, nausea, vomiting, diarrhea, abdominal pain, slimming etc.

A history of tuberculosis is sought by the doctor.

The standard biological check-up may already show abnormalities

Specific examinations are also requested

Dynamic tests are performed to confirm the diagnosis

Other exams look for cause

These are mainly secondary adrenal insufficiency

Without treatment formerly, the disease evolved towards death by acute adrenal insufficiency.

Currently, alternative therapies (cortisone or hydrocortisone 20 to 40 mg / day and mineralocorticoid) allow a life pretty normal but accidents and various illnesses are particularly serious because the hormonal balance is fragile .

This is why the patient must constantly carry a treatment card and know how to increase the doses of medication (2 to 3 times) in case of stress "where he risks a crisis of acute adrenal insufficiency , For example

He must therefore always have at home medicines of advance, of "relief", in particular injectables.

The diet should normally be salted without additional potassium. The salt-free diet is prohibited.

It should be noted that synthetic anti-inflammatory corticosteroid derivatives (Cortancyl, Celestene, Solupred etc ...) absolutely can not replace cortisone.

In case of tuberculosis, the doctor associates an anti-tuberculosis treatment.

Signs of overdose of treatment should be known: high blood pressure, diabetes, Cushing's syndrome.

The cortical shrinkage which is an autoimmune disease with autoantibodies antisurrénale sometimes associated with other autoimmune diseases (thyroiditis, pernicious anemia), Tuberculosis, once the main cause, is increasingly rare in the United States.

Blood Ionogram: decreased sodium and chlorine, increased potassium Anemia, hypereosinophilia Hypoglycaemia Increased urea

collapsed in the morning plasma cortisol, urinary 17 hydroxycorticoïdes reduced (zero in women); 17-keto steroids and urinary free cortisol decreased, plasma renin activity high (reflecting lower aldosterone) and lower urinary aldosterone tétrahydroaldostérone,;. The Dosage of plasma ACTH shows high numbers.

Thorn test negative: the administration of synthetic ACTH (Synacthene) to try to stimulate the adrenal cortex ended in failure since there is more glandular tissue in response to stimulation test to Corticoliberin (CRF) makes it possible to differentiate the different origins of the adrenal insufficiency. This review is being used less and less.

X-ray of the lumbar region: tuberculous adrenal calcification, lumbar-abdominal Scanner, MRI; antisurrénales of antibody research; radiography skull and sella to search an enlarged pituitary gland, chest radiography and research of Mycobacterium tuberculosis (TB ).

Temporary adrenal insufficiency following treatment with corticosteroids by negative retro-control of the gland at rest and triggering adrenal insufficiency upon cessation of treatment; secondary adrenal insufficiencies associated with lesions of the pituitary gland; Causes secondary to conditions indirectly affecting the hypothalamic-pituitary-adrenal axis (prolonged anaemias, chronic diarrhea, myxedema ...) Br>

In case of infectious diseases, especially influenza, After a surgical procedure After a trauma In the case of strong emotions, Of stress ...