Recognising and treating adrenal gland diseases
Expert diagnosis and treatment at the hormone & metabolism centre in Mainz
Location and function
The adrenal glands produce a variety of hormones. Each organ is subdivided into the adrenal cortex and the adrenal medulla. The native, vital hormone cortisol is formed in the adrenal cortex. The original substance is cholesterol – numerous other hormones that also have their own effects are formed during this process. Besides male hormones (androgens), these also include aldosterone, a hormone that regulates the blood pressure. The stress hormones (catecholamines) are also formed in the adrenal medulla. They too regulate the blood pressure and pulse.
Adrenal gland diseases
Addison’s disease is the primary adrenal gland hypofunction. Secondary adrenal gland hypofunction occurs in pituitary gland diseases. Symptoms of adrenal gland hypofunction can include:
- Lack of appetite
- Abdominal pain
- Low blood pressure
- Weight loss
- Brown colouration of the skin
The most frequent cause of the primary hypofunction is an autoimmune disease. In this case, native antibodies are formed to attack the adrenal glands, as a result of which the organ perishes. Rarer causes include tuberculosis, sarcoidosis or metastases of other primary tumours. Treatment in an emergency (‘acute adrenal crisis’) is via a ‘cortisone injection’: any cortisone preparation can be injected in this case (i.m, i.v. or s.c.). Rectal administration of cortisone (emergency suppository) is also very popular. Continuous substitution is carried out with hydrocortisone and/or fludrocortisone (Astonin H®).
This clinical picture is triggered by a usually benign node in the area of the adrenal glands that autonomously produces too much cortisone. It is characterised by the following:
- Truncal obesity
- Diabetes mellitus
- Skin changes with reddened skin
- Chapped skin
- Cracked nails
- Hair loss
- Moon-shaped face
- Buffalo hump
- Reddish, livid stripes on the abdomen
Diagnosis is carried out by means of a dexamethasone suppression test. Localisation of the assumed adenoma is carried out by means of magnetic resonance imaging. The therapy of choice is an experienced endocrine surgeon who heals the patient with an adrenalectomy (removal of an adrenal gland). For differential diagnosis purposes, Cushing’s disease (pituitary disease/autonomous and elevated production of ACTH) must be ruled out.
Increased production of male hormones is caused by an enzyme defect, leading to the masculinisation of females:
- Beard growth
- Hair loss
- Irregular menstrual cycle
- Infertility (childlessness)
This disease is called the adrenogenital syndrome (AGS). The diagnosis is backed-up with molecular biological tests. In the endocrinology practice, the ACTH test is carried out following dexamethasone administration the evening before. Dexamethasone is also used in an extremely low dose in therapy, as is genuine hydrocortisone.
This is an autonomous increased catecholamine production disease. It involves the autonomous formation of too many stress hormones. This leads to hypertension and even life-threatening hypertension crises. Diagnosis is carried out by determining the stress hormones in the blood or urine; if necessary, a suppression test must be performed (clonidine). Localisation is carried out by means of an MIBG scintigram. The therapy of choice is surgery (adrenalectomy) following corresponding prior treatment.
This involves autonomous aldosterone production. The cause is a unilateral or bilateral adenoma; in rare cases, also hyperplasia of both adrenal glands. In patients with arterial hypertension (high blood pressure) and hypokalaemia, a diagnosis is carried out by determining the aldosterone/renin quotient or with a sodium chloride stress test. The therapy of choice is surgical removal of the adenoma (adrenalectomy) or drug therapy with an antagonist (Aldactone®).
Often, a hormonally inactive adenoma of the adrenal glands is found randomly during an MRI examination. This involves a benign adrenal gland adenoma that does not autonomously produce any hormones. In this case, laboratory testing is conducted by the endocrinologist – if this is negative, the adenoma (< 5 cm) can be monitored and checked once a year (MRI and hormone laboratory).
Adrenal gland carcinoma:
This malignant disease of one or both adrenal glands is very rare. On diagnosis, these carcinomas are usually already bigger than 10 cm and the prognosis is poor. Adrenolytic drugs can have a life-prolonging effect.