Panhypopituitarism is also known as Simmonds’ disease or Simmond’s syndrome. In the condition, too few or no hormones are produced in the anterior pituitary gland.
What is panhypopituitarism?
Panhypopituitarism is a disorder of the anterior pituitary gland. The anterior pituitary represents the largest part of the pituitary gland. It produces the hormones ACTH (adrenocorticotropic hormone), TSH (thyroid stimulating hormone), MSH (melanocyte stimulating hormone), FSH (follicle stimulating hormone), LH (luteinizing hormone), prolactin and somatotropin. All of these hormones are deficient or absent in panhypopituitarism. Thus, it is a generalized insufficiency of the anterior pituitary gland. Panhypopituitarism can occur acutely or take a chronic course.
Causes
The main cause of panhypopituitarism is a tumor of the pituitary gland. In this case, the disease takes a subacute or chronic course. Autoimmune processes can also affect pituitary function. Rather rarely, panhypopituitarism results from Sheehan syndrome. In Sheehan’s syndrome, there is a loss of function of the anterior pituitary gland due to a lack of blood supply after birth in the mother. Acute insufficiencies of the anterior pituitary are also triggered by trauma. Only rarely is the entire pituitary stalk torn off in an accident. However, impairment of the pituitary gland due to an accident is quite common. Studies from the United States show that nearly half of all patients with moderate traumatic brain injury have impaired pituitary function.
Symptoms, complaints, and signs
Only when 80 percent of the pituitary gland has been destroyed do the first clinical symptoms appear. These are explained by the hormone deficiency. As a result of TSH deficiency, hypothyroidism develops. TSH causes the thyroid gland to produce the thyroid hormones T3 and T4. When T3 and T4 are deficient, affected patients suffer from reduced performance, listlessness, weakness, fatigue, constipation, hair loss and depression. The hormone ACTH shows its effect at the adrenal cortex. Under the stimulation of ACTH, the adrenal cortex produces glucocorticoids, mineralocorticoids and steroids. In particular, when ACTH is deficient, too little cortisol is produced. The main symptom of this secondary adrenal insufficiency is weakness. Affected individuals also lose weight, vomit frequently, and have low blood pressure. Hyperpigmentation of the skin is also typical. However, if there is a simultaneous deficiency of MSH, the hyperpigmentation may be absent. Patients who produce too little melanocyte stimulating hormone are very pale. The deficiency of follicle stimulating hormone and luteinizing hormone results in gonadal hypofunction. In women, this is noticeable by the absence of menstrual bleeding. Affected women are temporarily infertile. After pregnancy, panhypopituitarism manifests itself with a deficiency of prolactin through agalactia. The medical term agalactia refers to the lack of milk production during the breastfeeding period. Agalactia usually occurs in association with Sheehan syndrome. In panhypopituitarism, no or too little growth hormones are also produced. This leads to short stature in childhood. In adulthood, the deficiency manifests as truncal obesity. In truncal obesity, there is a tendency for increased fat storage on the trunk. Coronary heart disease (CHD) can also be a consequence of the lack of growth hormones. To help remember the symptoms of panhypopituitarism, use the 7 A’s: No eyebrows, no axillary hair, agalactia and amenorrhea, apathy and adynamia, and alabaster pallor.
However, typical symptoms also include lowered blood pressure and lowered urinary osmolality.
Diagnosis and course of the disease
The clinical picture already provides the first clues to the disease. Previous events, such as trauma or childbirth, may substantiate the suspicion. After a detailed history, basal hormone diagnostics or endocrinologic function diagnostics are usually performed in the patient. The individual pituitary subfunctions are tested. Laboratory chemical diagnostics show decreased pituitary hormones such as ACTH or FSH. The target hormones of these regulatory hormones are also decreased.Thus, too little thyroid or adrenal cortical hormones are also found in the blood. In order to determine whether the disorder originates from the anterior pituitary lobe itself or from the superior hypothalamus, a stimulation test can be performed. This involves stimulating the hormone secretion of the pituitary gland at various levels. The most important test is the insulin hypoglycemia test. In this test, the patient is injected with insulin. This results in a significant lowering of blood glucose. Normally, due to a massive stress reaction, increased ACTH, cortisol and somatotropin would be released. If the increase fails to occur, there is pituitary or hypothalamic damage. To rule out the hypothalamus as the cause, other releasing hormone tests can be performed. These tests include the CRH test, the GHRH test, and the TRH test. Since the cause of pituitary insufficiency is usually a tumor, diagnostic imaging must always be performed if panhypopituitarism is suspected. Therefore, an MRI is usually performed. Because pituitary tumors also endanger the optic nerves due to their spatial location, an ophthalmologic diagnosis should also be performed.
Complications
In most cases, panhypopituitarism results in a hormone deficit, which can lead to various complications and complaints. However, the further course depends strongly on this deficit, so that usually no general prediction can be made about it. Furthermore, those affected by panhypopituitarism suffer from a malfunction of the thyroid gland. Due to this, the affected person suffers from fatigue and general exhaustion. Patients also often suffer from hair loss, depression and constipation. The quality of life of the affected person is considerably reduced due to the disease. If this complaint is not treated, it can also result in adrenal insufficiency, which can have a negative effect on the life expectancy of the affected person. Weight loss or low blood pressure may also develop due to panhypopituitarism and continue to restrict the daily life of the affected person. If the disease occurs in childhood, it results in short stature or various heart diseases. As a rule, the treatment of the disease always depends on the cause. In the case of a tumor, it must be removed. In this case, no general course can be predicted, since the tumor may have spread to other regions of the body.
When should you go to the doctor?
Changes in hormone balance indicate a health disorder. If they are constant or increase in intensity, a doctor is needed. If there are complaints such as hair loss, changes in the appearance of the skin, as well as disorders of the digestive tract, a visit to the doctor is necessary. If sexually mature women experience irregularities or a lack of menstruation, a clarification of the complaints is recommended. In case of an unfulfilled desire to have children despite all efforts, a check-up is recommended to clarify the fertility status. Decreased libido or sexual dysfunction are signs of an irregularity in the organism that should be treated. Behavioral abnormalities, mood swings, and personality changes are warning signs that should be followed up. If withdrawal behavior or reduced participation in social life is evident, a follow-up visit to a physician or therapist is advisable. Changes in weight, repeated vomiting and nausea are also alarming signals. Disturbances in the growth process in children and adolescents, short stature, a pale appearance, and irregularities in heart rhythm should be presented to a physician. Sluggishness, apathy, easy fatigability and increased need for sleep are considered signs of health impairment and should be examined by a physician. Fat deposits, swelling, or a general feeling of malaise and illness should be evaluated by a physician. Consultation with a physician is recommended for people with decreased wellness.
Treatment and therapy
Treatment of panhypopituitarism depends on the cause. If the condition is based on a tumor, the therapeutic approach depends on the tumor size, tumor type, and the threat of damage from the mass. As a rule, a combination of surgical and medicinal measures is used.Radiation therapy is also used. Often, the impaired pituitary function can be restored in this way. If restoration is not possible, the missing hormones must be substituted.
Outlook and prognosis
Sudden hypopituitarism of the pituitary gland can lead to various dysfunctions and degrees of severity. Depending on the cause and effect, it is a severe to very severe acute condition. The outlook for people suffering from hypopituitarism (panhypopituitarism) can therefore only be assessed according to the causes of the hypopituitarism. A comprehensive diagnosis is mandatory. Some of the causes of hypopituitarism of the pituitary gland can be treated quite well today. In this case, the prognosis is much more positive. The administration of missing hormones can often improve the quality of life of those affected. Their chances of survival increase to a normal life expectancy. If the missing hormones cannot be administered, the survival expectancy for this disorder can still be ten to fifteen years. The situation is different for patients who fall into a pituitary coma. The reason for this may be severe stress. Pituitary coma can be caused by untreated hypothyroidism or pituitary hypofunction, or stroke. It is a life-threatening situation. It requires immediate emergency measures. The prognosis depends on how quickly these measures are taken. In addition, the severity of the sequelae that have developed, or the age of the person affected, are also criteria. Without immediate administration of glucocorticoids and thyroid hormones, many coma patients die.
Prevention
Panhypopituitarism cannot be prevented.
Aftercare
In most cases, only limited measures of direct aftercare are available to the affected person with panhypopituitarism. For this reason, early diagnosis is very important in this disease to avoid other complications or symptoms. Therefore, affected individuals should ideally seek medical attention at the first signs and symptoms of panhypopituitarism. As a rule, self-healing cannot occur. Sufferers are dependent on taking various medications that can alleviate these symptoms. Here, the affected person should pay attention to the prescribed dosage so that the complaints can be alleviated. Regular checks and examinations by a doctor are also very important to avoid other complications or complaints that can reduce the quality of life of the affected person. It is not uncommon for those affected by panhypopituitarism to also depend on the help and support of friends and family, which can help prevent the development of depression and other psychological complaints. The further course of panhypopituitarism depends thereby strongly on the time of the diagnosis, so that thereby usually no general prediction is possible.
What you can do yourself
Panhypopituitarism often triggers a hormone deficit, that is why the symptoms should be closely monitored. To avoid or alleviate complications, affected individuals should contact a doctor early on. Signs such as fatigue and exhaustion may be an indication of an exacerbation. Further symptoms such as depression, hair loss and constipation reduce the quality of life. For this reason, it is essential to seek medical treatment for the condition. By regularly taking the prescribed medication, the thyroid dysfunction associated with the disease can be controlled. If particular behavioral abnormalities or mood swings occur, patients should follow up on these warning signs and be honest with their doctor. Social behavior plays a role in this context that should not be underestimated. If necessary, treatment with a psychotherapist is advisable. When psychological moods are balanced, patients find it much easier to cope better with the physical complaints and the specific symptoms. Even for children and adolescents, such therapy can therefore be very important. In the case of repeated nausea, vomiting and strong changes in body weight, patients should not hesitate for long, but make a doctor’s appointment soon.By acting quickly on such alarm signals, sufferers get their symptoms under control quite well.