What are the potential life-threatening complications of acromegaly. How does acromegaly develop and what causes this rare hormonal disorder. What are the common symptoms and risk factors associated with acromegaly.

Understanding Acromegaly: A Rare Hormonal Disorder

Acromegaly is an uncommon endocrine disorder characterized by excessive production of growth hormone (GH) in the body. This condition primarily affects middle-aged adults, although symptoms can manifest at any age. The disorder occurs when the pituitary gland, located at the base of the brain, produces an abnormally high amount of growth hormone over an extended period.

The prevalence of acromegaly is relatively low, with an estimated 3 to 14 cases per 100,000 people. Despite its rarity, the impact on affected individuals can be significant, potentially leading to various health complications if left untreated.

How does acromegaly differ from gigantism?

While acromegaly and gigantism are both caused by excess growth hormone, they differ in their onset and manifestation. Gigantism occurs when excessive GH production begins before the end of puberty, resulting in abnormal height increase. In contrast, acromegaly develops in adults after the growth plates have fused, leading to enlargement of bones and soft tissues rather than increased height.

Causes and Mechanisms of Acromegaly

The primary cause of acromegaly in over 90% of cases is a benign tumor in the pituitary gland, known as a pituitary adenoma. These tumors secrete excessive amounts of growth hormone, triggering a cascade of hormonal events in the body.

What role does IGF-I play in acromegaly?

When elevated levels of growth hormone enter the bloodstream, they signal the liver to produce insulin-like growth factor I (IGF-I). This hormone is responsible for the actual growth of bones and body tissues. Additionally, high levels of IGF-I can alter the body’s metabolism of glucose and lipids, potentially leading to complications such as type 2 diabetes, hypertension, and heart disease.

While the exact cause of pituitary tumors remains unclear, genetic factors may play a role in their development. Some cases of acromegaly in young adults have been linked to specific gene defects, suggesting a hereditary component in certain instances.

Recognizing the Symptoms of Acromegaly

The symptoms of acromegaly often develop gradually over several years, which can delay diagnosis and treatment. Common physical changes associated with the disorder include:

• Enlargement of hands and feet, often noticed through changes in ring or shoe size
• Facial features becoming more pronounced, with enlargement of the lips, nose, and tongue
• Changes in bone structure, including protruding brow and lower jaw
• Thickening and coarsening of the skin, often accompanied by increased sweating and body odor
• Deepening of the voice
• Growth of skin tags

In addition to these physical changes, individuals with acromegaly may experience:

• Persistent headaches
• Joint pain and arthritis
• Vision problems
• Sleep apnea
• Carpal tunnel syndrome

Life-Threatening Complications of Acromegaly

While acromegaly is treatable in most cases, it can lead to serious and potentially life-threatening complications if left unmanaged. The most severe complications that can pose a risk to life include:

How does acromegaly affect the cardiovascular system?

Acromegaly can significantly impact heart health, leading to conditions such as:

• Hypertension (high blood pressure)
• Cardiomyopathy (enlargement and weakening of the heart muscle)
• Arrhythmias (irregular heartbeats)
• Heart valve disorders

These cardiovascular complications can increase the risk of heart failure and sudden cardiac death, making them among the most serious threats to individuals with untreated acromegaly.

What respiratory complications can arise from acromegaly?

Sleep apnea is a common and potentially dangerous complication of acromegaly. This condition causes repeated pauses in breathing during sleep, which can lead to:

• Chronic fatigue
• Increased risk of accidents
• Cardiovascular strain
• Cognitive impairment

In severe cases, sleep apnea can result in sudden death during sleep, particularly if left untreated.

How does acromegaly increase cancer risk?

Individuals with acromegaly have an elevated risk of developing certain types of cancer, particularly colon cancer. The increased production of IGF-I is believed to promote the growth of both benign and malignant tumors. Regular cancer screenings are crucial for early detection and treatment in acromegaly patients.

Diagnostic Approaches for Acromegaly

Diagnosing acromegaly can be challenging due to its gradual onset and the similarity of some symptoms to other conditions. However, early detection is crucial for preventing complications and improving outcomes. The diagnostic process typically involves:

What blood tests are used to diagnose acromegaly?

The primary blood tests used in diagnosing acromegaly include:

• IGF-I level measurement: Elevated levels of IGF-I are a reliable indicator of acromegaly
• Growth hormone suppression test: This test assesses the body’s ability to suppress GH production after glucose intake

What imaging studies are employed in acromegaly diagnosis?

If blood tests suggest acromegaly, imaging studies are typically performed to locate and characterize the pituitary tumor. These may include:

• Magnetic Resonance Imaging (MRI) of the pituitary gland
• Computed Tomography (CT) scan, if MRI is contraindicated

These imaging techniques help determine the size and location of the tumor, which is crucial for planning treatment.

Treatment Strategies for Acromegaly

The primary goals of acromegaly treatment are to reduce GH production, alleviate symptoms, and prevent or manage complications. Treatment approaches may include:

What surgical options are available for acromegaly?

Surgery is often the first-line treatment for acromegaly caused by pituitary adenomas. Transsphenoidal surgery, where the tumor is removed through the nose and sinuses, is the most common approach. This minimally invasive procedure aims to remove the tumor while preserving normal pituitary function.

How effective are medications in managing acromegaly?

Several types of medications can be used to control acromegaly, either as primary treatment or in combination with surgery:

• Somatostatin analogs: These drugs inhibit GH production and can shrink tumor size
• GH receptor antagonists: These medications block the effects of GH in the body
• Dopamine agonists: These can be effective in some cases, particularly when the tumor also produces prolactin

What role does radiation therapy play in acromegaly treatment?

Radiation therapy may be recommended if surgery and medication are ineffective or not feasible. It can help reduce tumor size and GH production over time, although its effects are generally slower than other treatment modalities.

Living with Acromegaly: Long-Term Management and Prognosis

While acromegaly can have significant impacts on health and quality of life, proper management can greatly improve outcomes. Long-term care for individuals with acromegaly typically involves:

• Regular monitoring of GH and IGF-I levels
• Ongoing assessment and management of complications
• Lifestyle modifications to support overall health
• Psychological support to address the emotional aspects of living with a chronic condition

Can the effects of acromegaly be reversed?

With successful treatment, many symptoms of acromegaly can improve or even resolve. However, some changes, particularly those related to bone growth, may be permanent. Early diagnosis and treatment are key to minimizing long-term effects and improving quality of life.

What is the life expectancy for individuals with treated acromegaly?

When acromegaly is diagnosed early and managed effectively, life expectancy can return to near-normal levels. However, this underscores the critical importance of prompt diagnosis, appropriate treatment, and ongoing management of the condition and its potential complications.

Emerging Research and Future Directions in Acromegaly Treatment

Ongoing research in the field of acromegaly aims to improve diagnosis, treatment, and quality of life for affected individuals. Some promising areas of investigation include:

What new treatment modalities are being explored for acromegaly?

Researchers are exploring several innovative approaches to acromegaly treatment, including:

• Gene therapy targeting the underlying causes of pituitary tumors
• Novel drug delivery systems for more effective and convenient medication administration
• Immunotherapy approaches to target tumor cells more specifically

How might personalized medicine impact acromegaly management?

Advances in genetic testing and molecular biology are paving the way for more personalized treatment strategies. By identifying specific genetic markers or tumor characteristics, clinicians may be able to tailor treatments more effectively to individual patients, potentially improving outcomes and reducing side effects.

As research progresses, the hope is that new insights will lead to earlier diagnosis, more targeted treatments, and improved quality of life for individuals living with acromegaly. Ongoing clinical trials continue to explore new therapeutic options and refine existing treatment protocols, offering hope for enhanced management of this rare but impactful disorder.

Acromegaly – NIDDK

On this page:

• What is acromegaly?
• How common is acromegaly?
• Who is more likely to develop acromegaly?
• What are the complications of acromegaly?
• What are the symptoms of acromegaly?
• What causes acromegaly?
• How do doctors diagnose acromegaly?
• How do doctors treat acromegaly?
• Clinical Trials for Acromegaly

What is acromegaly?

Acromegaly is a disorder that occurs when your body makes too much growth hormone (GH). Produced mainly in the pituitary gland, GH controls the physical growth of the body. In adults, too much of this hormone causes bones, cartilage, body organs, and other tissues to increase in size. Common changes in appearance include enlarged or swollen nose, ears, hands, and feet.

The pituitary gland sits at the base of the brain.

How common is acromegaly?

Acromegaly is rare. Scientists estimate that about 3 to 14 of every 100,000 people have been diagnosed as having acromegaly. ¹

Who is more likely to develop acromegaly?

Acromegaly is most often diagnosed in middle-aged adults, but symptoms can appear at any age. In children, too much growth hormone causes a condition called gigantism rather than acromegaly. Gigantism occurs when excess GH begins before the end of puberty, when children’s growth plates fuse or close. Having too much GH before the growth plates close causes children to grow tall in height.

What are the complications of acromegaly?

Acromegaly is treatable in most people. But because symptoms come on slowly, health problems can develop before the disorder is diagnosed and treated.

Health problems can include

• type 2 diabetes
• high blood pressure
• heart disease
• sleep apnea
• arthritis
• carpal tunnel syndrome
• other conditions affecting the bones and muscles

People with acromegaly also have an increased risk for colon polyps, which may develop into colon cancer if not removed.

Some people with acromegaly may have a genetic condition that can lead tumors to develop in different parts of their bodies. Increased GH can cause these other tumors to grow.

Untreated, acromegaly can lead to serious health problems and early death. But when successfully treated, symptoms generally improve and may go away altogether. Life expectancy may return to normal.²

What are the symptoms of acromegaly?

Symptoms of acromegaly can vary from person to person. Common changes in physical appearance include

• hands and feet become larger and swollen—you may notice a change in ring or shoe size, especially shoe width
• lips, nose, and tongue become larger
• bone changes: brow and lower jaw jut out, bridge of the nose gets bigger, and space between teeth increases
• skin becomes thick, coarse, and oily
• sweating and skin odor increase
• voice becomes deeper
• skin tags—small, usually flesh-colored growths of skin that have a raised surface—may get larger or darker

Other common symptoms include

• headaches
• joint aches
• vision problems

What causes acromegaly?

Acromegaly develops when the pituitary gland releases too much GH into the body over a long period of time. When GH enters the blood, this signals the liver to produce another hormone, called insulin-like growth factor I (IGF-I). IGF-I is the hormone that actually causes bones and body tissue to grow. High levels of this hormone also cause changes in how the body processes blood glucose (blood sugar) and lipids (fats), which can lead to type 2 diabetes, high blood pressure, and heart disease.

In more than 9 out of 10 cases, acromegaly is caused by a tumor in the pituitary gland, called a pituitary adenoma.³ More rarely, the cause may be a tumor in another part of the body.

Although scientists don’t know what causes these tumors to develop, genetic factors may play a role. In young adults, acromegaly has been linked to defects in certain genes.

Pituitary tumors

Pituitary tumors are almost always benign, or noncancerous. Some tumors grow slowly, and symptoms of too much GH may not be noticed for many years. Other tumors may grow rapidly.

Depending on its size and location, the tumor may press against other pituitary tissue. Possible effects include

• changes in menstruation in women
• erectile dysfunction in men
• changes in thyroid hormone, which can affect weight, energy levels, hair, and skin
• decreases in cortisol, which can cause weight loss, dizziness, tiredness, low blood pressure, and nausea

A tumor that grows large in size may also press against nearby parts of the brain. This can lead to other symptoms, such as headaches and vision problems.

Some pituitary tumors that create growth hormone can also increase the levels of other hormones in the body. For example, the tumor may produce prolactin, the hormone that prompts the mammary glands to produce milk. This can lead to breast milk discharge in women.

Nonpituitary tumors

Rarely, acromegaly is caused by tumors located in the hypothalamus—a small area of the brain near the pituitary gland, pancreas, lungs, or other parts of the chest or abdomen. Some of these tumors make growth hormone themselves. But more often, the tumors produce growth hormone-releasing hormone (GHRH), a hormone that signals the pituitary gland to make growth hormone.

How do doctors diagnose acromegaly?

Blood tests

Doctors most often diagnose acromegaly by ordering two blood tests that help determine if your body is making too much GH.

• IGF test. Levels of GH in the blood can change throughout the day. A reliable way to track GH in the body is by measuring the level of IGF-I in the blood. In most cases, a high IGF-I level suggests that you have acromegaly.
• Oral glucose tolerance test. To confirm the diagnosis, your doctor will order an oral glucose tolerance test. For this test, you will drink a sugary liquid. A health professional will then test your blood every half hour for 2 hours to measure growth hormone levels. The sugar in the drink will normally cause GH levels to fall. But if your body is making too much of the hormone, these levels will not go down enough—thereby confirming the diagnosis of acromegaly.

Imaging tests

If the blood tests confirm that your body is making too much GH, your doctor will conduct imaging tests to locate and measure the tumor that may be causing the problem. Two commonly used tests are

• Magnetic resonance imaging. The preferred test for viewing a pituitary tumor is the magnetic resonance imaging (MRI) scan. The MRI scan uses radio waves and magnets to create detailed images of your internal organs and soft tissues without x-rays.
• Computed tomography scan. If an MRI is not a good option for you (for example, if you have a pacemaker or other implant that has metal), your doctor may order a computed tomography (CT) scan instead. The CT scan uses a combination of x-rays and computer technology to create images of your organs and other internal parts of your body.

An MRI can show the location and size of a pituitary tumor.

If the imaging test doesn’t find a pituitary tumor, your doctor will look for nonpituitary tumors as the cause of your high GH levels.

How do doctors treat acromegaly?

Treatment options include surgery, medicines, and radiation therapy. The goals of treatment are to control tumor size, return GH and IGF-I levels back to normal, improve symptoms, and manage related health problems. No single treatment is right for everyone. Your doctor will recommend a treatment plan that works for you, depending on factors such as your age, tumor size, severity of symptoms, GH and IGF-I levels, and health status.

Surgery

Doctors can remove most pituitary tumors using a method called transsphenoidal surgery. The operation is done through the nose and sphenoid sinus, a hollow space in the skull behind the nasal passages and below the brain. Two approaches to this surgery are

• with a microscope—a magnifying tool
• with an endoscope—a thin, lighted tube with a tiny camera

In both approaches, the surgeon uses advanced MRI imaging to scan the area around the tumor before surgery. He or she then makes a small cut inside your nostril to view the area and remove the tumor using tiny, special tools. In microscopic surgery, the surgeon uses a microscope to magnify the area. In endoscopic surgery, an endoscope camera sends images to a television monitor instead. Risks and results are similar for both approaches.³

When the tumor that is creating too much GH is not located in the pituitary gland, other types of surgery are used to remove the tumor. Removing these nonpituitary tumors also lowers GH levels and improves acromegaly symptoms.

Risks. Complications from surgery can include bleeding, cerebrospinal fluid leaks, meningitis, sodium (salt) and water imbalance, and low levels of pituitary hormones.³

Outcomes. The surgery is considered a success if blood levels of GH and IGF-I return to normal after 12 weeks. The cure rate right after surgery is about 85 percent for small tumors and 40 to 50 percent for large tumors. ³

When successful, the surgery relieves pressure on nearby areas of the brain and causes GH levels to drop right away. Soft tissue swelling may get better within a few days but facial changes may take longer to improve.

Surgery is most successful in people with smaller pituitary tumors. Success largely depends on the skill and experience of the surgeon, as well as the location of the tumor. Even experienced surgeons may not be able to remove the tumor if it’s too close to parts of the brain where surgery would be risky. However, surgeons may be able to remove part of the tumor.

Postsurgery treatments. In most cases, levels of GH and IGF-I improve but don’t go back to normal. If levels of these hormones are still too high or begin to rise again, you may need further treatment. Most often, this will involve taking medicines. In some cases, your doctor may recommend a second surgery.

Medicines

Currently, three types of medicines are used to treat acromegaly, but they are not a cure. The medicines may be used alone or in combination with each other.

Somatostatin analogs. The medicines most often used to treat acromegaly are called somatostatin analogs (SSAs). These drugs curb the release of GH and may also reduce the size of the pituitary tumor. Several studies have shown that these drugs are safe and effective for long-term treatment. The medicines are delivered by injection, but scientists are currently studying other options, such as pills.⁴ The most common side effects of SSAs are cramps, gas, and diarrhea. These effects are usually mild and go away over time. Some people may develop gallstones that usually do not cause symptoms. Hair loss is possible and, in rare cases, permanent. Control of blood sugar usually improves but, rarely, may worsen.

Dopamine agonists. These medicines inhibit GH production and tumor growth, but not as well as SSAs do. Dopamine agonists are most likely to work in people who have mild GH excess and those who have both acromegaly and hyperprolactinemia (too much of the hormone prolactin). The medicines are taken by mouth. Side effects can include nausea, stuffed nose, tiredness, headache, dizziness when standing, nightmares, and mood changes.

Growth hormone-receptor antagonists. Unlike the other two medicines, GH-receptor antagonists do not stop the body from making too much GH. Instead, they block GH from signaling the body to make more IGF-I. The drug is taken in the form of a daily injection under the skin that patients can administer themselves. Side effects can include liver problems.

Radiation therapy

The third treatment option is radiation therapy, which uses high-energy x-rays or particle waves to kill tumor cells. This type of treatment may be recommended if surgery isn’t possible or fails to remove all tumor tissue, and medicines are not an option or working for you.

Stereotactic. The preferred type of radiation therapy is stereotactic radiation therapy, which uses 3-D imaging to precisely aim high doses of radiation to the tumor from various angles. ³ The treatment can sometimes be done in a single session, reducing the risk of damage to nearby tissue. However, a single dose may not work for very large tumors and tumors located close to nerves that affect vision.

Conventional. The second option is conventional radiation therapy, which also targets the tumor with external beams. This type of radiation therapy delivers small doses of radiation in a series of treatments over 4 to 6 weeks.

Radiation therapy uses high-energy rays to destroy tumor cells in the pituitary gland.

As radiation treatment lowers GH and IGF-I levels over time, it may take years for this treatment to noticeably improve acromegaly symptoms. Your doctor is likely to prescribe medicines while you wait for GH and IGF-I levels to go back to normal and for symptoms to improve.

All forms of radiation therapy cause other pituitary hormones to slowly decrease over time. About half of people treated with radiation therapy will need hormone replacement after treatment ends. Radiation can also impair a patient’s fertility.

Vision loss and brain injury are rare complications. Rarely, other types of tumors can develop many years later in areas that were in the path of the radiation beam.

Clinical Trials for Acromegaly

The NIDDK conducts and supports clinical trials in many diseases and conditions, including endocrine diseases. The trials look to find new ways to prevent, detect, or treat disease and improve quality of life.

What are clinical trials for acromegaly?

Clinical trials—and other types of clinical studies—are part of medical research and involve people like you. When you volunteer to take part in a clinical study, you help doctors and researchers learn more about disease and improve health care for people in the future.

Researchers are studying many aspects of acromegaly and gigantism, such as

• use of medicine to treat gigantism in children and adolescents
• genetic factors that may cause pituitary tumors to develop, and how to treat the tumors and related complications in children and adults

Find out if clinical studies are right for you.

What clinical studies for acromegaly are looking for participants?

You can view a filtered list of clinical studies on acromegaly that are open and recruiting at
www.ClinicalTrials.gov. You can expand or narrow the list to include clinical studies from industry, universities, and individuals; however, the NIH does not review these studies and cannot ensure they are safe. Always talk with your health care provider before you participate in a clinical study.

References

Causes, Symptoms, Treatment and Faq’s

Acromegaly is a condition that happens when the pituitary gland produces excess growth hormone that affect many parts of your body. The onset of this condition is slow and can be challenging to diagnose early. Though acromegaly can cause life-threatening complications, it can be managed.

What is Acromegaly?

Your pituitary gland sits at the base of your brain and is responsible for releasing the growth hormone. When the gland releases too much of this hormone in adults, it can cause the body to have enlarged bones, cartilage, organs, and other tissues.

In about 90% of all cases of acromegaly, the excessive release of growth hormone is caused by the presence of a benign tumor on the pituitary gland. This type of tumor is known as a pituitary adenoma.

What are the symptoms of Acromegaly?

Most of the symptoms of acromegaly are visually apparent. However, these symptoms can vary from person to person. Some of the more common visual acromegaly symptoms include:

● Thick, coarse, and oily skin

● Deeper voice

● A more prominent nose, lips, and tongue

● Hands and feet become bigger.

● Increased body odor and sweating

● More extensive and darker skin tags

● Brow bone or jaw may jut out.

● Increased spacing between the teeth

● muscle weakness and fatigue

● Severe snoring issues due to obstruction of upper airway

● Headaches

● Impaired vision

● Pain & limited joint mobility

● Menstrual cycle irregularities in women

● Erectile dysfunction in men

● Enlarged organs like the heart

● Loss of interest in sex

Non-visual acromegaly symptoms include:

● Joint aches

● Vision problems

● Headaches

What are the complications of Acromegaly?

In most cases, acromegaly can be treated. However, since the condition develops slowly, you could experience further complications before your condition is diagnosed. These complications can be life-threatening. Some of these complications include:

● High blood pressure

● Type 2 diabetes

● Arthritis

● Carpal tunnel syndrome

● Heart disease

● Sleep apnoea

● Compression of the spinal cord

● Goiter

● Pre-cancerous growths on the colon

As you can see, some of the complications of acromegaly are dangerous to your health. This is why it is essential to get a diagnosis at the earliest and manage the condition before these complications can manifest.

Diagnosis of Acromegaly

Your doctor may ask about your medical history and conduct a physical examination. The following investigations are also performed :

● GH and IGF-I measurement: Elevated levels of these hormones suggest acromegaly.

● Growth hormone suppression test: GH blood levels are measured before and after you drink a preparation of sugar (glucose). Generally, glucose ingestion depresses the levels of GH. If you have acromegaly, the GH level in your body will tend to stay high.

● Imaging: Your doctor will suggest that you undergo an imaging procedure like MRI (magnetic resonance imaging), to help locate the exact location and size of a tumour of your pituitary gland. If no pituitary tumors are seen, your doctor may look for nonpituitary tumors as the cause of your high GH levels.

What are the treatments for Acromegaly?

One of the first lines of treatment will be to address the underlying reason for the increased growth hormone. Other treatments will center around reducing the adverse effects of the condition. You might need a combination of these different types of treatments.

Surgery

Your doctor might recommend a treatment known as transsphenoidal surgery. It involves removing the tumorous growth on your pituitary gland through the nose. In some cases, the surgeon might even be able to remove the entire tumor. However, growth hormone levels can still be elevated after surgery. In this case, medications and radiation treatments will be required.

Medications

The most important step to treat acromegaly is to control the growth hormone. Medicines can be prescribed to do precisely this. There are three types of medications that can limit the effects of growth hormone.

● Medicines to reduce the production of growth hormone: These drugs will be injected into the gluteal muscle once a month to limit the excessive secretion of growth hormone by the pituitary gland.

● Medicines to reduce the production of hormones: These are oral medications that are effective in some people.

● Treatments to stop the effects of growth hormone: This drug has to be used daily to block the effects of growth hormone. These are called Growth Hormone Antagonists .

Radiation

You might require radiation therapy after surgery. This is done to destroy any remaining tumor cells and to reduce the growth hormone. There are three types of radiation therapy.

● Conventional radiation therapy: This treatment cycle lasts 4-6 weeks, where you will be given regular doses of radiation. However, the effects of this treatment might be felt only years later.

● Stereotactic radiosurgery: This treatment subjects only the tumor cells to a single dose of radiation. You will feel the full effects of this therapy within five years.

● Proton beam therapy: A targeted treatment, proton beam therapy is given over several doses. The effects of this therapy appear sooner than the other two treatments.

How can Acromegaly be prevented?

Unfortunately, scientists and medical professionals have not been able to determine the cause of tumors on the pituitary gland. This means that acromegaly cannot be prevented. However, an early diagnosis can help you avoid the onset of further complications.

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Frequently Asked Articles (FAQs)

1. What is the difference between gigantism and acromegaly?

Acromegaly and gigantism are closely related. The former affects middle-aged adults, whereas gigantism is seen when excess growth hormones are secreted in children.

2. What is the prognosis of acromegaly?

The prognosis of acromegaly depends on when the condition is treated. If the right treatment is given, a person with acromegaly can expect average life expectancy. However, if left untreated, especially with the related complications, life expectancy can reduce by ten years.

3. What organs are affected by acromegaly?

The most noticeable organ effects are in the heart, kidneys, and vocal cords, though other organs can be affected too.

4. Is acromegaly reversible?

Some features of the condition cannot be reversed, particularly those affecting the bones. If you have developed other complications like high blood pressure and diabetes, then you will have to regulate those conditions for your entire life.

5. What is the life expectancy of an individual with acromegaly?

The life expectancy of someone with acromegaly after treatment is the same as the general population. However, if you do not get the right treatment, it can be a life-threatening condition. This is why it is essential to consult with a doctor at the earliest sign of acromegaly.

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Acromegaly, symptoms and treatment, symptoms, diagnosis and treatment | Alpha

The reasons why this happens are also known: the disease provokes a pituitary adenoma that produces excessive secretion of growth hormone, as well as non-pituitary tumors that produce a factor that releases this hormone.

In the Nizhny Novgorod clinic “Alfa Health Center” the disease can be diagnosed during the examination by an endocrinologist. Sometimes, in the presence of characteristic symptoms, it is detected during the passage of the comprehensive program “Life without a headache”: pain in acromegaly is not uncommon.

Reasons

This severe neuroendocrine disease is caused by overproduction of somatotropin, or growth hormone. Often, the root cause of the pathological process is a benign neoplasm that produces somatotropic hormone in isolation. Provoke the appearance of a tumor can regular traumatic brain injury, chronic sinusitis and tumor of the hypothalamus. Cases of genetic inheritance of the disease are known. Often, with acromegaly, the tumor increases the production of not only somatotropic hormone, but also prolactin.

The vast majority of known cases are associated with pituitary adenoma or somatotropin secretion. But data have also been recorded on the development of acromegaly under the influence of ectopic secretion of a hormone that releases growth hormone, and that is already responsible for pituitary hyperplasia.

Symptoms

The prevalence of acromegaly in European countries is estimated at approximately one case in 100-250 thousand. According to medical records, most often the diagnosis is made in patients of the middle age group. Among women and men over 40 years of age, the prevalence of acromegaly is approximately the same. Due to the fact that it develops gradually and progresses slowly, the disease is most often detected in the 4-10th year after the onset of development.

The active production of growth hormone leads to increased production of somatomedins: the so-called growth factors, among which the most important role is played by somatomedin C, or insulin growth factor. It is produced in the liver. In addition, acromegaly is accompanied by the production of growth factors of various organs – cartilage, bone and others. Sometimes, if the disease began in adolescence, elongation of the bones of the skeleton is possible. This clinical syndrome in acromegaly is called gigantism.

For patients with acromegaly, a gross change in appearance is characteristic. They are getting bigger:

• language;
• lips;
• brow ridges;
• nose.

Pathological folding and thickening of the skin is observed. Diastema develops – the gap between the teeth increases. Often prognathism is one of the types of profiling of the face, suggesting the protrusion of the lower jaw forward. Hands and feet increase in size.

The voice with acromegaly becomes coarse. This is due to thickening of the vocal cords. Another reason for the change in voice is the expansion of the paranasal cavities.

Patients with acromegaly may complain of pain in the spine and joints. They can be caused by the development of arthropathy and their destabilization. In the area of ​​\u200b\u200bnatural folds, hyperpigmentation of the skin, villous-warty growths are observed, and in women, increased male-type body hair growth is possible – a syndrome also called hirsutism.

Acromegaly is often accompanied by an increase in the thyroid gland and the appearance of nodular formations. The patient may develop other diseases of the endocrine system – for example, insulin-resistant diabetes mellitus. Cardiovascular pathologies caused by acromegaly are of great danger – high blood pressure, cardiomegaly, microcardiodystrophy. The latter is characterized by the fact that it can cause coronary insufficiency and, as a result, death.

The defeat of the respiratory system in acromegaly is manifested by obstructive sleep apnea. The syndrome is associated with a pathological increase in the size of the epiglottis and tongue. Also, in a patient with acromegaly, the frequency of restrictive lung diseases that are associated with changes in the spine (kyphoscoliosis) increases. There may be a decrease in the sensitivity of the fingers. The symptom is explained by compression of the nerve due to changes in the soft tissues in the wrist area. Characteristic for acromegaly and signs of general malaise – reduced ability to work, unreasonable weakness and fatigue.

As the tumor grows in size, it puts pressure on healthy pituitary cells. The result of this is a violation of the normal functioning of the body. In this case, acromegaly is the cause of reproductive and sexual dysfunction, which manifests itself:

• menstrual disorders and female infertility;
• decreased potency and libido in men;
• various neurological symptoms;
• the appearance of headaches, poorly relieved by analgesics, sometimes – simultaneously with the appearance of photophobia and lacrimation.

Complications

Acromegaly, as it progresses, can cause high blood pressure and the development of arterial hypertension. Common complications of the disease in the form of cardiomyopathy (enlargement of the heart muscle in size) and other disorders of the cardiovascular system. In patients with acromegaly, diabetes mellitus, osteoarthritis, and thyroid pathology are not uncommon.

Other possible complications include:

• colon polyps;
• loss or impairment of vision;
• spinal cord compression;
• hypopituitarism – reduced secretion of pituitary hormones.

Patients with acromegaly often develop sleep apnea, a condition in which breathing stops briefly during sleep. For women, the risk of developing a benign tumor of the uterus – fibroids increases. With early treatment of acromegaly, the severity or likelihood of all these complications is reduced.

Risk of disease

If acromegaly is not treated on time, it leads to the development of severe complications and death. Approximately half of patients die before the age of 50 years. At the same time, the overwhelming number of deaths in acromegaly (60%) is associated with cardiovascular complications. Another quarter is pulmonary and respiratory complications and 15% – the development of oncological processes, most often the digestive system.

The danger of acromegaly is also that it often remains unrecognized. The rarity of this disease, as well as the gradual development of changes in appearance over a long period, do not arouse suspicion in the patient. Delayed diagnosis and treatment of acromegaly increases the risk of complications. With a timely visit to the doctor, on the contrary, it is possible to significantly reduce the external manifestations of the pathology and side effects.

Stages

There are 4 stages of acromegaly:

• preacromegaly;
• hypertrophic;
• tumor;
• cachectic.

The first stage is diagnosed extremely rarely due to the mild severity of symptoms characteristic of acromegaly.

In the hypertrophic stage, acromegaly can be recognized by enlargement of individual organs, changes in facial contours, and protrusion of the lower jaw. Characteristic at this stage is the coarsening of the voice, weight gain, visual impairment. There are frequent cases of excessive sweating due to the fact that the sweat glands also increase in size. General weakness and headaches are also included in the clinical picture of hypertrophic acromegaly.

At the tumor stage, the patient develops increased intracranial pressure. This can result in severe headaches, hearing loss and vision loss. The tumor stage of acromegaly is often accompanied by muscle atrophy and weakness.

The outcome of the disease is the cachectic stage. This is a complete exhaustion that develops in the absence of therapy.

Diagnostics

To diagnose acromegaly, the patient is prescribed an oral glucose tolerance test. Confirmation of the diagnosis is a biochemically elevated concentration of somatotropic hormone in the blood serum. The doctor also takes into account the level of insulin-like growth factor when diagnosing acromegaly.

Imaging studies are usually sufficient to assess the volume and extent of tumor spread. The clinical consequences of acromegaly are determined by taking echocardiography and analyzing sleep apnea attacks.

Treatment

Treatment for acromegaly includes surgery, radiation therapy, and drug suppression of growth hormone secretion.

Ablative therapy is indicated in most cases. The approach varies depending on the individual characteristics of the patient, but transsphenoidal resection is most often used due to minimal trauma. It is prescribed if the level of pituitary hormones, which is fixed after a glucose load, reaches normal values.

Irradiation of the tumor is carried out with an ultrahigh voltage current. In some cases, radiation therapy and surgery are combined.

Medical therapy is carried out if surgery and radiation therapy are contraindicated. The doctor prescribes drugs that effectively suppress the secretion of growth hormone. Somatostatin analogs or bromocriptine mesylate are most commonly used.

The vast majority of patients after a course of therapy show improvement. Acromegaly can be brought under control, as a result of which the patient’s life expectancy reaches the average value for the population. However, even with a high level of hormonal control and timely treatment, some negative consequences of the disease, including changes in the quality of life, regularly occurring joint pain, remain.

Diagnosis and treatment of acromegaly in Moscow

Alfa Health Center strongly recommends visiting an endocrinologist if symptoms of the disease are detected. Without laboratory tests, accurate diagnosis is impossible. Our clinic in Moscow is provided with everything necessary to promptly conduct an examination and begin treatment. Contact us, we are always happy to help.

Acromegaly

Acromegaly is a hormonal disorder in which the pituitary gland produces an excess amount of growth hormone in adulthood. It usually affects middle-aged people. In children, an excess of growth hormone is called gigantism and is characterized by rapid bone lengthening and abnormal growth.

In advanced cases, acromegaly is a severe, life-threatening condition, but the availability of treatments can prevent the risk of complications from developing.

Causes

Acromegaly is caused by an overproduction of growth hormone by the pituitary gland (the pituitary gland is a small gland located at the base of the brain, behind the pons, that produces numerous hormones). A hormone produced by the pituitary gland stimulates the production of insulin-like growth factor-1 in the liver. In turn, the latter stimulates the growth of bones and other tissues.

In adults, the most common cause of excess growth hormone production is a tumor.

• Benign pituitary tumor (adenoma) . Occurs most often. The tumor produces an excess amount of growth hormone, which leads to the development of symptoms of acromegaly.
• Non-pituitary tumors: eg tumors of the lung or pancreas. Sometimes these tumors produce the hormone somatoliberin, which stimulates the pituitary gland to produce large amounts of growth hormone.

Symptoms

Since acromegaly tends to develop slowly, early symptoms may not be apparent for many years.

Sometimes changes are detected on the part of the patient himself when comparing his old photographs with new ones:

• Disproportionate growth of arms and legs: changes are noticed when patients cannot put a ring that they wore constantly on their finger, or when the size gradually increases wearable shoes;
• gradual change in the shape of the face: characterized by a protruding lower jaw, enlarged nose, thickened lips and wide spaces between the teeth;
• coarse, enlarged facial features;
• rough, greasy, thickened skin;
• excessive sweating and body odor;
• skin papillomas;
• fatigue and muscle weakness;
• coarse, hoarse voice due to thickening of the vocal cords and changes in the shape of the paranasal sinuses;
• severe snoring due to constriction of the upper airways;
• visual disturbances and headaches due to compression of adjacent structures of the brain by the tumor that caused acromegaly;
• extended tongue;
• pain and limitation of movement in joints;
• enlarged organs, eg heart;
• sexual disorders: loss of interest in sex, menstrual irregularities in women and erectile dysfunction in men.

Possible complications

• Hypertension,
• cardiovascular diseases, especially cardiomyopathy,
• osteoarthritis,
• diabetes mellitus,
• goiter,
• precancerous growths on the colon mucosa (polyps),
• apnea during sleep: a condition in which breathing stops and resumes repeatedly during sleep,
• carpal tunnel syndrome,
• spinal cord compression,
• loss of vision,
• premature death.

When should I see a doctor?

Seek medical attention if you experience symptoms that may be associated with acromegaly, such as enlarged hands and facial changes. There is no need to delay visiting a doctor, as the patient needs to start receiving appropriate treatment and care as soon as possible.

Acromegaly usually develops slowly, so family members may not notice a change in the patient’s condition at first. At the first symptoms, it is necessary to undergo an appropriate examination.

Diagnosis

Diagnosis is carried out on the basis of the clinical picture, medical examination and anamnesis.

The following diagnostic methods may also be needed:

• Determination of blood levels of growth hormone and insulin-like growth hormone-1.
• Growth hormone suppression test (glucose test): measures growth hormone levels before and after taking a special glucose solution. Usually, glucose intake reduces the level of growth hormone, but in the case of acromegaly, its concentration remains high.
• MRI: to determine the exact location and size of a pituitary tumor.

Treatment

The main goal of treatment with is to reduce the level of growth hormone production and the effect of the tumor on the pituitary gland and surrounding tissues.

The patient may need various treatments:

• Surgical treatment: in general, the removal of pituitary tumors is performed endoscopically, with access through the nose (endoscopic transsphenoidal surgery). Removal of the tumor can correct the production of growth hormone and eliminate compression of the tissues surrounding the pituitary gland. In cases where it is impossible to remove the entire tumor, a stable increase in growth hormone levels is observed after surgery. In this case, there is a need for subsequent drug or radiation treatment.
• Drug therapy: drugs that reduce growth hormone production (somatostatin analogs), lower hormone levels (dopamine agonists), or drugs that block the effects of growth hormone.
• Radiation therapy: kill any remaining tumor cells and slowly lower the concentration of growth hormone. To noticeably reduce the symptoms of acromegaly, this treatment can take years.

Radiotherapy methods include:

• Conventional radiotherapy . It is carried out on working days, within 4-6 weeks.
• Proton beam therapy. Provides a high dose of radiation to the tumor while preventing exposure to adjacent normal tissue.