AI Summary Overview
What is Acromegaly?
Acromegaly is an endocrine disorder that arises from an overproduction of growth hormone in the pituitary gland, a small, bean-shaped gland located in the brain. The condition does not increase height as the bone growth plates have already fused, but it does cause enlargement of the hands, feet, facial bones, and soft tissues.
If left untreated, acromegaly can cause serious complications, like high blood pressure, diabetes, heart disease, and sleep apnea. Early diagnosis and treatment can help control hormone levels and reduce the risk of complications.
What are the Types of Acromegaly?
The following are the types of acromegaly based on the source of excess growth hormone:
-
Pituitary-Dependent Acromegaly Pituitary-dependent acromegaly is the most common type of this condition. It occurs when a non-cancerous pituitary adenoma leads to the secretion of excessive amounts of growth hormone without the control of the body's own regulatory mechanisms.
-
Ectopic Acromegaly In ectopic acromegaly, a tumour occurs outside the pituitary gland (e.g., lungs, intestines, or pancreas), leading to the excessive production of either GH or growth hormone-releasing hormone (GHRH). This disorder is usually a very rare condition.
How Common is Acromegaly in India?
In India, acromegaly has a reported annual incidence of 0.49 per lakh person-years, based on clinical studies. Despite being a rare condition, it is often diagnosed late, typically between 30 and 50 years of age. The gradual onset of symptoms and their similarity to other health conditions can delay recognition and lead to misdiagnosis.
A significant number of patients with acromegaly have pituitary macroadenomas, which are tumours larger than 1 cm. Before reaching an endocrinologist, patients may first consult orthopaedic surgeons, dermatologists or dental practitioners for individual symptoms or physical changes. This often contributes to delays in identifying the underlying hormonal disorder.
What are the Symptoms of Acromegaly?
The symptoms of acromegaly usually develop slowly over several years, making the condition difficult to recognise during its early stages. As growth hormone levels remain elevated, the physical changes become more noticeable and complications may develop.
The common early warning signs and advanced symptoms of acromegaly are listed below:
| Early Warning Signs | Advanced Symptoms |
|
|
What are the Causes of Acromegaly?
The causes of acromegaly include:
-
GH-Secreting Pituitary Adenoma: It is a benign tumour in the somatotroph cells of the pituitary gland that produces growth hormone.
-
GHRH: Rare neuroendocrine tumours outside the pituitary gland may secrete GHRH, which stimulates excess GH production, which indirectly stimulates excess GH production.
-
Hereditary Genetic Disorder:
Some forms of acromegaly are due to genetic inheritance and include the following three disorders, which are characterised by mutations in tumour-suppressor genes:
- Multiple Endocrine Neoplasia Type 1 (MEN1)
- Carney syndrome
- Familial Isolated Pituitary Adenoma (FIPA)
-
Familial Isolated Pituitary Adenoma (FIPA): It is a condition where pituitary tumours run in families. Individuals with AIP gene mutations frequently develop growth hormone- or prolactin-secreting adenomas at a characteristically early age.
Stages of Acromegaly
There is no clinically recognised staging approach for acromegaly. However, the disease normally develops slowly as the ongoing excess of growth hormone (GH) and insulin-like growth factor-1 (IGF-1) involves bones, soft tissues and internal organs. Early detection and treatment of the disease can slow its progression and lessen the risk of significant complications.
The progression of acromegaly is outlined below for better understanding:
| Progression Phase | Features |
| Phase 1: Early Hormonal Changes | Growth hormone and IGF-1 levels begin to rise, but physical changes are often subtle. Patients may notice increased sweating, headaches, fatigue, or a gradual increase in shoe or ring size. |
| Phase 2: Progressive Physical Changes | Enlargement of the hands, feet, jaw, nose, and facial bones becomes more noticeable. Joint pain, thickened skin, excessive sweating, and changes in facial appearance gradually develop. |
| Phase 3: Advanced Disease with Complications | Long-term hormone excess may lead to diabetes, high blood pressure, heart disease, sleep apnoea, arthritis, vision problems, and pituitary hormone deficiencies if left untreated. |
Diseases Similar to Acromegaly
Generally, diseases such as gigantism and Cushing syndrome show similar symptoms to those of acromegaly. Several endocrine disorders can cause hormonal imbalance and physical changes comparable to those seen in acromegaly. However, the age of onset, the hormone involved and clinical characteristics are different amongst these diseases.
The key differences between acromegaly and similar endocrine disorders are briefly given in the table below:
Acromegaly Vs. Gigantism Vs. Cushing Syndrome
| Feature | Acromegaly | Gigantism | Cushing Syndrome |
| How it Happens | Excess growth hormone is produced after the growth plates have closed, usually due to a pituitary adenoma. | Excess growth hormone develops before the growth plates close during childhood, causing excessive height. | Excess cortisol is produced due to prolonged steroid use or disorders affecting the pituitary or adrenal glands. |
| Affected Area | Bones of the hands, feet, face, soft tissues, and several internal organs | Entire skeleton and body growth | Whole body, particularly fat distribution, muscles, skin, and metabolism |
| Associated Symptoms | Enlarged hands and feet, coarse facial features, joint pain, sweating, headaches, and vision problems | Excessive height, enlarged hands and feet, delayed puberty, headaches, and vision changes | Round face, weight gain around the abdomen, muscle weakness, purple stretch marks, high blood pressure, and diabetes |
| Severity | Can cause serious long-term complications if untreated | May result in permanent skeletal abnormalities and metabolic complications | May lead to cardiovascular disease, osteoporosis, infections, and diabetes if untreated |
| Can It Be Reversed? | Partially. Hormone levels can often be controlled, and disease progression can be stopped. However, some bone enlargement may be permanent. | Partially. Early treatment can control hormone levels, but excessive height and skeletal changes cannot usually be reversed. | Often yes. Many patients improve after treating the underlying cause, although recovery depends on the duration and severity of cortisol excess. |
What are the Complications Associated With Acromegaly?
Complications associated with untreated acromegaly include the following:
-
Cardiovascular Complications: This is the most common and life-threatening complication. GH produces an enlarged heart, hypertension, and arrhythmias. Heart complications have been observed to be the leading cause of premature death among patients suffering from acromegaly.
-
Diabetes Mellitus Type 2: Insulin resistance occurs due to GH interference with insulin function and results in poor glucose tolerance.
-
Colon Cancer: Colon cells are stimulated by IGF-1, which is associated with an increased risk of colorectal cancer among patients with acromegaly.
-
Osteoarthritis: Continuous presence of elevated levels of GH brings about metabolic effects and degeneration of bones and joints, leading to an arthritic condition that might require bone replacement surgery.
-
Sleep Apnea: When sleep apnea is not treated because of acromegaly, increased exhaustion during the day can lead to cardiovascular conditions, including hypertension.
-
Loss of Vision: Pressure exerted on the optic chiasm by the growth can cause loss of vision through optic atrophy if the pressure is not relieved.
-
Hypopituitarism: A tumour in the pituitary gland can bring about damage to the normal pituitary gland, causing a hormonal imbalance.
When Should I See My Healthcare Provider?
Consider consulting an endocrinologist if you display any of the following acromegaly symptoms:
-
Growth in the size of hands and feet among adults
-
Coarsening of facial appearance with features such as an enlargement of the jaw, nose, and forehead
-
Headaches that are persistent and cannot be managed with over-the-counter medication
-
Vision problems with trouble seeing things out of your peripheral vision
-
Excessive sweating without a clear cause, and when no physical exertion or extreme heat has been experienced
-
Distress and inflammation in joints involving more than one joint without any known cause
-
Adults younger than 50 years suffering from sleep apnea, Type 2 diabetes mellitus, and hypertension
-
Have a family history of pituitary tumours and multiple endocrine neoplasias
How is Acromegaly Diagnosed?
Below are the medical tests that can help diagnose acromegaly:
| Test Name | Normal Range | Borderline | Confirmed Diagnosis |
| IGF-1 (Age & Sex Adjusted) | Normal range for age & sex | Above normal limit | Increased above normal age-adjusted levels |
| OGTT with 75g Glucose (Nadir GH Level) | GH suppresses below 0.4 ng/mL | GH suppresses to 0.4–1.0 ng/mL | GH fails to suppress (remains ≥ 0.4 ng/mL) |
| Fasting Blood Glucose | <100 mg/dL | Between 100-125 mg/dl (pre-diabetes) | >126mg/dl (Diabetes Mellitus) |
Note: The test results for IGF-1 need to be considered against the backdrop of age and sex-specific normal values. The presence of high levels of IGF-1 may confirm acromegaly, but the OGTT-GH test needs to be conducted as well.
How is Acromegaly Treated?
Treatment of acromegaly often includes the following methods:
-
Surgery (Transsphenoidal Adenomectomy) Surgery represents one of the best options in treating the majority of patients suffering from acromegaly. It involves excision of the tumour through a nasal and sinus route (no incisions are needed) by using either endoscopy or microscopic techniques.
-
Drug Therapy Drug therapy includes cases where surgery is not applicable due to certain complications, failure of surgical treatment, or to reduce tumour size before carrying out surgery.
-
Radiation Therapy Where control of the disease cannot be achieved by means of surgery and medicines, gamma knife or fractionation radiation therapy is used. The complication arising from this kind of treatment is hypopituitarism (failure of the pituitary gland to produce hormone(s)), which may occur later post-therapy.
-
Monitoring and Complications Doctors regularly monitor patients based on their IGF-1 and GH levels, perform colonoscopy to check for developing colon cancer, and assess their cardiovascular system, sleep apnea, and joints.
Common Medicines Used to Treat Acromegaly
Medicines for acromegaly help reduce growth hormone production, lower insulin-like growth factor-1 (IGF-1) levels, shrink certain pituitary tumours, and control symptoms. They are commonly prescribed when surgery is not suitable, before surgery to reduce tumour size or if hormone levels remain high after surgery.
Common medicines used to treat acromegaly include:
-
Octreotide LAR: Octreotide lowers growth hormone and IGF-1 levels and may reduce pituitary tumour size. Long-acting octreotide formulations are available and commonly used in Indian specialist practice.
-
Lanreotide Autogel: Lanreotide reduces growth hormone secretion and IGF-1 levels. It is an alternative to octreotide for long-term control, although availability may differ across Indian treatment centres.
-
Cabergoline: Cabergoline is an oral medicine used mainly when IGF-1 levels are only mildly elevated or when the pituitary tumour also produces prolactin. It may be combined with octreotide or lanreotide when one medicine does not provide adequate control.
-
Pasireotide LAR: Pasireotide may be prescribed when acromegaly remains uncontrolled despite octreotide or lanreotide. It requires close blood-glucose monitoring because it can cause or worsen hyperglycaemia and diabetes.
-
Pegvisomant: Pegvisomant blocks the effects of growth hormone and lowers IGF-1 levels. It may be considered when other medicines do not adequately control acromegaly, particularly when glucose intolerance is present. Liver-function tests and pituitary imaging are required during treatment.
Note: Medicines may control acromegaly, but they do not permanently cure most patients. Surgery remains the preferred treatment for many growth hormone-secreting pituitary tumours. The choice of medicine depends on the tumour size, hormone levels, response to previous treatment, and the patient's overall health. An endocrinologist should always guide treatment.
Living with Acromegaly: Daily Challenges and Caregiving
Living with acromegaly can affect appearance, mobility, sleep, heart health and emotional well-being. Regular monitoring allows for maintaining hormone control, early detection of recurrence and decreasing the risk of consequences, which might impact everyday living.
Some of the common challenges and essential care points of acromegaly patients include:
-
Challenges
- Joint and Muscle Problems: Joint pain, stiffness, weakness and enlarged hands or feet may affect mobility and daily activities.
- Changes in Appearance: Facial and physical changes may affect confidence, body image and social interactions.
- Sleep and Energy Problems: Sleep apnoea, headaches and persistent tiredness may interfere with work and concentration.
- Associated Health Conditions: Diabetes, high blood pressure, heart disease and vision problems may require additional treatment.
-
Caregiving and Patient Safety
- Regular Hormone Monitoring: Treatment success and tumour recurrence are monitored by routine blood testing for GH and IGF-1 levels and occasional MRI scans.
- Medicine Adherence: Caregivers can support patients in taking medicines as prescribed and attending follow-up appointments with the endocrinologist.
- Monitoring for Complications: Regularly check blood pressure, blood glucose, eyesight, heart health and sleep-related symptoms to identify issues early.
- Supporting Healthy Lifestyle Habits: Maintaining a healthy weight, staying physically active within individual limits, and controlling associated conditions such as diabetes can improve long-term outcomes.
-
Expenses
- Regular Specialist Consultations: Long-term treatment typically involves multiple endocrinology appointments, hormone assays, and imaging tests.
- Medicines and Monitoring: Patients requiring lifelong medicines or regular injections should plan for ongoing treatment expenses and laboratory investigations.
- Surgery and Radiotherapy: Some people may require surgery to the pituitary gland, and further surgery or radiotherapy if hormone levels are still unstable.
- Financial Planning: Comprehensive health insurance with adequate coverage can help manage the cost of surgery, medicines, hospitalisation and long-term follow-up.
What is the Prognosis for Acromegaly?
The prognosis for acromegaly patients diagnosed early and receiving appropriate treatment is usually favourable. Once they undergo successful treatment, their heart disease, sleep apnea, and glucose intolerance will be managed.
Acromegaly remains a manageable disease if IGF-1 is normalised through continuous medical treatment. This gives patients a good quality of life.
How Can I Prevent Acromegaly?
Acromegaly usually cannot be avoided or prevented, but the following tips can help ensure proper management and minimise the issues associated with the condition:
-
Consult geneticists in case of a family history of pituitary adenomas, MEN1 syndrome, or Carney complex.
-
Report changes in the dimensions of shoes and rings to the physician.
-
Regularly check the blood pressure, blood glucose, and cholesterol levels.
-
Avoid any delays in seeing the endocrinologist due to headaches and facial or limb changes.
-
Consult the doctor if diagnosed with Type 2 diabetes mellitus or obstructive sleep apnea before the age of 50, and if no other risk factors are present.
Acromegaly Treatment Cost in India
The cost of acromegaly treatment in India depends on the size of the pituitary tumour, the treatment approach, the need for long-term medicines and whether surgery or radiotherapy is required. While surgery is often the primary treatment, some patients may require lifelong follow-up and hormone control.
An overview of the approximate acromegaly treatment expenses is provided below:
| Treatment Component | Approximate Cost Range |
| Endocrinologist Consultation | ₹800 - ₹2,500 per visit |
| IGF-1 Blood Test | ₹1,500 - ₹4,000 |
| Growth Hormone Suppression Test (Glucose) | Up to ₹3,500 |
| MRI Brain (Pituitary) | ₹2,200 - ₹10,000 |
| Visual Field Examination | ₹8,00 - ₹2,500 |
| Transsphenoidal Pituitary Surgery | ₹1.5 lakh - ₹3 lakh+ |
| Radiotherapy (if required) | ₹60,000 - ₹3 lakh |
Note: The above costs are approximate and may vary depending on the hospital, city, doctor's consultation fees, diagnostic tests, tumour size, treatment approach, duration of hospitalisation, and the medicines prescribed. Long-term medical therapy, particularly with injectable hormone-controlling medicines, may significantly increase the overall treatment cost for some patients.
Does Health Insurance Cover Acromegaly?
Yes. Health insurance does cover acromegaly treatment. The coverage includes hospitalisation, surgical removal of pituitary tumours, radiotherapy, and diagnostic testing, depending on the policy terms. Ongoing medical therapy is often managed on an outpatient basis and may only be covered if your policy includes an outpatient (OPD) benefit.
However, if there is an underlying genetic condition, such as multiple endocrine neoplasia type 1 (MEN1), then it is considered a pre-existing condition. In such cases, a waiting period of up to 3 years may apply before you file a claim.
Disclaimer: Insurance coverage, waiting periods, and policy benefits vary by provider and region. Please consult your insurance provider directly to confirm your specific coverage details.
How Much Health Insurance Coverage is Needed for Acromegaly Treatment?
Coverage for treatment of acromegaly depends on the severity of the condition, the size of the tumour, the treatment method, and the hospital. Patients who require ongoing treatment with medication, repeat surgeries, radiotherapy, and management of underlying conditions may require coverage of ₹15 to ₹20 lakh.
Coverage exclusions and inclusions vary by policy, so review policy details and terms and conditions carefully.
Frequently Asked Questions (FAQs)
-
Q1. What are acromegaly symptoms?
Ans: Acromegaly symptoms usually include enlargement of the foot and hand bones in adults, a coarse facial structure with enlarged jaws, nasal bones, and brow ridges, and the need for larger-sized shoes and rings. -
Q2. Is acromegaly the same as gigantism?
Ans: No. Gigantism is diagnosed in children and teenagers when their bone growth plates remain open, leading to height growth. In acromegaly, the fusion process is completed, causing an excessive growth of feet, hands, and face in adults. -
Q3. Can acromegaly be cured?
Ans: If the pituitary tumour is successfully removed surgically, some patients may achieve long-term remission or cure from acromegaly, while others may require long-term treatment.
-
References
-
https://www.niddk.nih.gov/health-information/endocrine-diseases/acromegaly
-
https://www.ncbi.nlm.nih.gov/books/NBK431086/
-
https://www.apollohospitals.com/procedures/transsphenoidal-pituitary-tumor-surgery
-
https://www.yashodahospitals.com/procedure-cost/glucose-tolerance-test-during-pregnancy-ogtt-cost-in-india/
-
