November 2014, 99(11):3933–3951 | Laurence Katzenelson, Edward R. Laws, Jr, Shlomo Melmed, Mark E. Molitch, Mohammad Hassan Murad, Andrea Utz, and John A. H. Wass
The Endocrine Society has developed clinical practice guidelines for the management of acromegaly, a chronic disorder caused by excessive growth hormone (GH) secretion. The guidelines aim to address important clinical issues regarding the evaluation and management of acromegaly, including biochemical assessment, therapeutic algorithms, and management during pregnancy. Key recommendations include:
1. **Diagnosis**: Measure IGF-1 levels in patients with typical clinical manifestations of acromegaly, especially those with acral and facial features. Measure IGF-1 in patients without typical manifestations but with associated conditions such as sleep apnea, type 2 diabetes, arthritis, carpal tunnel syndrome, hyperhidrosis, and hypertension. Measure serum IGF-1 to rule out acromegaly in patients with a pituitary mass. Avoid relying on random GH levels for diagnosis. Confirm the diagnosis by finding lack of suppression of GH to < 1 μg/L following documented hyperglycemia during an oral glucose load. Perform imaging studies to visualize tumor size and appearance, with MRI as the preferred modality.
2. **Presentation and Management of Comorbidities and Mortality Risk**: Evaluate all patients for associated comorbidities such as hypertension, diabetes, cardiovascular disease, osteoarthritis, and sleep apnea. longitudinally monitor and rigorously manage these comorbidities. Screen for colon neoplasia with colonoscopy at diagnosis. Assess for hypopituitarism and replace hormone deficits.
3. **Goals of Management**: Suggest a biochemical target goal of age-normalized serum IGF-1 values and a random GH < 1.0 μg/L as therapeutic goals. Maintain the same GH and IGF-1 assay throughout management.
4. **Surgery**: recommend transsphenoidal surgery as the primary therapy in most patients. Consider repeat surgery for residual intrasellar disease. Against routine preoperative medical therapy to improve biochemical control after surgery. For patients with severe pharyngeal thickness and sleep apnea, or high-output heart failure, suggest medical therapy with somatostatin receptor ligands (SRLs) preoperatively. In patients with parasellar disease, suggest surgical debulking to improve subsequent response to medical therapy. Measure IGF-1 and random GH at 12 weeks post-surgery and perform imaging studies to visualize residual tumor.
5. **Medical Therapy**: recommend medical therapy in patients with persistent disease following surgery. For significant disease, suggest use of SRLs or pegvisomant as initial adjuvant therapy. For modest elevations of serum IGF-1 and mild signs of GH excess, suggest a trial of a dopamine agonist like cabergoline. Suggest serial imaging with MRI to evaluate tumor size in patients receiving pegvisomant. Combine SRLs and cabergoline for mild to moderate GH excess. Suggest use of SRLs as primary therapy in patients who cannot be cured by surgeryThe Endocrine Society has developed clinical practice guidelines for the management of acromegaly, a chronic disorder caused by excessive growth hormone (GH) secretion. The guidelines aim to address important clinical issues regarding the evaluation and management of acromegaly, including biochemical assessment, therapeutic algorithms, and management during pregnancy. Key recommendations include:
1. **Diagnosis**: Measure IGF-1 levels in patients with typical clinical manifestations of acromegaly, especially those with acral and facial features. Measure IGF-1 in patients without typical manifestations but with associated conditions such as sleep apnea, type 2 diabetes, arthritis, carpal tunnel syndrome, hyperhidrosis, and hypertension. Measure serum IGF-1 to rule out acromegaly in patients with a pituitary mass. Avoid relying on random GH levels for diagnosis. Confirm the diagnosis by finding lack of suppression of GH to < 1 μg/L following documented hyperglycemia during an oral glucose load. Perform imaging studies to visualize tumor size and appearance, with MRI as the preferred modality.
2. **Presentation and Management of Comorbidities and Mortality Risk**: Evaluate all patients for associated comorbidities such as hypertension, diabetes, cardiovascular disease, osteoarthritis, and sleep apnea. longitudinally monitor and rigorously manage these comorbidities. Screen for colon neoplasia with colonoscopy at diagnosis. Assess for hypopituitarism and replace hormone deficits.
3. **Goals of Management**: Suggest a biochemical target goal of age-normalized serum IGF-1 values and a random GH < 1.0 μg/L as therapeutic goals. Maintain the same GH and IGF-1 assay throughout management.
4. **Surgery**: recommend transsphenoidal surgery as the primary therapy in most patients. Consider repeat surgery for residual intrasellar disease. Against routine preoperative medical therapy to improve biochemical control after surgery. For patients with severe pharyngeal thickness and sleep apnea, or high-output heart failure, suggest medical therapy with somatostatin receptor ligands (SRLs) preoperatively. In patients with parasellar disease, suggest surgical debulking to improve subsequent response to medical therapy. Measure IGF-1 and random GH at 12 weeks post-surgery and perform imaging studies to visualize residual tumor.
5. **Medical Therapy**: recommend medical therapy in patients with persistent disease following surgery. For significant disease, suggest use of SRLs or pegvisomant as initial adjuvant therapy. For modest elevations of serum IGF-1 and mild signs of GH excess, suggest a trial of a dopamine agonist like cabergoline. Suggest serial imaging with MRI to evaluate tumor size in patients receiving pegvisomant. Combine SRLs and cabergoline for mild to moderate GH excess. Suggest use of SRLs as primary therapy in patients who cannot be cured by surgery