ICD-10: E26.02

Glucocorticoid-remediable aldosteronism (GRA) is a specific form of primary hyperaldosteronism characterized by the overproduction of aldosterone, a hormone that regulates sodium and potassium levels in the body. The condition is linked to a genetic mutation that causes the adrenal glands to produce excess aldosterone in response to adrenocorticotropic hormone (ACTH). This results in hypertension and electrolyte imbalances, which can lead to various health complications if left untreated.

Clinical Description

Pathophysiology

GRA is caused by a genetic rearrangement that leads to the expression of the aldosterone synthase gene under the control of the ACTH-responsive promoter. This means that, unlike typical cases of primary hyperaldosteronism, where aldosterone production is primarily regulated by the renin-angiotensin system, GRA patients have aldosterone production that is responsive to glucocorticoids. This unique mechanism allows for the potential treatment of the condition with glucocorticoids, which can suppress aldosterone production and alleviate symptoms[1][2].

Symptoms

Patients with GRA often present with:
- Hypertension: High blood pressure is the most common symptom, often resistant to standard antihypertensive treatments.
- Hypokalemia: Low potassium levels can lead to muscle weakness, fatigue, and arrhythmias.
- Metabolic Alkalosis: This condition can occur due to the loss of hydrogen ions and potassium, leading to an imbalance in the body's acid-base status.
- Increased Urinary Aldosterone: Patients may exhibit elevated levels of aldosterone in urine tests, which is a hallmark of the condition[3][4].

Diagnosis

Diagnosis of GRA typically involves:
- Plasma Aldosterone Concentration (PAC): Elevated levels of aldosterone in the blood.
- Plasma Renin Activity (PRA): Low renin levels, which is indicative of primary hyperaldosteronism.
- Genetic Testing: Identification of the specific genetic mutation associated with GRA can confirm the diagnosis.
- Suppression Testing: Administration of glucocorticoids to assess the suppression of aldosterone production can also be diagnostic[5][6].

ICD-10 Code E26.02

The ICD-10-CM code E26.02 specifically refers to glucocorticoid-remediable aldosteronism. This code is part of the broader category of hyperaldosteronism (E26), which encompasses various forms of the condition. The designation of E26.02 is crucial for accurate medical coding, billing, and epidemiological tracking of this specific disorder.

Clinical Implications

Understanding and correctly coding GRA is essential for:
- Treatment Planning: Effective management often involves the use of glucocorticoids, which can significantly improve patient outcomes.
- Monitoring: Regular follow-up and monitoring of blood pressure and electrolyte levels are necessary to prevent complications.
- Research and Epidemiology: Accurate coding helps in the collection of data for research purposes, contributing to a better understanding of the condition and its prevalence[7][8].

Conclusion

Glucocorticoid-remediable aldosteronism is a genetically driven form of primary hyperaldosteronism that can lead to significant health issues if not properly diagnosed and managed. The ICD-10 code E26.02 is vital for healthcare providers to ensure appropriate treatment and monitoring of affected patients. Early recognition and intervention can lead to improved health outcomes and quality of life for individuals with this condition.

References

1. ICD-10-CM Code for Glucocorticoid-remediable aldosteronism E26.02 - AAPC.
2. 2025 ICD-10-CM Diagnosis Code E26.02 - The Web's Free 2023 ICD-10-CM.
3. Hyperaldosteronism E26 - ICD-10-CM Codes.
4. E26.02 Glucocorticoid-remediable aldosteronism - ICD-10-CM.
5. ICD-10-CM Code for Primary hyperaldosteronism E26.0.
6. 2025 ICD-10-CM Diagnosis Code E26: Hyperaldosteronism.
7. Other endocrine and metabolic disorders.
8. Glucocorticoid-remediable aldosteronism - E26.02 ICD 10 Code.

Glucocorticoid-remediable aldosteronism (GRA), classified under ICD-10 code E26.02, is a rare form of primary aldosteronism characterized by excessive production of aldosterone due to a genetic mutation. This condition is particularly notable for its unique response to glucocorticoid therapy, which can suppress aldosterone secretion. Below, we explore the clinical presentation, signs, symptoms, and patient characteristics associated with GRA.

Clinical Presentation

Overview

GRA typically presents in individuals with hypertension and hypokalemia, often at a younger age than those with other forms of primary aldosteronism. The condition is caused by a genetic mutation that leads to the inappropriate production of aldosterone, primarily due to the fusion of the aldosterone synthase gene with the 11β-hydroxylase gene, resulting in a chimeric protein that is regulated by adrenocorticotropic hormone (ACTH) rather than the renin-angiotensin system.

Age of Onset

Patients with GRA often present in their 20s to 40s, which is earlier than the typical age of onset for other forms of primary aldosteronism, where patients are usually diagnosed in their 30s to 60s[12][13].

Signs and Symptoms

Common Symptoms

1. Hypertension: The most prominent symptom, often resistant to standard antihypertensive treatments. Patients may experience severe hypertension that can lead to complications such as cardiovascular disease[12].
2. Hypokalemia: Low potassium levels are common, leading to symptoms such as muscle weakness, fatigue, and cramps. In severe cases, hypokalemia can cause arrhythmias[12][13].
3. Metabolic Alkalosis: Due to the loss of hydrogen ions and potassium, patients may develop metabolic alkalosis, which can manifest as muscle twitching or spasms[12].
4. Headaches: Often related to elevated blood pressure, patients may report frequent headaches[12].
5. Polyuria and Polydipsia: Increased urination and thirst can occur due to the effects of aldosterone on renal function[12].

Physical Examination Findings

• Elevated Blood Pressure: Persistent hypertension is a hallmark finding during clinical evaluation.
• Signs of Hypokalemia: Physical signs may include muscle weakness or cramping, and in severe cases, signs of cardiac arrhythmias may be observed[12][13].

Patient Characteristics

Demographics

• Gender: GRA can affect both males and females, but some studies suggest a slight male predominance[12].
• Family History: Given its genetic basis, a family history of hypertension or early cardiovascular disease may be present in affected individuals[12][13].

Genetic Considerations

GRA is often inherited in an autosomal dominant pattern, and genetic testing may reveal mutations in the genes associated with aldosterone production. This genetic predisposition is crucial for understanding the familial nature of the condition and its implications for screening family members[12][13].

Comorbidities

Patients with GRA may have associated conditions due to long-standing hypertension, including:
- Cardiovascular disease
- Chronic kidney disease
- Metabolic syndrome

Conclusion

Glucocorticoid-remediable aldosteronism (ICD-10 code E26.02) is characterized by early-onset hypertension and hypokalemia, with a unique response to glucocorticoid therapy. Understanding the clinical presentation, signs, symptoms, and patient characteristics is essential for timely diagnosis and management. Given its genetic basis, awareness of family history and potential screening for relatives can be beneficial in managing this condition effectively. Early intervention can help mitigate the long-term complications associated with uncontrolled hypertension and hypokalemia.

Glucocorticoid-remediable aldosteronism (GRA), represented by the ICD-10 code E26.02, is a specific form of primary hyperaldosteronism characterized by the overproduction of aldosterone that can be suppressed by glucocorticoids. Understanding the alternative names and related terms for this condition can enhance clarity in medical documentation and communication. Below are some of the key alternative names and related terms associated with E26.02.

Alternative Names for Glucocorticoid-Remediable Aldosteronism

1. Familial Hyperaldosteronism Type I: This term is often used interchangeably with glucocorticoid-remediable aldosteronism, particularly in genetic contexts, as GRA is inherited in an autosomal dominant manner.

2. GRA Syndrome: A shorthand reference to glucocorticoid-remediable aldosteronism, emphasizing its syndromic nature.

3. Aldosterone-Producing Adenoma (APA): While not identical, this term is related as both conditions involve excessive aldosterone production. However, GRA is distinct in that it is responsive to glucocorticoids.

4. Glucocorticoid-Sensitive Hyperaldosteronism: This term highlights the unique feature of GRA where aldosterone secretion can be suppressed by glucocorticoids.

Related Terms

1. Primary Hyperaldosteronism (PHA): A broader category that includes conditions like GRA and aldosterone-producing adenomas. It refers to the overproduction of aldosterone from the adrenal glands.

2. Conn's Syndrome: This term is often used to describe primary hyperaldosteronism, particularly when caused by an adrenal adenoma. It is important to note that GRA is a specific subtype of primary hyperaldosteronism.

3. Adrenal Hyperplasia: In some cases, GRA may be associated with adrenal hyperplasia, a condition where the adrenal glands are enlarged and produce excess hormones.

4. Mineralocorticoid Excess: This term refers to the overall condition of having excess mineralocorticoids, including aldosterone, which is a hallmark of GRA.

5. Aldosterone Escape: A phenomenon that can occur in patients with GRA where aldosterone levels may rise despite glucocorticoid treatment, although this is more commonly discussed in the context of other forms of hyperaldosteronism.

Conclusion

Understanding the alternative names and related terms for glucocorticoid-remediable aldosteronism (ICD-10 code E26.02) is crucial for accurate diagnosis, treatment, and communication among healthcare professionals. These terms not only facilitate better understanding of the condition but also help in distinguishing it from other forms of hyperaldosteronism. If you have further questions or need more specific information, feel free to ask!

Glucocorticoid-remediable aldosteronism (GRA) is a rare form of primary aldosteronism characterized by the overproduction of aldosterone due to a genetic mutation. The diagnosis of GRA, which corresponds to the ICD-10 code E26.02, involves several criteria and diagnostic steps. Below is a detailed overview of the criteria used for diagnosing this condition.

Clinical Presentation

Symptoms

Patients with GRA may present with symptoms related to hyperaldosteronism, which can include:
- Hypertension: Often resistant to standard antihypertensive treatments.
- Hypokalemia: Low potassium levels, which can lead to muscle weakness, fatigue, and arrhythmias.
- Metabolic Alkalosis: An increase in blood pH due to the loss of hydrogen ions.

Laboratory Tests

Hormonal Evaluation

1. Plasma Aldosterone Concentration (PAC): Elevated levels of aldosterone are typically found in patients with GRA.
2. Plasma Renin Activity (PRA): In GRA, the renin levels are usually suppressed due to the feedback mechanism of high aldosterone levels. The PAC/PRA ratio is often used to assess the likelihood of primary aldosteronism.

Genetic Testing

• Identification of Genetic Mutations: Genetic testing can confirm the presence of mutations in the CYP11B1 and CYP11B2 genes, which are responsible for the enzyme pathways leading to aldosterone production. The presence of a chimeric gene resulting from the fusion of these genes is indicative of GRA.

Imaging Studies

Adrenal Imaging

• CT or MRI of the Adrenal Glands: Imaging studies may be performed to rule out adrenal adenomas or other structural abnormalities. In GRA, imaging may not show significant abnormalities, as the condition is often due to genetic factors rather than tumors.

Response to Treatment

Glucocorticoid Suppression Test

• Response to Glucocorticoids: A hallmark of GRA is the suppression of aldosterone production in response to glucocorticoid therapy. Patients may show a significant decrease in plasma aldosterone levels after administration of glucocorticoids, which is not typically seen in other forms of primary aldosteronism.

Differential Diagnosis

Exclusion of Other Conditions

• It is essential to differentiate GRA from other causes of hyperaldosteronism, such as adrenal adenomas (Conn's syndrome) or bilateral adrenal hyperplasia. This is typically done through hormonal assays and imaging studies.

Summary

In summary, the diagnosis of glucocorticoid-remediable aldosteronism (ICD-10 code E26.02) involves a combination of clinical evaluation, hormonal testing, genetic analysis, and response to glucocorticoid treatment. The identification of elevated aldosterone levels, suppressed renin activity, and a positive response to glucocorticoids are critical components of the diagnostic criteria. Proper diagnosis is essential for effective management and treatment of this condition, which can significantly impact a patient's quality of life due to its association with hypertension and electrolyte imbalances.

Glucocorticoid-remediable aldosteronism (GRA), classified under ICD-10 code E26.02, is a genetic condition characterized by excessive production of aldosterone, a hormone that regulates sodium and potassium levels in the body. This condition is particularly notable because it can be effectively managed with glucocorticoids, which suppress the overproduction of aldosterone. Below, we explore the standard treatment approaches for GRA, including pharmacological interventions, monitoring, and lifestyle modifications.

Pharmacological Treatment

Glucocorticoids

The cornerstone of treatment for glucocorticoid-remediable aldosteronism is the administration of glucocorticoids. These medications help to reduce the secretion of aldosterone by providing negative feedback to the adrenal glands. Commonly used glucocorticoids include:

• Hydrocortisone: This is often the first-line treatment due to its effectiveness and safety profile.
• Prednisone: Another option that may be used depending on the patient's specific needs and response to treatment.

The dosage of glucocorticoids is typically tailored to the individual, aiming to achieve normal aldosterone levels while minimizing side effects associated with long-term glucocorticoid use, such as weight gain, osteoporosis, and increased risk of infections[1][2].

Mineralocorticoid Receptor Antagonists

In some cases, particularly when glucocorticoids alone are insufficient to control blood pressure or manage symptoms, mineralocorticoid receptor antagonists like spironolactone or eplerenone may be added to the treatment regimen. These medications help counteract the effects of excess aldosterone, particularly in managing hypertension associated with GRA[3].

Monitoring and Follow-Up

Regular Monitoring

Patients diagnosed with GRA require regular monitoring to assess the effectiveness of treatment and to adjust dosages as necessary. Key parameters to monitor include:

• Blood pressure: Regular checks are essential, as hypertension is a common complication of GRA.
• Electrolyte levels: Monitoring sodium and potassium levels helps to ensure that the treatment is effectively managing aldosterone levels without causing significant imbalances.
• Aldosterone levels: Periodic measurement of plasma aldosterone concentration can help determine the adequacy of treatment[4].

Endocrinology Consultations

Regular follow-ups with an endocrinologist are recommended to ensure comprehensive management of the condition. This includes evaluating the patient's response to treatment, managing any side effects, and making necessary adjustments to the treatment plan.

Lifestyle Modifications

Dietary Changes

Patients with GRA may benefit from dietary modifications, particularly in sodium intake. A low-sodium diet can help manage hypertension and reduce the burden on the cardiovascular system. Additionally, ensuring adequate potassium intake is important, especially if potassium levels are low due to the effects of aldosterone[5].

Physical Activity

Encouraging regular physical activity can also be beneficial. Exercise helps in managing blood pressure and improving overall cardiovascular health. However, patients should consult with their healthcare provider to develop a safe and effective exercise plan tailored to their condition.

Conclusion

In summary, the standard treatment for glucocorticoid-remediable aldosteronism primarily involves glucocorticoid therapy, with potential adjunctive use of mineralocorticoid receptor antagonists. Regular monitoring and lifestyle modifications play crucial roles in managing the condition effectively. Patients should work closely with their healthcare providers to ensure optimal treatment outcomes and to address any complications that may arise during the course of therapy.