### Date : 2024-06-19 18:11 ### Topic : Cushing Syndrome #medicine #internalmedicine ---- ### Cushing Syndrome Overview **Cushing syndrome** is a condition caused by prolonged exposure to high levels of cortisol, a hormone produced by the adrenal glands. It can result from endogenous overproduction of cortisol or exogenous sources, such as long-term use of corticosteroid medications. ### Pathophysiology Cushing syndrome can be classified into two main types based on the source of excess cortisol: 1. **Endogenous Cushing Syndrome:** The body produces too much cortisol, which can occur due to: - **[[Pituitary Adenomas (Cushing's Disease)]]:** Benign tumors in the pituitary gland that produce excess adrenocorticotropic hormone (ACTH), stimulating the adrenal glands to produce more cortisol. - **Adrenal Adenomas or Carcinomas:** Tumors in the adrenal glands that directly increase cortisol production. - **[[Ectopic ACTH Syndrome]]:** Non-pituitary tumors (often in the lungs) that produce ACTH. 2. **Exogenous Cushing Syndrome:** ==Prolonged use of corticosteroid medications== like prednisone or dexamethasone. ### Clinical Features **Physical Symptoms:** - **Weight Gain:** Particularly in the face (moon face), upper back (buffalo hump), and abdominal area. - **Skin Changes:** Thin, fragile skin that bruises easily, purple stretch marks (striae) on the abdomen, thighs, and breasts. - **Muscle Weakness:** Especially in the upper arms and thighs. - **Osteoporosis:** Bone thinning, leading to fractures. - **Hypertension:** High blood pressure. - **Hyperglycemia:** High blood sugar levels, which can lead to diabetes. **Psychological Symptoms:** - **Mood Changes:** Irritability, anxiety, depression. - **Cognitive Impairment:** Memory and concentration difficulties. **Other Symptoms:** - **Menstrual Irregularities:** In women, irregular or absent menstrual periods. - **Hirsutism:** Excessive hair growth on the face and body in women. - **Decreased Libido and Erectile Dysfunction:** In men. ### Diagnosis Diagnosis of Cushing syndrome involves a combination of clinical evaluation, laboratory tests, and imaging studies: 1. **Clinical History and Physical Examination:** - Assess for characteristic signs and symptoms. 2. **Laboratory Tests:** - **24-Hour Urinary Free Cortisol Test:** Measures cortisol levels in urine collected over 24 hours. - **Late-Night Salivary Cortisol Test:** Measures cortisol levels in saliva late at night when they should be low. - **Low-Dose Dexamethasone Suppression Test:** Evaluates how cortisol levels respond to synthetic glucocorticoids. 3. **Imaging Studies:** - **MRI of the Pituitary Gland:** To detect pituitary tumors. - **CT or MRI of the Adrenal Glands:** To identify adrenal tumors. - **CT Scan of the Chest and Abdomen:** To locate ectopic ACTH-producing tumors. ### Example Case Study **Patient Profile:** - **Name:** Jee Hoon Ju - **Age:** 45 - **Occupation:** Accountant **Medical History:** - **Symptoms:** Jee Hoon Ju reports significant weight gain over the past year, particularly in his face and abdomen. He has noticed purple stretch marks on his abdomen, increased facial hair growth, and easy bruising. He also experiences high blood pressure and elevated blood sugar levels. **Physical Examination:** - **Facial Appearance:** Rounded face (moon face). - **Upper Back:** Fat deposition (buffalo hump). - **Skin:** Thin skin with purple striae on the abdomen. **Laboratory Tests:** - **24-Hour Urinary Free Cortisol:** Elevated cortisol levels. - **Late-Night Salivary Cortisol:** Elevated cortisol levels. - **Low-Dose Dexamethasone Suppression Test:** No suppression of cortisol levels. **Imaging:** - **MRI of the Pituitary Gland:** Reveals a pituitary adenoma. **Diagnosis:** Based on the clinical features, laboratory test results, and imaging findings, Jee Hoon Ju is diagnosed with Cushing's disease due to a pituitary adenoma. ### Management 1. **Surgical Treatment:** - **Transsphenoidal Surgery:** To remove the pituitary adenoma. - **Adrenalectomy:** In cases of adrenal tumors, surgical removal of the adrenal gland may be necessary. - **Ectopic ACTH-Producing Tumors:** Surgical removal of the tumor if located. 2. **Medications:** - **Steroidogenesis Inhibitors:** Medications such as ketoconazole or metyrapone to reduce cortisol production. - **Pituitary-Directed Drugs:** Medications like cabergoline or pasireotide for pituitary adenomas. 3. **Radiation Therapy:** - For patients with pituitary tumors who cannot undergo surgery or if surgery is not fully effective. 4. **Management of Symptoms and Complications:** - **Hypertension:** Antihypertensive medications. - **Hyperglycemia:** Management of blood sugar levels with diet, medications, or insulin. - **Osteoporosis:** Calcium and vitamin D supplements, bisphosphonates. 5. **Monitoring and Follow-Up:** - Regular follow-up visits to monitor cortisol levels and adjust treatment as necessary. - Long-term follow-up to manage any residual symptoms or complications. ### Conclusion Cushing syndrome is a complex condition caused by prolonged exposure to high levels of cortisol. Early diagnosis and appropriate management, including surgical intervention, medications, and management of associated symptoms, are crucial to prevent complications and improve the quality of life for patients. Regular monitoring and follow-up care are essential to ensure effective treatment and long-term outcomes.