December 8, 2008

Coding for Cushing’s Syndrome
For The Record
Vol. 20 No. 25 P. 27

Cushing’s syndrome (ICD-9-CM code 255.0) is a condition caused by long-time exposure to too much cortisol, a hormone that regulates the body’s blood pressure and metabolism. Also documented as hypercortisolism, it can be caused by the use of oral corticosteroid medication or when the body makes too much cortisol.

Exogenous Cushing’s syndrome develops from a cause outside of the body—in other words, from the patient taking corticosteroids over an extended period of time. Prednisone, dexamethasone (Decadron), and methylprednisolone (Medrol) are medications that can lead to Cushing’s syndrome.

Endogenous Cushing’s syndrome is due to the body’s own cortisol overproduction, such as from excess production by one or both adrenal glands or overproduction of the adrenocorticotropic hormone (ACTH) that regulates cortisol production. Endogenous Cushing’s syndrome may be caused by a benign pituitary gland tumor (pituitary adenoma), also called Cushing’s disease; ectopic ACTH, a secreting tumor which may be located in the lung or pancreas; or primary adrenal gland disease (adrenal adenoma), which may be benign or cancerous tumors.

Code 255.0 includes the following terms:

• Adrenal hyperplasia due to excess ACTH;

• Cushing’s syndrome: NOS, iatrogenic, idiopathic, pituitary-dependent;

• Ectopic ACTH syndrome;

• Iatrogenic syndrome of excess cortisol; and

• Overproduction of cortisol.

Coding directives state to use an additional E code to identify cause if it is drug induced.

Signs and Symptoms
The characteristic signs of Cushing’s syndrome include the following:

• a fatty hump between the shoulders (buffalo hump);

• a rounded face (moon face); and

• pink or purple stretch marks (striae) on the skin of the abdomen, thighs, breasts, and arms.

Other common signs and symptoms are as follows:

• weight gain (especially around the waist);

• fatigue;

• muscle weakness;

• facial flushing;

• thin and fragile skin that bruises easily;

• slow healing of cuts and infections;

• depression, anxiety and irritability;

• loss of emotional control;

• thicker or more visible body and facial hair;

• acne;

• irregular or absent menstrual periods in women;

• erectile dysfunction in men; and

• headache.
Patients with Cushing’s syndrome may develop complications, including hypertension (401.9), osteoporosis (733.00), secondary diabetes (249.00), kidney stones (592.0), frequent or unusual infections, and a loss of muscle mass or strength.

Diagnosis
Cushing’s syndrome is diagnosed by a physician with a thorough physical exam and the following diagnostic tests:

• urine and blood tests that evaluate the hormone levels to show whether the body is producing excessive cortisol;

• saliva tests, as high levels of cortisol from a late-night sample of saliva may be indicative of Cushing’s syndrome; and

• CT and MRI may detect tumors.

Cushing’s syndrome is difficult to diagnose because the signs and symptoms may be similar to other conditions.

Treatment
The treatment goal is to lower the high level of cortisol in the body, which may be accomplished by reducing corticosteroid use. The medication may be lowered or slowly stopped, but a patient should not stop this medication abruptly.

The patient may undergo surgery if the cause of the Cushing’s syndrome is a tumor. The surgery may be followed up with radiation therapy.

Finally, the following medications may be administered to control the excessive production of cortisol: ketoconazole (Nizoral), mitotane (Lysodren), or metyrapone (metopirone).

Coding and sequencing for Cushing’s syndrome are dependent on the physician documentation in the medical record and application of the Official Coding Guidelines for inpatient care. Also, use specific AHA Coding Clinic for ICD-9-CM and American Medical Association CPT Assistant references to ensure complete and accurate coding.

— This information was prepared by Audrey Howard, RHIA, of 3M Consulting Services. 3M Consulting Services is a business of 3M Health Information Systems, a supplier of coding and classification systems to nearly 5,000 healthcare providers. The company and its representatives do not assume any responsibility for reimbursement decisions or claims denials made by providers or payers as the result of the misuse of this coding information. More information about 3M Health Information Systems is available at www.3mhis.com or by calling 800-367-2447.