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September 20, 2004

Coding for Lupus
For The Record

Vol. 16 No. 19 Page 44

Lupus is a chronic inflammatory disease that can affect many parts of the body, including the skin, joints, kidneys, heart, lungs, blood vessels, and brain. It is an autoimmune disease, which means the body’s defenses attack its own tissues, causing inflammation and tissue damage.

Types of Lupus
• Systemic lupus erythematosus (SLE) affects many parts of the body. SLE is assigned to ICD-9-CM code 710.0 with an additional code for any associated manifestation, which will be discussed later. If the physician documents only “lupus,” it often refers to SLE and is assigned to code 710.0.

• Discoid lupus erythematosus (695.4) affects the skin. A red, raised rash may appear on the face, scalp, or elsewhere. This condition may later develop into SLE.

• Drug-induced lupus is caused by medications and causes symptoms similar to SLE that go away when the drug is discontinued. Common medications that cause drug-induced lupus include hydralazine, procainamide, methyldopa, quinidine, isoniazid, phenytoin (Dilantin), and carbamazepine (Tegretol). If the medication causing lupus was taken as prescribed, it is considered an adverse reaction and the manifestation (lupus) would be sequenced first followed by the E-code identifying the specific drug. Drug-induced lupus is assigned to code 695.4. If the medication was not properly administered, then it is considered a poisoning and the appropriate poisoning code identifying the medication is sequenced first followed by the manifestation (lupus – 695.4) and the appropriate poisoning E-code.

• Neonatal lupus affects some newborn babies of women with SLE or certain other immune system disorders. Although this condition is rare, it may cause serious heart defects, skin rash, liver abnormalities, or low blood counts.

Signs and Symptoms
Some of the common signs and symptoms of lupus include the following:

• Rash, which may include a butterfly-shaped rash (malar rash) across nose and cheeks or a scaly, disk-shaped rash (discoid rash) on face, neck, or chest

• Arthritis

• Persistent fatigue

• Fever over 100° F

• Sensitivity to sunlight

• Skin ulcers on tongue or inside nose or mouth

• Kidney problems

• Serositis — inflammation of the lining of some organs such as heart and lungs, which may cause painful breathing, shortness of breath, or chest pain

• Raynaud’s phenomenon — pale or purple fingers or toes from cold and stress

• Swelling in abdomen or ankles

• Unusual hair loss

• Unusual bleeding or bruising

• Dark urine

Diagnosis
Laboratory and diagnostic tests used to diagnose lupus include the following:

• Complete blood count — results may indicate anemia as well as low white blood cell counts

• Erythrocyte sedimentation rate — measures inflammation in the body; results faster than normal may indicate lupus

• Kidney and liver assessment

• Urinalysis — results may show increased protein level or blood cells in urine if the lupus has affected the kidneys

• Antinuclear antibody (ANA) test — a positive test for the presence of these antibodies may indicate lupus

• Chest x-ray to assess inflammation of the lungs

• ECG (electrocardiogram) to identify irregular rhythms, damage to the heart, or enlargement of heart

• Syphilis test — lupus antibodies in the blood may cause the test to be falsely positive; a positive test does not mean the patient has syphilis

• Complement levels measure the blood level of a group of proteins — results will be lower in patients with lupus

If the patient has four of the following 11 clinical and laboratory findings, it is a strong indication that the patient has lupus: malar rash, discoid rash, marked sensitivity to sunlight, ulcers, arthritis, serositis, renal disorder, neurological disorder, hematologic disorder, ANA, or immunologic disorder.

Complications
As stated earlier, lupus may affect many body parts. Remember that code 710.0 will be sequenced first. The code assignment shown below will be the second listed code.

• Kidney problems, which may be detected through urine and blood tests, may include nephritis/glomerulonephritis (580.81, 582.81, or 583.81 depending on acute, chronic, or unspecified nephritis) or nephritic syndrome (581.81).

• Central nervous system problems may include encephalitis (323.8), headache (784.0), dizziness (780.4), depression (311), and seizure (780.39).

• Blood and blood vessel problems may include anemia (280-285), leukopenia (288.0), and thrombocytopenia (287.4). If the patient has all three of these components, then assign code 284.8, Pancytopenia, instead of a code for each individual condition. (AHA Coding Clinic for ICD-9-CM, 1991, first quarter, page 14).

• Lung problems (517.8) may include pleurisy or a noninfectious form of pneumonia.

• Heart problems may include acute myocarditis (422.0), endocarditis (424.91), and acute pericarditis (420.0).

Treatment
Treatment of lupus depends on which organs are affected and the severity. The most common medications used include the following:

• NSAIDs (nonsteroidal anti-inflammatory drugs), which reduce fever, joint, and other tissue inflammation, include ibuprofen (Advil, Motrin) and naproxen sodium (Aleve, Naprosyn).

• Antimalarial drugs treat fatigue, joint pain, skin rashes, and inflammation of the surface of organs such as the heart and lung. Common drugs include hydrochloroquine (Plaquenil), chloroquine (Aralen), and quinacrine (Atabrine).

• Corticosteroids, which suppress inflammation, include prednisone, hydrocortisone, methylprednisolone, and dexamethasone.

• Immunosuppressive medications, which reduce normal immune response, include azathioprine (Imuran), cyclophosphamide (Cytoxan, Neosar), methotrexate (Rheumatrex, Folex), chlorambucil (Leukeran), cyclosporine (Neoral, Sandimmune), and mycophenolate.

Coding and sequencing for lupus are dependent upon 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, and Vicki Sippel, RHIA, of 3M Health Information Systems (800-367-2447), a leading supplier of coding and classification systems to nearly 4,000 healthcare providers. The company and its representatives do not assume any responsibility for reimbursement decisions or claims denials made by providers or payors as the result of the misuse of this coding information.

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