November 12, 2007

Coding for Systemic Lupus Erythematosus
For The Record
Vol. 19 No. 23 P. 45

Lupus is an autoimmune and chronic inflammatory disease that can affect many parts of the body, including the skin, joints, kidneys, heart, lungs, blood vessels, and brain.

Types of Lupus
Systemic lupus erythematosus (SLE) is assigned to ICD-9-CM code 710.0 with an additional code for any associated manifestation (discussed later). If the physician only documents lupus, this often refers to SLE. Discoid lupus erythematosus (695.4) may cause a red, raised rash to appear on the face, scalp, or elsewhere and may later develop into SLE.

Drug-induced lupus (695.4) occurs from a reaction to medications and causes symptoms similar to SLE that disappear when the drug is discontinued. Common medications causing this 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. If the medication was not properly administered, it is considered a poisoning, and the appropriate poisoning code identifying the medication is sequenced first followed by the manifestation and the appropriate poisoning E-code.

Neonatal lupus affects some newborn babies of women with SLE or other certain 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
These include rash (such as a butterfly-shaped rash [malar rash] across the nose and cheeks or a scaly, disk-shaped rash [discoid rash] on the face, neck, or chest); arthritis; persistent fatigue; fever higher than 100°; sunlight sensitivity; skin ulcers on the tongue or inside the nose or mouth; kidney problems; serositis (inflammation of the lining of some organs 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; and dark urine.

Diagnosis
Laboratory and diagnostic tests for lupus include a complete blood count since results may indicate anemia or low white blood cell counts; erythrocyte sedimentation rate to measure inflammation with faster-than-normal results possibly indicating lupus; kidney and liver assessment; urinalysis to show an increased protein level or blood cells in urine if the lupus has affected the kidneys; antinuclear antibody because a positive test may indicate lupus; chest x-ray to assess lung inflammation; electrocardiogram to identify irregular heart rhythms, damage, or enlargement; syphilis test because lupus antibodies in the blood may cause it to be falsely positive, though a positive test does not mean the patient has syphilis; and complement levels to measure the blood level of a protein group with lower results in patients with lupus.

If the patient has four of the following 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, antinuclear antibody, and immunologic disorder.

Complications
Code 710.0 will be sequenced first for lupus with the following being the second listed code:

• Kidney problems 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 (288.50), and thrombocytopenia (287.4). If the patient has all three, assign code 284.1, 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 depends on which organs are affected and the severity. The most common medications used include nonsteroidal anti-inflammatory drugs to reduce fever, joint, and other tissue inflammation; antimalarial drugs to treat fatigue, joint pain, skin rashes, and inflammation of organ surfaces; corticosteroids to suppress inflammation; and immunosuppressive medications to reduce normal immune response.

Coding and sequencing for SLE 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.