A disorder of the connective tissue with unknown cause. It is chronic and marked by inflammation. The condition can involve the kidneys, serous surfaces, joints, and vessel walls. The targeted population is predominantly young women, but it also can occur in children.

Many theories suggest a role in genetic, hormonal, immune, and environmental factors, but the cause of systemic lupus erythematosus (SLE) is unknown.

SLE may begin quickly with fever portraying an acute infection, or it may develop very slowly over months or years with episodes of fever and discomfort. Initially, there may be headache, epilepsy, or psychoses. Any organ system may manifest symptoms. Examples of manifestations of systemic lupus erythematosus are as follows:

• Joint symptoms demonstrate intermittent pains to acute inflammation of many joints. These problems occur in 90% of patients, and may exist for years before other problems appear.

• Skin lesions include the characteristic butterfly pattern of cheek lesions, which are round, red, firm, and resemble skin discoloration patches with pimples. The lesions are apparent on the face, exposed areas of the neck, upper chest, and elbows.

• Bouts of recurrent pleurisy, with or without fluid collection, is common. Abnormalities of pulmonary function of a minor nature happen frequently.

• The pericardium is often inflamed.

• Generalized lymph node swelling occurs often, particularly in children, young adults, and Blacks that suffer with the condition. An enlarged spleen occurs in 10% of patients.

• Headaches, personality change, epilepsy, stroke, psychoses, and organic brain syndrome show central nervous system (CNS) involvement.

• Involvement of the kidney may be benign, without symptoms, or it may progress and be fatal. The most common manifestation is protein in the urine.

Tests for antinuclear antibody, anti-DNA, and lupus erythematosus cells are the most definitive tests for SLE in a majority of patients with the active disease. Pleurisy or lupus pneumonitis may be seen on chest x-ray. An electrocardiogram (EKG) may disclose abnormal conduction with cardiac involvement or inflammation of the pericardium. A biopsy of the kidney determines the stage of the disease and extent of kidney involvement.

Depending on the extent of disease and organ affected, treatment plans vary. Treatment includes: nonsteroidal anti-inflammatory drugs (NSAIDs); topical applications (corticosteroid creams); intralesional corticosteroids or antimalarials; cytotoxic drugs; antihypertensive drugs; and dietary changes.

The more severe the disease, the greater the risk of drug-induced complications that can further increase morbidity and mortality. Examples of the drug-induced complications include: infection from immunosuppression agents and coronary artery disease from chronic corticosteroid use. The course of SLE is chronic and relapsing, often with long periods (years) of remission. The prognosis has improved significantly in recent years. When the acute phase is controlled, prognosis for the long-term is usually good.

1. This disease shall be granted service connection although not otherwise established as incurred in or aggravated by service if manifested to a compensable degree within the applicable time limits under §3.307 following service in a period of war or following peacetime service on or after January 1, 1947, provided the rebuttable presumption provisions of §3.307 are also satisfied [38 CFR 3.309 [Disease subject to presumptive service connection], §3.309(a) [chronic disease].

2. May be entitled to special monthly compensation where the veteran has a single service-connected disability rated as 100% and/or other requirements/qualifications under 38 CFR §3.350 [Special monthly compensation ratings]. Also reference M21-1 V.ii.3.A. — Determining the Issues.

3. Not to be combined with ratings under Diagnostic Code 7809.

Evaluate this condition either by combining the evaluations for residuals under the appropriate system, or by evaluating Diagnostic Code 6350, whichever method results in a higher evaluation [38 CFR §4.88b Schedule of ratings-infectious diseases, immune disorders and nutritional deficiencies].