Systemic Lupus Erythematosus

Definition Lupus is a condition of chronic inflammation caused by an autoimmune disease Lupus can affect many parts of the body, including the joints, skin, kidneys, heart, lungs, blood vessels, and brain.

When only the skin is involved, the condition is called discoid lupus. Definition When only the skin is involved, the condition is called discoid lupus. When internal organs are involved, the condition is called systemic lupus erythematosus (SLE).

Other Types of Lupus Subacute cutaneous lupus erythematosus refers to skin lesions that appear on parts of the body exposed to sun. The lesions do not cause scarring. Neonatal lupus is a rare disease that can occur in newborn babies of women with SLE or Sjögren's syndrome It is suspected that neonatal lupus is caused by autoantibodies in the mother's blood called anti-Ro (SSA) and anti-La (SSB)

Drug-induced lupus Hydralazine Quinidine Procainamide Dilantin Other Types of Lupus Drug-induced lupus Hydralazine Quinidine Procainamide Dilantin Isoniazide D-Penicillamine

SLE are more common in women than men (about eight times more common) Who gets it? SLE are more common in women than men (about eight times more common) Lupus is three times more common in African American women than in Caucasian women Can affect all ages but most commonly begins from age 20 to 45 years It is more frequent in African-Americans, Native American, and Asians Lupus can run in families

At present, there is no cure for lupus Description At present, there is no cure for lupus However, lupus can be effectively treated with drugs, and most people with the disease can lead active, healthy lives

Clinical Manifestation Patients with SLE appear to be at high risk for coronary artery disease. Infections, especially of the respiratory and urinary systems, also are common in patients with the disease and are difficult to distinguish from flares of lupus activity.

MORBIDITY AND MORTALITY In one cohort study it is found that within seven years of diagnosis, 61 percent of patients developed clinically detectable organ damage, with neuropsychiatric (20.5 percent), musculoskeletal (18.5 percent), and renal (15.5 percent) organ systems most commonly affected.

MORBIDITY AND MORTALITY Since the 1950s, the five-year survival rate for patients with systemic lupus erythematosus has increased from 50 percent to a range of 91 to 97 percent. Higher mortality rates are associated with seizures, lupus nephritis, and azotemia.

Clinical Features

Malar Rash

Discoid Rash Discoid lesions are thick, scarring, usually raised or flat, red, with well-defined borders, which appear on sun-exposed surfaces, but in rare cases can be found on non-sun-exposed areas. They generally do not itch.

What causes lupus? The precise reason for the abnormal autoimmunity that causes lupus is not known Inherited genes, viruses, ultraviolet light, and drugs may all play some role It also is known that some women with SLE can experience worsening of their symptoms prior to their menstrual periods. This phenomenon, together with the female predominance of SLE, suggest that female hormones play an important role in the expression of SLE

No single test establishes the diagnosis of systemic lupus It is based on clinical and laboratory criteria The most useful lab test is the antinuclear antibody (ANA) The American College of Rheumatology (ACR) recommends ANA testing in patients who have two or more unexplained signs or symptoms listed in the earlier slide

Classification Criteria for SLE Definition

The ANA value has typical “peripheral” pattern Elevation of the antinuclear antibody (ANA) titer to 1:40 or higher is the most sensitive of the ACR diagnostic criteria. The ANA value has typical “peripheral” pattern More than 99 percent of patients with systemic lupus erythematosus have an elevated ANA titer at some point although a significant proportion of patients may have a negative ANA titer early in the disease.

Diagnosis When the ANA is positive, an ANA profile including an anti-double-strand DNA, an anti-Sm (anti-Smith) antibody test, anti-SSA and anti-SSB is done. Both the anti-ds-DNA and anti-SM antibody test are 100% specific for SLE (ie, they are only positive in patients in patients with SLE).

Diagnosis So in working up a case of possible SLE, first do an ANA. If the ANA is positive, do an ANA profile. If the ANA is negative and clinical signs strongly suggest SLE, then check for anti-SSA/Ro antibodies, If this test is positve, the patient probably has “ANA-negative” SLE (rare)

Diagnosis

Treatment

Treatment

Treatment

Treatment

Treatment Patients with central nervous system manifestations of lupus erythematosus who present with status epilepticus, organic brain syndrome or coma can be treated with intravenous methylprednisolone pulse therapy. Patients with severe or resistant symptoms may also require treatment with intravenous cyclophosphamide and/or plasmapheresis. However, it is usually necessary to rule out other conditions that may mimic central nervous system manifestations of systemic lupus erythematosus, including infection and toxic metabolic states.

Treatment Patients with systemic lupus erythematosus have higher levels of homocysteine, a known risk factor for atherosclerosis. Intervention, in the form of both lifestyle modifications and pharmacologic therapy (folate), may be appropriate in patients with SLE.

Treatment Antiphospholipid antibody syndrome is one of the most common causes of acquired hypercoagulability in the general population and is much more common in patients with systemic lupus erythematosus Patients who have had venous or arterial thrombosis appear to benefit from maintenance therapy with high-intensity (International Normalized Ratio of 3 to 4) warfarin (Coumadin).

In Summary Systemic lupus erythematosus (SLE) is an autoimmune disease. SLE is characterized by the production of unusual antibodies in the blood. SLE is more common in women than men. The cause(s) of SLE is(are) unknown, however, heredity, viruses, ultraviolet light, and drugs all may play some role. Up to 10% of patients with SLE isolated to the skin will develop the systemic form of lupus (SLE). 4 out of 11 criteria help in the diagnose SLE. Treatment of SLE is directed toward decreasing inflammation and/or the level of autoimmune activity. Patients with SLE can prevent "flares" of disease by avoiding sun exposure and not abruptly discontinuing medications.

What is the most sensitive test for SLE? Questions: What is the most sensitive test for SLE? (a) anti-double-stranded DNA antibody (b) anti-smith- antibody (c) ANA (d) anti-histone antibody

2. Which of the following is NOT a cause of drug-induced lupus? Questions: 2. Which of the following is NOT a cause of drug-induced lupus? (a) Procainamide (b) Hydralazine (c) INH (d) Theophylline (e) Dilantin

Questions: 3. Which of the following is NOT 1 of the 11 diagnostic criteria for SLE (a) Malar rash (b) Positive ANA (c) Serositis (d) Renal disease (e) Fever