Systemic Lupus Systemic Lupus Erythematosus Erythematosus

DefinitionDefinition

Lupus is a condition of chronic Lupus is a condition of chronic inflammation caused by an inflammation caused by an autoimmune disease autoimmune disease

Lupus can affect many parts of the Lupus can affect many parts of the body, including the joints, skin, body, including the joints, skin, kidneys, heart, lungs, blood vessels, kidneys, heart, lungs, blood vessels, and brain. and brain.

DefinitionDefinition

When only the skin is involved, the When only the skin is involved, the condition is called condition is called discoiddiscoid lupus. lupus.

When internal organs are involved, When internal organs are involved, the condition is called the condition is called systemicsystemic lupus erythematosus (SLE).lupus erythematosus (SLE).

Other Types of LupusOther Types of Lupus

Subacute cutaneous lupus Subacute cutaneous lupus erythematosuserythematosus refers to skin lesions that refers to skin lesions that appear on parts of the body exposed to appear on parts of the body exposed to sun. The lesions do not cause scarring.sun. The lesions do not cause scarring.

Neonatal lupusNeonatal lupus is a rare disease that is a rare disease that can occur in newborn babies of women can occur in newborn babies of women with SLE or Sjögren's syndrome with SLE or Sjögren's syndrome

It is suspected that neonatal lupus is caused by It is suspected that neonatal lupus is caused by autoantibodies in the mother's blood called anti-Ro autoantibodies in the mother's blood called anti-Ro (SSA) and anti-La (SSB) (SSA) and anti-La (SSB)

Other Types of LupusOther Types of Lupus

Drug-induced lupusDrug-induced lupus

1.1. Hydralazine Hydralazine 2.2. QuinidineQuinidine3.3. ProcainamideProcainamide4.4. DilantinDilantin5.5. IsoniazideIsoniazide6.6. D-PenicillamineD-Penicillamine

Who gets it?Who gets it?

SLE are more common in women than SLE are more common in women than men (about eight times more common)men (about eight times more common)

Lupus is three times more common in Lupus is three times more common in African American women than in African American women than in Caucasian women Caucasian women

Can affect all ages but most commonly Can affect all ages but most commonly begins from age 20 to 45 yearsbegins from age 20 to 45 years

It is more frequent in African-It is more frequent in African-Americans, Native American, and AsiansAmericans, Native American, and Asians

Lupus can run in families Lupus can run in families

DescriptionDescription

At present, there is no cure for lupus At present, there is no cure for lupus However, lupus can be effectively However, lupus can be effectively

treated with drugs, and most people treated with drugs, and most people with the disease can lead active, with the disease can lead active, healthy lives healthy lives

Clinical ManifestationClinical Manifestation

Patients with SLE appear to be at Patients with SLE appear to be at high risk for coronary artery disease. high risk for coronary artery disease.

Infections, especially of the Infections, especially of the respiratory and urinary systems, respiratory and urinary systems, also are common in patients with the also are common in patients with the disease and are difficult to disease and are difficult to distinguish from flares of lupus distinguish from flares of lupus activity. activity.

MORBIDITY AND MORTALITYMORBIDITY AND MORTALITY

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

MORBIDITY AND MORTALITYMORBIDITY AND MORTALITY

Since the 1950s, the five-year Since the 1950s, the five-year survival rate for patients with survival rate for patients with systemic lupus erythematosus has systemic lupus erythematosus has increased from 50 percent to a increased from 50 percent to a range of 91 to 97 percent. range of 91 to 97 percent.

Higher mortality rates are Higher mortality rates are associated with seizures, lupus associated with seizures, lupus nephritis, and azotemia. nephritis, and azotemia.

Clinical FeaturesClinical Features

Malar RashMalar Rash

Discoid RashDiscoid 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?What causes lupus?

The precise reason for the abnormal The precise reason for the abnormal autoimmunity that causes lupus is not autoimmunity that causes lupus is not known known

Inherited genes, viruses, ultraviolet light, Inherited genes, viruses, ultraviolet light, and drugs may all play some role and drugs may all play some role

It also is known that some women with SLE It also is known that some women with SLE can experience worsening of their can experience worsening of their symptoms prior to their menstrual periods. symptoms prior to their menstrual periods. This phenomenon, together with the female This phenomenon, together with the female predominance of SLE, suggest that female predominance of SLE, suggest that female hormones play an important role in the hormones play an important role in the expression of SLE expression of SLE

DiagnosisDiagnosis

No single test establishes the diagnosis No single test establishes the diagnosis of systemic lupus of systemic lupus

It is based on clinical and laboratory It is based on clinical and laboratory criteria criteria

The most useful lab test is the The most useful lab test is the antinuclear antibody (ANA) antinuclear antibody (ANA)

The American College of Rheumatology The American College of Rheumatology (ACR) recommends ANA testing in (ACR) recommends ANA testing in patients who have two or more patients who have two or more unexplained signs or symptoms listed in unexplained signs or symptoms listed in the earlier slidethe earlier slide

DefinitionDefinition

Classification Criteria for SLE

ANAANA

Elevation of the antinuclear antibody Elevation of the antinuclear antibody (ANA) titer to 1:40 or higher is the most (ANA) titer to 1:40 or higher is the most sensitive of the ACR diagnostic criteria. sensitive of the ACR diagnostic criteria.

The ANA value has typical “peripheral” The ANA value has typical “peripheral” patternpattern

More than 99 percent of patients with More than 99 percent of patients with systemic lupus erythematosus have an systemic lupus erythematosus have an elevated ANA titer at some point although elevated ANA titer at some point although a significant proportion of patients may a significant proportion of patients may have a negative ANA titer early in the have a negative ANA titer early in the disease. disease.

DiagnosisDiagnosis

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

DiagnosisDiagnosis

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

DiagnosisDiagnosis

TreatmentTreatment

TreatmentTreatment

TreatmentTreatment

TreatmentTreatment

TreatmentTreatment

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

TreatmentTreatment

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

TreatmentTreatment

Antiphospholipid antibody syndromeAntiphospholipid antibody syndrome is is one of the most common causes of acquired one of the most common causes of acquired hypercoagulability in the general population hypercoagulability in the general population and is much more common in patients with and is much more common in patients with systemic lupus erythematosus systemic lupus erythematosus

Patients who have had venous or arterial Patients who have had venous or arterial thrombosis appear to benefit from thrombosis appear to benefit from maintenance therapy with high-intensity maintenance therapy with high-intensity (International Normalized Ratio of 3 to 4) (International Normalized Ratio of 3 to 4) warfarin (Coumadin). warfarin (Coumadin).

In SummaryIn Summary

Systemic lupus erythematosus (SLE) is an autoimmune Systemic lupus erythematosus (SLE) is an autoimmune disease. disease.

SLE is characterized by the production of unusual SLE is characterized by the production of unusual antibodies in the blood. antibodies in the blood.

SLE is more common in women than men. SLE is more common in women than men. The cause(s) of SLE is(are) unknown, however, heredity, The cause(s) of SLE is(are) unknown, however, heredity,

viruses, ultraviolet light, and drugs all may play some viruses, ultraviolet light, and drugs all may play some role. role.

Up to 10% of patients with SLE isolated to the skin will Up to 10% of patients with SLE isolated to the skin will develop the systemic form of lupus (SLE). develop the systemic form of lupus (SLE).

4 out of 11 criteria help in the diagnose SLE. 4 out of 11 criteria help in the diagnose SLE. Treatment of SLE is directed toward decreasing Treatment of SLE is directed toward decreasing

inflammation and/or the level of autoimmune activity. inflammation and/or the level of autoimmune activity. Patients with SLE can prevent "flares" of disease by Patients with SLE can prevent "flares" of disease by

avoiding sun exposure and not abruptly discontinuing avoiding sun exposure and not abruptly discontinuing medications. medications.

Questions:Questions:

1.1. What is the most sensitive test for What is the most sensitive test for SLE?SLE?

(a) anti-double-stranded DNA (a) anti-double-stranded DNA antibodyantibody

(b) anti-smith- antibody (b) anti-smith- antibody

(c) ANA(c) ANA

(d) anti-histone antibody(d) anti-histone antibody

Questions:Questions:

2. Which of the following is NOT a 2. Which of the following is NOT a cause of drug-induced lupus?cause of drug-induced lupus?

(a) Procainamide(a) Procainamide

(b) Hydralazine(b) Hydralazine

(c) INH(c) INH

(d) Theophylline(d) Theophylline

(e) Dilantin(e) Dilantin

Questions:Questions:

3. Which of the following is NOT 1 of 3. Which of the following is NOT 1 of the 11 diagnostic criteria for SLEthe 11 diagnostic criteria for SLE

(a) Malar rash(a) Malar rash

(b) Positive ANA(b) Positive ANA

(c) Serositis(c) Serositis

(d) Renal disease(d) Renal disease

(e) Fever(e) Fever