Download Systemic Lupus Erythematosus

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.
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)
Other Types of Lupus

Drug-induced lupus
1.
Hydralazine
Quinidine
Procainamide
Dilantin
Isoniazide
D-Penicillamine
2.
3.
4.
5.
6.
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
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
Diagnosis




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
ANA



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 antiSm (anti-Smith) antibody test, anti-SSA and antiSSB is done. Both the anti-ds-DNA and antiSM 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 “ANAnegative” 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 highintensity (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.
Questions:
1.
What is the most sensitive test for SLE?
(a) anti-double-stranded DNA antibody
(b) anti-smith- antibody
(c) ANA
(d) anti-histone antibody
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