Transcript Systemic Lupus Erythematosus

Lupus Erythematosus
Dr. Mohamed nasr
Types of Lupus:
 Cutaneous (skin) lupus
 primarily affects the skin but may involve the hair and mucous
membranes
 also commonly called discoid lupus
 Systemic lupus erythematosus (SLE)
 affects any system in the body
 Drug-induced lupus erythematosus (DILE)
 Side effect of long term use of certain medications
 Symptoms overlap with those of SLE
 Neonatal lupus
 very rare
 acquired from the passage of maternal autoantibodies
Cutaneous Lupus
 ACUTE: Typical photosensitive malar rash
when acute
Highly associated with systemic LE (almost 100%)
 SUBACUTE: This variant is psoriasiform or
annular
~50% of these patients will meet criueria for SLE
 CHRONIC: ie Discoid Lupus
Most patients (85-90% never develop systemic lupus)
Systemic Lupus Erythematosus
 Chronic autoimmune
disease
 Most common form
of lupus
 Autoantibodies
produced by own
immune system
recognize own DNA as
foreign
 Lupus “wolf”
Understanding the causes:
 Unknown
 Possible Factors:
 genetics
 environmental
 hormonal
 May explain why lupus
occurs more frequently
in females than in
males
 NOT infectious
Screening and Diagnosis:
 Difficult
 Usually takes months to even years
 Laboratory tests:
 Antinuclear antibody (ANA) test that detects the presence
of autoantibodies that attack your own cells
 blood tests for anemia, low white-cell count,
abnormalities in organ function
 urinalysis
 electrocardiogram or echocardiogram to check the heart
 chest x-ray
Eleven Criteria Used for the Diagnosis
of Lupus:
 Malar Rash
 Rash over cheeks
 Discoid Rash
 Red raised patches
 Photosensitivity
 Reaction to sunlight
 Oral Ulcers
 Ulcers in nose or mouth
 Arthritis
 Two or more joints
 Serositis
 Pleuritis or pericarditis
Eleven Criteria cont…
 Renal Disorder
Excessive protein in the urine or cast.
 Neurologic Disorder
Seizures
 Hematologic Disorder
Hemolytic anemia or leukopenia
 Immunologic Disorder
Positive anti-double stranded anti-DNA test
 Antinuclear Antibody
Positive test
Why organs are attacked:
 Due to autoantibodies
Also referred to as anti-nuclear antibodies
Antibodies produced by the immune system
Attack the RNA and DNA in the nucleus of own cells
Systems Affected
Musculoskeletal system
-- avascular necrosis
-- muscle inflammation
Kidney system
Nervous system
-- seizures
-- nerve paralysis
-- severe depression
-- psychosis
-- strokes
Blood and Lymph system
-- anemia
-- thrombocytopenia
Systems Affected
Stomach, Intestines, Liver, and Associated Organs
-- ulcers
-- abdominal pains
Skin and Hair
-- rash and alopecia
Heart and Blood Vessels
-- pericarditis
-- arthrosclerosis
-- spasms of the artery
Lungs
-- pleurisy, pneumonia, and pleural effusion
Eyes
-- rarely involved except for retina
The simplest of treatments include:
1- Anti-inflammatory drugs like Aspirin.
2- Anti- malarial drugs.
3- Immunosuppressive medications.
4- Corticosteroids.
Subacute Cutaneous Lupus
Erythematosus
 Widespread, non-scarring but often
photosensitive rash.
 Annular or papulosquamous morphology.
 Mild systemic disease common but renal
involvement rare.
 Positive ANA in most patients, but anti-nDNA
uncommon.
 Anti-Ro in two thirds patients.
Subacute Cutaneous Lupus
Discoid Lupus Erythematous (DLE)
Most scarring and chronic form of
cutaneous lupus.
Discoid shaped plaques with white scale,
with time, lesions become atrophic.
Can lead to scarring alopecia.
Few patients meet criteria for SLE (6%).
The characteristics of DLE lesions:
1. Persistent localized erythema.
2. Adherent scales related to the dilated
follicles.
3. Follicular plugging.
4. Redness & telangiectasia of the border.
5. Atrophy & scarring of the center.
Follicular Plugging
Diagnosis = lupus band test
Presence of IgG & C in linear pattern at
dermo-epidermal junction below lamina
densa in involved sun-exposed skin
only.
Treatment:
Treat with intralesional or topical steroids,
sun avoidance & antimalarial if severe or
large areas involved.