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Systemic Lupus
Erythematosus
Joko Anggoro
Bagian Ilmu Penyakit Dalam
FK Unram/RSUP NTB

WHAT IS LUPUS?

Lupus or SLE is inflammatory autoimmune disease that can affect
the skin, joints, kidneys, lungs, nervous system, and other organs
of the body.
Lupus is Latin for wolf,

and "erythro" is derived from ,
Greek for "red." All explanations originate with the reddish, butterfly-
shaped malar rash that the disease classically exhibits across the
nose and cheeks.
Systemic Lupus Erythematosus
(SLE)
Inflammatory autoimmune disease of
unknown etiology
Morbidity
Disease associated
Corticosteroid associated
Corticosteroid use as high as 89%
1-2

Mortality 5-10% at 10 years
Early - active disease and infections
Late - atherosclerosis
1. Zonana-Nacach et al., 2000 2. Urowitz et al., ACR meeting 2000 (Abstract)
Background
Lupus affects 10 times as
many women as men.
Treatment depends on the
symptoms and their severity.
Because it is a complex
disease, lupus requires
treatment by a rheumatologist
and the patients active
participation in working towards
good health.
Several kinds of lupus
1. Systemic lupus erythematosus (SLE). The word systemic
means the disease can affect many parts of the body.
2. Discoid lupus erythematosus is a chronic skin disorder in which a
red, raised rash appears on the face, scalp, or elsewhere.
3. Subacute cutaneous lupus erythematosus refers to skin lesions
that appear on parts of the body exposed to sun.
4. Drug-induced lupus is a form of lupus caused by medications.
5. Neonatal lupus is a rare disease that can occur in newborn babies
of women with SLE, Sjgrens syndrome, or no disease at all.

Aetiology
The cause is unknown but there are several predisposing
factors:
- Heredity
- Genetics
- Complement
- Sex hormone status
Premenopausal women are most
frequently affected.
- Immunological factors
Loss of self-tolerance has several
consequences
- Environmental tiggers
Immunological factors
B cell activation results in increased autoantibody
(mainly IgG) production to a variety (up to 2000) of
antigens (nuclear, cytoplasmic and plasma menbrane),
e.g. ANA, anti-dsDNA.
Development of and failure to remove immune
complexes from the circulation leads to deposition of
complexes in the tissue, causing vasculitis and disease
(e.g. glomerulonephritis).
There is impaired T cell regulation of the immune
response.
There is abnormal cytokine production (IL-1 and IL-2),
although its exact role in the pathogenesis is unknown. IL-
6 and IL-10 levels are often reised.
TNF- promoters have also been linked to SLE.
Environmental triggers
Drugs such as hydralazine, methyldopa,
isoniazid, D-penicillamine and minocycline
can induce lupus not associated with anti-
dsDNA. Flare-ups can be induced by the
contraceptive pill and hormone
replacement therapy (HRT).
Ultraviolet light is another well-recognized
trigger.
Model of
SLE
pathogene
sis
Common Symptoms of
Lupus
Painful or swollen joints and muscle pain
Unexplained fever
Red rashes, most commonly on the face
Chest pain upon deep breathing
Unusual loss of hair
Pale or purple fingers or toes from cold or stress (Raynauds
phenomenon)
Sensitivity to the sun
Swelling (edema) in legs or around eyes
Mouth Ulcers
Swollen glands
Extreme fatigue

The 1997 Update of the 1982 American College of Rheumatology Revised Criteria for
Classification of Systemic Lupus Erythematosus
Criterion Definition
1. Malar Rash Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds
2. Discoid rash Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions
3. Photosensitivity Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation
4. Oral ulcers Oral or nasopharyngeal ulceration, usually painless, observed by physician
5. Nonerosive Arthritis Involving 2 or more peripheral joints, characterized by tenderness, swelling, or effusion
6. Pleuritis or Pericarditis a) Pleuritis--convincing history of pleuritic pain or rubbing heard by a physician or evidence of pleural effusion
OR
b) Pericarditis--documented by electrocardigram or rub or evidence of pericardial effusion
7. Renal Disorder a) Persistent proteinuria > 0.5 grams per day or > than 3+ if quantitation not performed
OR
b) Cellular casts--may be red cell, hemoglobin, granular, tubular, or mixed
8. Neurologic Disorder a) Seizures--in the absence of offending drugs or known metabolic derangements; e.g., uremia, ketoacidosis, or electrolyte
imbalance
OR
b) Psychosis--in the absence of offending drugs or known metabolic derangements, e.g., uremia, ketoacidosis, or electrolyte
imbalance
9. Hematologic Disorder a) Hemolytic anemia--with reticulocytosis
OR
b) Leukopenia--< 4,000/mm
3
on 2 occasions
OR
c) Lymphopenia--< 1,500/ mm
3
on 2 occasions
OR
d) Thrombocytopenia--<100,000/ mm
3
in the absence of offending drugs
10. Immunologic Disorder a) Anti-DNA: antibody to native DNA in abnormal titer
OR
b) Anti-Sm: presence of antibody to Sm nuclear antigen
OR
c) Positive finding of antiphospholipid antibodies on:
1. an abnormal serum level of IgG or IgM anticardiolipin antibodies,
2. a positive test result for lupus anticoagulant using a standard method, or
3 a false-positive test result for at least 6 months confirmed by Treponema pallidum immobilization or fluorescent treponemal
antibody absorption test
11. Positive Antinuclear Antibody An abnormal titer of antinuclear antibody by immunofluorescence or an equivalent assay at any point in time and in the absence of
drugs
2009 American College of Rheumatology
Investigations
CBC
Antinuclear antibody (ANA) (90%)sensitive
Anti-double strand (ds) DNAspecific
Antiphospholipid antibodies
Anti-Smith antibodies
Rheumatoid factor (25%)
Serum complement levels are reduced during active disease
Anticardiolipin antibodies (35-45%)
Serological tests for syphilis (30%)
Immunoglobulins are raised (usually IgG and IgM)
Creatinine and BUN
Management
The disease and its management should
be discussed, pointing out that the
prognosis is much improved though
patients are advised to avoid excessive
exposure to sunlight and should reduce
cardiovascular risk factors.

Summary Key treatment
NSAIDs
Antimalaria: chloroquine or
hydroxychloroquine: IL- 1, IL-6, IL-18
and TNF- serum levels after 3 months
Corticosteroids: prednisone
Immunosuppressive drugs: methotrexate
(MTX), azathioprine, cyclophosphamide,
mycophenolate mofetil.
Anti CD-20 antibody (Rituximab)
Lupus May 2006 vol. 15 no. 5 268-275
Course and prognosis
An episodic course is characteristic, with exacerbations
and complete remissions that may last for long periods.
A chronic course is occasionally seen. Earlier estimates
of the mortality in SLE were exaggerated; 10-year
survival rate is about 90%.
Cause of death : vasculitis, infection, renal failure
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