Systemic Lupus

Erythematosus
• Systemic lupus erythematosus (SLE) is a
chronic, inflammatory autoimmune
disorder.
• It may affect the skin, joints, kidneys, and
other organs.
 Causes, incidence, and risk
factors
 
SLE (lupus) is an autoimmune disease. This means
there is a problem with the body's normal immune
system response. Normally, the immune system helps
protect the body from harmful substances. But in
patients with an autoimmune disease, the immune
system can't tell the difference between harmful
substances and healthy ones. The result is an
overactive immune response that attacks otherwise
healthy cells and tissue. This leads to chronic (long-
term) inflammation.
• The underlying cause of autoimmune
diseases is not fully known. Some
researchers think autoimmune diseases
occur after infection with an organism that
looks like certain proteins in the body. The
proteins are later mistaken for the
organism and wrongly targeted for attack
by the body's immune system.
 Classification Criteria for Systemic Lupus
Erythematosus
• * Before a patient can be classified with systemic lupus
erythematosus, at least four of the following 11 disorders must be
present:

• Malar rash
Discoid rash
Photosensitivity
Oral ulcers
Arthritis
Serositis

• Renal disorder
Neurologic disorder
Hematologic disorder
Immunologic disorder
Antinuclear antibodies
 Symptoms 
• Symptoms vary from person to person,
and may come and go. The condition may
affect one organ or body system at first.
Others may become involved later. Almost
all people with SLE have joint pain and
most develop arthritis. Frequently affected
joints are the fingers, hands, wrists, and
knees.
 Symptoms
• Inflammation of various parts of the heart
may occur as pericarditis, endocarditis, or
myocarditis. Chest pain and arrhythmias
may result from these conditions.
 General symptoms include:
• Fever
• Fatigue
• General discomfort, uneasiness or ill feeling (malaise)
• Skin rash - a "butterfly" rash over the cheeks and bridge of the
nose affects about half of those with SLE. The rash gets worse
when in sunlight. The rash may also be widespread.
• Sensitivity to sunlight
• Joint pain and swelling
• Arthritis
• Swollen glands
• Muscle aches
• Nausea and vomiting
• Pleurisy (causes chest pain)
• Pleural effusions
• Seizures
• Psychosis
Внесуставные поражения
при суставном синдроме

Фотосенсибилизационная
сыпь при системной красной
волчанке
Внесуставные поражения
при суставном синдроме

Аллопеция у
пациента с
системной красной
волчанкой
Severe deformities and contractures of the finger
joints in systemic lupus erythematosus.
 Additional symptoms that may be
associated with this disease:
• Blood in the urine
• Coughing up blood
• Nosebleed
• Swallowing difficulty
• Skin color is patchy
• Red spots on skin
• Fingers that change color upon pressure or in the cold
• Numbness and tingling
• Mouth sores
• Hair loss
• Abdominal pain
• Visual disturbance
• Blood disorders, including blood clots
 Tests used to diagnose SLE may
include:
• Antinuclear antibody (ANA) panel
including anti-DNA and anti-Smith
antibodies
• Chest x-ray showing pleuritis or
pericarditis
• Urinalysis to show blood, casts, or
protein in the urine
• CBC
• Kidney biopsy
 This disease may also alter the
results of the following tests
• WBC count
• Serum globulin electrophoresis
• Rheumatoid factor
• Urine protein
• Serum protein electrophoresis
• Mononucleosis spot test
• ESR
• Cryoglobulins
• Coombs' test - direct
• Complement component 3 (C3)
• Complement
• Anti-thyroid microsomal antibody
• Anti-thyroglobulin antibody
• Anti-mitochondrial antibody
• Anti-smooth muscle antibody
Treatment of Polyarthritis in Patients with Systemic
Lupus Erythematosus
Drug Comment

NSAIDs Avoid gastrotoxic NSAIDs; counter with

cytoprotective therapy

Antimalarials Hydroxychloroquine (Plaquenil) is the

antimalarial drug used most often in the
United States; ophthalmologic
monitoring is recommended every
six to 12 months
Treatment of Polyarthritis in Patients with Systemic
Lupus Erythematosus
Drug Comment

Glucocorticoids Individual joints may benefit from intra-articular

injection of triamcinolone (Aristospan); severe

polyarthritis flare-ups may be treated with
intravenous "pulse therapy" consisting of 1,000 mg
of methylprednisolone (Solumedrol) daily for three
days; use of prednisone for maintenance therapy
should be limited to 10 mg or less daily
Treatment of Polyarthritis in Patients with Systemic
Lupus Erythematosus

Drug Comment
Immunosuppressive
drugs Methotrexate
(Rheumatrex) or
azathioprine (Imuran) can be

used as steroid-sparing
drugs; methotrexate cannot
be used during pregnancy
Musculoskeletal Complications of Systemic Lupus
Erythematosus
• Osteonecrosis (avascular necrosis of bone)
– Occurs in 14 percent of patients with systemic lupus
erythematosus
– Most commonly affects the hip joints
– Early detection requires MRI
– Core decompression of bone is an effective treatment in early
stages of the disease
• Osteoporosis
– Occurs in 64 percent of patients with systemic lupus
erythematosus
– Osteoporosis of the lumbar spine is associated with the highest
dosage of prednisone and the cumulative effects of prednisone
– Calcium, vitamin D, calcitonin and bisphosphonates are effective
treatments (even in premenopausal women with osteoporosis)
 Management of Cutaneous Lupus
Erythematosus
Treatment agent Comment

Sun block Blocks both UVA and UVB radiation

Antimalarials Use of combination antimalarial therapy

(hydroxychloroquine [Plaquenil] and
quinacrine) or chloroquine (Aralen),
which has more risk of retinopathy, is
sometimes necessary
 Management of Cutaneous Lupus
Erythematosus
Treatment agent Comment

Dapsone G6PD status should be checked

Retinoids Avoid using in pregnant women

Corticosteroids Use of corticosteroids may be necessary

as part of initial therapy for severe discoid
lupus or for lupus vasculitis; intradermal
corticosteroids are helpful for individual
discoid lesions, especially in the scalp
 Management of Cutaneous Lupus
Erythematosus
Treatment agent Comment

Immunosuppressive drugs Methotrexate (Rheumatrex)

or azathioprine (Imuran) is
used as steroid-sparing drug
Thalidomide
(Synovir) One of the most effective
drugs for treatment of discoid
lupus, but teratogenicity and
neuropathy will limit its
acceptance and
 Characteristics of Lupus Nephritis
• Occurs in approximately 50 percent of patients
with systemic lupus erythematosus
• More common in black people
• Renal biopsy can be helpful in identifying the
activity of lupus nephritis and the degree of
chronicity (scarring)
• Cyclophosphamide (Cytoxan) is more effective
than corticosteroids alone for the treatment of
severe forms of lupus nephritis (diffuse
proliferative glomerulonephritis)
 Neurologic Manifestations of
Systemic Lupus Erythematosus
• Psychosis
Seizures
Organic brain syndrome
Stroke
Cognitive function abnormalities
Transverse myelopathy
Mononeuritis multiplex
 Characteristics of Premature
Atherosclerosis
• Present in 6 to 10 percent of patients with
systemic lupus erythematosus
• Associated with duration of disease
• Corticosteroid treatment increases the
levels of cardiovascular risk factors,
including weight, blood pressure,
cholesterol and homocysteine levels
Characteristics of Antiphospholipid
Antibody Syndrome
• 50 percent of patients with systemic lupus
erythematosus manufacture antiphospholipid antibodies
but often do so intermittently and at low titer
• The two clinically important antiphospholipid antibodies
are lupus anticoagulant and anticardiolipin antibody
• Presentations of antiphospholipid antibody syndrome
include thrombosis, recurrent or late pregnancy loss, and
thrombocytopenia
• Long-term management of patients who have had a
thrombotic event resulting from antiphospholipid antibody
syndrome includes high-intensity warfarin (Coumadin)
therapy (INR of 3 to 4)