SLE

Definition
Systemic lupus
erythematosus (SLE)
is an autoimmune
disorder
characterized by
antibodies to nuclear
and cytoplasmic
antigens, multisystem
inflammation, protea
n clinical
manifestations, and a
relapsing and
remitting course.
 Epidemiology
• Worldwide, the prevalence of SLE varies.
• More than 90% of cases of SLE occur in
women, frequently starting at childbearing
age.
• The risk of SLE development in men is similar
to that in prepubertal or postmenopausal
women.
• The prevalence of SLE is highest in women
aged 14 to 64 years. SLE does not have an age
predilection in males
 Etiology
• Although the specific cause of SLE is unknown, multiple genetic
predispositions and gene-environment interactions have been identified
Environmental and exposure-related causes of SLE are less clear.
Possible early-life risk factors include the following :
• Low birthweight (< 2,500 g)
• Preterm birth (≥1 month early)
• Childhood exposure to agricultural pesticides
Other potential factors include the following:
• Silica dust and cigarette smoking may increase the risk of developing SLE
• Estrogen use in postmenopausal women appears to increase the risk of
developing SLE.
• Photosensitivity is clearly a precipitant of skin disease
• Ultraviolet light stimulates keratinocytes, which leads not only to
overexpression of nuclear ribonucleoproteins (snRNPs) on their cell surfaces
but also to the secretion of cytokines that simulate increased autoantibody
production. [39]
• Breastfeeding is associated with a decreased risk of developing SLE
 Patophysiology
• these T cells secrete less interleukin (IL)-2, and
one defect in signaling seems to be linked to
an increase in calcium influx, possibly due to
changes in the CD3 signaling subunits. The
following seem to be adversely affected in T
cells from patients with SLE: effector activity
such as CD8 cytotoxicity; T-regulatory, B-cell
help; migration; and adhesion.
 Clinical Manifestation
• Malar rash
• Ulcers/mucocutaneous involvement
• Renal involvement, proteinuria, urinary cellular
casts
• Seizures
• Thrombocytopenia
• Hemolytic anemia
• Fever
• Lymphadenopathy.
 Clinical Manifestation
Patients may present with any of the following manifestations:
• Constitutional (eg, fatigue, fever, arthralgia, weight changes)
• Musculoskeletal (eg, arthralgia, arthropathy, myalgia, frank arthritis,
avascular necrosis)
• Dermatologic (eg, malar rash, photosensitivity, discoid lupus)
• Renal (eg, acute or chronic renal failure, acute nephritic disease)
• Neuropsychiatric (eg, seizure, psychosis)
• Pulmonary (eg, pleurisy, pleural effusion, pneumonitis, pulmonary
hypertension, interstitial lung disease)
• Gastrointestinal (eg, nausea, dyspepsia, abdominal pain)
• Cardiac (eg, pericarditis, myocarditis)
• Hematologic (eg, cytopenias such as leukopenia, lymphopenia,
anemia, or thrombocytopenia)
 Diagnosis
• The diagnosis of SLE is based on a
combination of clinical findings and laboratory
evidence.
• The ACR/EULAR classification requires an
antinuclear antibody (ANA) titer of at least
1:80 on HEp-2 cells or an equivalent positive
test at least once.
 Diagnosis
(Laboratory)
Laboratory studies used in the diagnosis of SLE are as
follows:
• CBC with differential
• Serum creatinine
• Urinalysis with microscopy
• ESR or CRP level
• Complement levels
• Liver function tests
• Creatine kinase assay
• Spot protein/spot creatinine ratio
• Autoantibody tests
 Diagnosis
• Joint radiography
• Chest radiography and chest CT scanning
• Echocardiography
• Brain MRI/MRA
• Cardiac MR
• Arthrocentesis
• Lumbar puncture
• Renal biopsy
 Diagnosis
• Joint radiography
• Chest radiography and chest CT scanning
• Echocardiography
• Brain MRI/MRA
• Cardiac MR
• Arthrocentesis
• Lumbar puncture
• Renal biopsy
 Management
Management of SLE often depends on the
individual patient’s disease severity and
disease manifestations, although
hydroxychloroquine has a central role for long-
term treatment in all SLE patients.
 Management
Medications used to treat SLE manifestations include the
following:
• Antimalarials (eg, hydroxychloroquine)
• Corticosteroids (eg, methylprednisolone, prednisone),
short-term use recommended
• Nonbiologic DMARDS: Cyclophosphamide, methotrexate,
azathioprine, mycophenolate, cyclosporine
• Nonsteroidal anti-inflammatory drugs (NSAIDS; eg,
ibuprofen, naproxen, diclofenac)
• Biologic DMARDs (disease-modifying antirheumatic drugs):
Belimumab, rituximab, and/or IV immune globulin
•