Systemic Lupus Erythematosus (SLE)
Ch. 4 p (124 – 135)March 2016Systemic Lupus Erythematosus (SLE)
Chronic multisystem inflammatory disease Associated with abnormalities of immune system Results from interactions among genetic, hormonal, environmental, and immunologic factorsSystemic Lupus Erythematosus
Affects the Skin Joints Serous membranes Renal system Hematologic system Neurologic systemSystemic Lupus Erythematosus
Chronic Characterized by exacerbations & remissions Most cases occur in women of childbearing yearsEtiology
Etiology is unknown Most probable causes Genetic influence Hormones Environmental factors Certain medicationsPathophysiology
Autoimmune reactions directed against constituents of cell nucleus, DNA Antibody response related to B and T cell hyperactivitySystemic Lupus Erythematosus
The cause of the breakdown in self-tolerance in SLE is unknown it may include excess or persistence of nuclear antigens, multiple inherited susceptibility genes, & environmental triggers (e.g., UV irradiation, which results in cellular apoptosis and release of nuclear proteins).Clinical Manifestations
Dermatologic Cutaneous vascular lesions Butterfly rash Oral/nasopharyngeal ulcers AlopeciaClinical Manifestations
Musculoskeletal Polyarthralgia with morning stiffness Arthritis Swan neck fingers Ulnar deviation Subluxation with hyperlaxity of jointsClinical Manifestations
Cardiopulmonary Tachypnea Pleurisy Dysrhythmias Accelerated CAD PericarditisClinical Manifestations
Renal Lupus nephritis Ranging from mild proteinuria to glomerulonephritisSLE, SKIN
SLE, GlomerulousClinical Manifestations
Nervous system Generalized/focal seizures Peripheral neuropathy Cognitive dysfunction Disorientation Memory deficits Psychiatric symptomsClinical Manifestations
Hematologic antibodies against blood cells Anemia Leukopenia Thrombocytopenia Coagulopathy Anti-phospholipid antibody syndromeClinical Manifestations
Infection Increased susceptibility to infections Fever should be considered serious Infections such as pneumonia are a common cause of deathDiagnostic Studies
No specific test SLE is diagnosed primarily on criteria relating to patient history, physical examination, and laboratory findingsLibman-Sacks vegetations
Libman-Sacks vegetations, (Libman-Sacks endocarditis), are on BOTH sides of the leafletThe diagnosis is established by demonstration of four or more of the criteria during any interval of observation.
Diagnostic Studies
Antinuclear antibodies ANA and other antibodies indicate autoimmune disease Anti-DNA (anti double stranded DNA), & anti-Smith antibody tests most specific for SLE LE (Lupus Erythematosus cells) prep can be positive with other rheumatoid diseases ESR & CRP are indicative of inflammatory activityAntigen
Ab SystemSLE
Drug-Induced LE
SS—Diffuse S—CREST
Many nuclear antigens (DNA, RNA, proteins)
Generic ANA (indirect IF)
>95
>95
70–90 70–90 50–80 Native DNA
Anti–double-stranded DNA 40–60 <5
<5
<5
<5
Histones
Antihistone
50–70 >95
<5
<5
<5
Core proteins of small nuclear RNP particles (Smith antigen)
Anti-Sm
20–30 <5
<5
<5
<5
RNP (U1RNP)
Nuclear RNP
30–40 <5
15
10
<5
RNP
SS-A(Ro)
30–50 <5
<5
<5
70–95 RNP
SS-B(La)
10–15 <5
<5
<5
60–90 DNA topoisomerase I
Scl-70
<5
<5
28–70 10–18 <5
RNP, ribonucleoprotein;
Diagnostic Tests
CBC for hematologic problems UA for lupus nephritis X-rays of affected joints Chest x-ray for pulmonary problems ECG for cardiac problemsChronic Discoid Lupus Erythematosus. disease with skin manifestations mimic SLE, face and scalp are usually affected, Only 5% to 10% of patients develop systemic dis. 35% of patients show a positive ANA test, Abs to ds DNA are rarely. skin biopsy show deposition of Ig and C3 at the dermoepidermal junction similar to that in SLE.
Drug-Induced Lupus Erythematosus SLE –like syndrome may develop in patients receiving hydralazine, procainamide, isoniazid, and D-penicillamine,Positive ANAs,Negative ds DNA Abs are rare,HLA-DR4 allele are at a greater risk The disease remits after withdrawal of the offending drug.
Lupus and pregnancy Infertility can result from SLE treatment regimen SLE is associated with complications of pregnancy Pregnancy & post partum can cause exacerbations of SLE Women with serious SLE should be counseled against pregnancy