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Systemic Lupus Erythematosus (SLE)

Ch. 4 p (124 – 135)March 2016

Systemic Lupus Erythematosus (SLE)

Chronic multisystem inflammatory disease Associated with abnormalities of immune system Results from interactions among genetic, hormonal, environmental, and immunologic factors

Systemic Lupus Erythematosus

Affects the Skin Joints Serous membranes Renal system Hematologic system Neurologic system

Systemic Lupus Erythematosus

Chronic Characterized by exacerbations & remissions Most cases occur in women of childbearing years

Etiology

Etiology is unknown Most probable causes Genetic influence Hormones Environmental factors Certain medications

Pathophysiology

Autoimmune reactions directed against constituents of cell nucleus, DNA Antibody response related to B and T cell hyperactivity

Systemic 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 Alopecia

Clinical Manifestations

Musculoskeletal Polyarthralgia with morning stiffness Arthritis Swan neck fingers Ulnar deviation Subluxation with hyperlaxity of joints

Clinical Manifestations

Cardiopulmonary Tachypnea Pleurisy Dysrhythmias Accelerated CAD Pericarditis

Clinical Manifestations

Renal Lupus nephritis Ranging from mild proteinuria to glomerulonephritis

SLE, SKIN

SLE, Glomerulous

Clinical Manifestations

Nervous system Generalized/focal seizures Peripheral neuropathy Cognitive dysfunction Disorientation Memory deficits Psychiatric symptoms

Clinical Manifestations

Hematologic antibodies against blood cells Anemia Leukopenia Thrombocytopenia Coagulopathy Anti-phospholipid antibody syndrome

Clinical Manifestations

Infection Increased susceptibility to infections Fever should be considered serious Infections such as pneumonia are a common cause of death

Diagnostic Studies

No specific test SLE is diagnosed primarily on criteria relating to patient history, physical examination, and laboratory findings

Libman-Sacks vegetations

Libman-Sacks vegetations, (Libman-Sacks endocarditis), are on BOTH sides of the leaflet


The 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 activity

Antigen

Ab System

SLE
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 problems


Chronic 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




رفعت المحاضرة من قبل: Dr Faeza Aftan Zghair Alrawi
المشاهدات: لقد قام 7 أعضاء و 158 زائراً بقراءة هذه المحاضرة








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