BL-SLE Flashcards
Myasthenia gravis, Goodpasture’s syndrome, and autoimmune thyroiditis are examples of:
Organ specific autoimmunity
Strongest SLE genetic association:
C4 null alleles
Which antibody may be involved in the pathogenesis of lupus nephritis? sle
Anti-double-stranded DNA (ds DNA) antibodies
Autoimmunity is defined as the generation of an immune response directed against
self
Organ specific autoimmunity is defined as an immune response directed against a:
single autoantigen or a restricted group of autoantigens within a given organ.
autoimmune destruction of only those organs expressing the relevant autoantigens.
SLE is a multigenic disease with a major _____contribution
MHC
The pathophysiology of SLE involves both Type _____antibody-mediated mechanisms.
II and III (2/3)
Both the ________immune systems are involved in the misdirected recognition of self as foreign in SLE.
innate and adaptive
SLE flares can be triggered by environmental exposures such as.
the sun
The ______ is a major target organ in SLE, and the extent of damage is directly correlated with overall patient outcomes.
kidney
The presence of _____ in SLE patients is a manifestation of loss of tolerance to self-antigen as well as a useful diagnostic marker.
high-titer ANA
Treatment of SLE is directed at decreasing exposure to disease triggers (sun blocks), decreasing the inflammatory response [NSAIDs, and corticosteroids], and decreasing the ______immune responses
cellular/humoral
_______, belimumab is approved for the treatment of SLE.
anti-B cell therapy
The presence of high-titer ANA in SLE patients is a manifestation of loss of _______as well as a useful diagnostic marker.
tolerance to self-antigen
Treatment of SLE is directed at decreasing exposure to disease triggers (sun blocks), decreasing the _____ [NSAIDs, and corticosteroids], and decreasing the cellular/humoral immune responses
inflammatory response
___________is defined as an immune response against multiple autoantigens rather than to autoantigens of a given organ.
Systemic autoimmunity
SLE is a ________ which affects multiple organ systems including the skin, joints, serosal surfaces (pleura and pericardium), kidneys, central nervous system, lungs, and hematologic system.
chronic, systemic autoimmune disease
SLE Criteria for disease classification include (11)
4 qualifies for SLE
- Malar rash
- Discoid rash
- Photosensitivity
- Oral ulcers
- Arthritis
- Serositis
- Renal involvement
- Central nervous system involvement (seizures or psychosis),
- Hematologic disorders
- Immunologic disorders
- Antinuclear antibody (ANA).
SLE is a disease primarily of the young women with a female to male ratio of _____, with onset after puberty reaching a peak during the childbearing years.
9:1
Predisposing Factors SLE
Genetics
HLA-DR3; and C4A null alleles
Interferon (IFN)-α and IFN-β
Predisposing Factors SLE
Environment
Sex hormones
UV exposure
Specific antibody-mediated disease (Type II) of SLE
Hemolytic anemia
anti-Phospholipid antibodies
Central Nervous System Manifestations
Immune complex mediated disease (Type III):of SLE
Lupus nephritis Antinuclear Antibodies (ANA):
Antinuclear antibodies (ANAs) are unusual antibodies, detectable in the blood, that have the capability of binding to certain structures within the _____ of the cells.
nucleus
HLA-DR4 association
RA
HLA-B27 association
AS
HLA-DR3 association
SLE- (SLE is 3 letters)
*not the strongest
C4 null allele is better
Shared epitope QKRAA association
RA
SLE demographics
young white female
Problem list for SLE
Pancytopenia Arthritis Alopecia Skin rash Photosensitive- malar Glomerulonephritis
________ (AB) can cause drug induced lupus
Minocycline
Why does SLE present with low RBC
autoimmune hemolyitic anemia (possibly)
major groups of autoantibodies produced in SLE
ABs are targeting tissues (path) or nuclear content (marker)
Anti-nuclear antibodies are a____ for SLE
marker
ANTIBODIES AGAINST _____ ARE PATHOLOGICAL
TISSUE
ANA test
a slide with cell w/ nucleus exposed
if ANA present, ABs bind the nucleus
AB that sees human AB with florescence tags added
if bound and light up, anti-nuclear antibodies are present
Repeat w/ dilution to determine + strength
Lupus has ______ autoantigens
multiple
as opposed to like myasthenia gravis which is specific
In serum sickness the antigen is:
Foreign
In lupus the antigen is:
Self
In SLE defective clearance of immune clearance/apoptotic cells- ______ is released and can be “seen” by immune system
DNA and RNA
Interferon (IFN)-α and IFN-β upregulate the expression of a variety of genes in _______.
lymphocytes
*This “IFN signature” of gene expression is more prevalent in patients with active SLE.
Basic problem of SLE
defective clearance of immune clearance
ineffective clearance of apoptotic cells
Does SLE have x-ray features?
NO
Environmental factors of SLE
Sun
Sex hormones
SLE will have a _____ immunoflorescence
Lumpy Bumpy
Goodpasture will have a _____ immunoflorescence
Linear
