Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
15 Cards in this Set
- Front
- Back
|
What are the two major forms of autoimmune disease? |
Localized - T cells and autoantibodies directed against a single cell type or organ (ex. Hashimoto's thyroiditis, autoimmune hemolytic anemia)
Systemic - autoimmune reaction directed against an array of antigens (ex. SLE, Sjögren's, SSc, RA) |
|
|
What are the four categories of ANA? |
Antibodies to: DNA Histones Non-histone proteins bound to RNA Nucleolar antigens
ANA in and of itself is non-specific |
|
|
How do you detect if a patient has autoantibodies?
What can a fluorescence pattern show? |
Indirect immunofluorescence assay.
Homogenous - generic; stains everything Speckled - non-DNA constituents Nucleolar - nucleolar RNA (SSc) Centromere - CREST syndrome |
|
|
What specific antibodies are detected in SLE, Sjögren's and Systemic Sclerosis? |
SLE - anti-dsDNA and SM antigens Sjö - Anti-RNP (SS-A, SS-B) SSc - Anti-DNA topoisomerase 1 |
|
|
What is the basis of Sjögren's syndrome?
What is the difference between primary and secondary? |
Lymphocytic infiltration and fibrosis of lacrimal/salivary glands - mostly CD4+ helper T cells. Results in dry eyes and dry mouth.
If only symptoms, primary. If present as part of constellation of symptoms of systemic autoimmune disease, secondary |
|
|
What may serology a patient with Sjögren's show?
What does a high titer of SS-A correspond to?
Will patients express RF? |
ANA's in 50-80% of patients; anti-SS-A and anti-SS-B in 90% which is most specific
High titer of SS-A corresponds to likelihood of extraglandular disease
75% patients will express RF without arthritis. |
|
|
What is the typical Sjögren's patient (age, gender)
What are some of the extraglandular complications?
What risk of cancer is present? |
~90% of patients are female between 35-40
Extra-glandular: synovitis, pulmonary fibrosis, peripheral neuropathy
Lymphadenopathy due to B-cell hyperplasia can increase risk of lymphoma by 40X |
|
|
What is the pathogenesis of systemic sclerosis?
What organ(s) are affected?
What is the molecular mechanism? |
Excessive fibrosis throughout the body.
Skin is most affected, but GI, kidneys, heart, muscles and lungs often affected, too
T-cells elaborate cytokines (IL, TGF, PDGF) which stimulate fibroblasts to produce collagen; also endothelial injury produces intimal fibrosis and platelet aggregation --> ischemia and fibrosis |
|
|
What are the two general categories of SSc?
What kind of ANA's do each show? |
Diffuse - widespread skin (truncal and acral) and visceral involvement; anti-DNA topoiso 1 in 28-70% of patients
Limited - disease limited to the acral skin; REST = Raynauds, Esophageal dysmotility, Sclerodactyly, Telangiectasia; anti-centromere antibodies |
|
|
What is the pathology of involvement in the skin and GI tract? |
Skin: edema, perivascular T-cells, swelling and degeneration of collagen, thickening and occlusion of blood vessels, thinning of epidermis, loss of rete pegs, atrophy of dermal appendages
GI - collagenous replacement of the muscularis, esp in the esophagus resulting in stricture formation and reflux; loss of villi in small bowel can cause malabsorption |
|
|
What involvement can SSc have in the kidneys, lungs and heart? |
Kidney's (60-70%) - fibrosis of blood vessel walls; hypertension in 30% of patients; renal failure
Lungs - pulmonary fibrosis, PHTN
Heart - pericarditis, myocardial fibrosis |
|
|
What ANA IF patterns are associated with SLE, Sjögrens and SSc? |
SLE: IF=homogenous; Ab-assay=anti-dsDNA and anti-Sm Sjögrens: IF=speckled; Ab-assay=anti-SS-A&B SSc: IF=nucleolar pattern; Ab-assay=anti-topoisomerase 1 |
|
|
What is the definition of RA?
What extraarticular areas may it affect?
What is RF and what %age have it? |
Chronic systemic inflammatory autoimmune disorder that attacks joints, leading to destruction of cartilage and ankylosis (fusion of bones)
May involve skin, blood vessels, heart, lungs, muscle
RF is an IgM autoAb for the Fc portion of IgG in 80% of patients |
|
|
What is the pathology of RF? |
Immune complexes form in sera, synovial fluid and synovial membranes and lead to T, B and m-phage infiltration.
Synovial hyperplasia, edema, thickening, incr. vascularity, fibrin deposition.
Fibrovascular pannus grows and erodes cartilage; deforms and fuses joint |
|
|
What happens in the skin and blood vessels? |
Rheumatoid nodules (fibrinoid necrosis of collagen); may form at other sites
Blood vessels show vasculitis with tissue infarction |
