viral and bacterial antigens are so similar to self antigens that an immune response to the pathogen results in an attack on self antigens (cross reacting) some pathogens are able to change antigens to appear like self cells
What is cross reactivity? (in autoimmunity)
What disease is abbreviated SLE?
Systemic Lupus Erythematosus aka Lupus
What is Lupus (SLE)?
A CHRONIC SYSTEMIC inflammatory disease that involves multiple body systems.
What type of immune response is seen with lupus?
Complement fixing complexes such as antibodies with DNA are deposited in the kidney, skin, joints and choroid plexus.
What is one of the frequent targets of antibodies/complement fixing complexes in lupus?
Nuclear particles (DNA, etc)
What areas of the body are frequently affected by lupus?
Kidney, skin, joints, and choroid plexus.
What is the choroid plexus?
Structure in the ventricles of the brain where cerebrospinal fluid (CSF) is produced.
What is the cause of SLE (lupus)?
It is unknown. Possibilities are genetic, hormonal, environmental and drug induced.
What are the most common symptoms of lupus (SLE)?
Joint pain (arthritis), sensitivity to infections, swollen lymph glands, acute and chronic glomerulonephritis, increased premature birth and spontaneous abortion (miscarriage), Cardiac problems such as pericarditis, tachycardia, ventricular enlargement, & photosensitive rash.
Is the course of the disease predictable?
No, it is highly variable.
What does "highly variable" refer to in SLE?
It is completely different from individual to individual and also an individual may have periods of remission and periods of disease.
What tissues are most commonly affected in SLE?
What are the functions of the glomerulus and nephrons?
filtering and concentrating
What does "Lupus" mean?
What is a photosensitive rash?
A rash that develops on skin exposed to UV light
What do we call the signature rash for lupus that covers the bridge of the nose and cheeks?
A "butterfly rash"
What percent of patients with lupus exhibit a photosensitive rash?
What would you be likely to see in a CBC (complete blood count) of a Lupus patient with regards to RBC's WBC's, and platelets?
A urinalysis in a person with lupus would likely show which of the following? a) blood b) decreased proteins c) bacteria d) increased proteins
a and c - blood and increased proteins
If you performed a BUN on a lupus patient would creatinine levels be increased or decreased?
increased due to renal damage
What is acrocyanosis and what laboratory findings go along with this condition?
bluish or cyanotic color of the extremities (most often hands)/Cryoglobulins
What would the complement profile in lupus look like?
C3 and C4 would be decreased
What test is ANA and what would the results in lupus be?
Anti-nuclear antibody - this is frequently seen in lupus
What tests on a lupus patient often get a false positive?
STS or RPR (tests for syphilis), and RA/RF (rheumatoid arthritis/rheumatoid factor)
What are STS and RPR tests?
Serum test for syphilis and rapid plasma reagin (also for syphilis)
What are RA and RF?
Rheumatoid arthritis and rheumatoid factor
What is an IFA?
Indirect fluorescent antibody
Explain how IFA is done in 7 steps. (8?)
1) Mouse kidney or human epithelial cells are fixed to a slide 2) a patient's serum is added and incubated and allowed to react with the cells on the slide 3)Incubate* 4) Fluorescent (Fluorescein isothiocyanate) labeled anti-human globulins (AHG anti-antibodies) are added 5)labeled AHG reacts with Antibodies attached to the antigen (cells on slide) 6) Slide is washed to removed excess AHG 7) the fluorescence is observed (titer or pattern) *In lecture bill stated that there would be another wash after the incubation of the serum and slide - this would make 8 steps
What are the two methods used to read an IFA?
Titer (serial dilution) and pattern
Explain what the following results of IFA indicate. (in SLE) a) less than or equal to 20 b) 1:40 to 1:160 c) greater than 160
a) negative b) suggestive of presences of ANA's (anti nuclear antibodies) c) highly suggestive of ANA's
What does a homogenouse or diffuse IFA pattern look like?
The entire nuclease is glowing GREEN.
What Antibodies are present in a diffuse or homogenous pattern?
Anti DNA -histone (anti-nucleoprotein) anti DNP (deoxyribonucleoprotein)
What disease(s) is/are associated with a diffuse or homogeneous IFA pattern?
SLE (lupus) or RA (rheumatoid arthritis)
1. What does a negative pattern look like?
No fluorescence in the cell. It might be greenish, but it isn't glowing
2. What does a peripheral or rim pattern look like?
The fluorescence is just along the edge-where the antibodies are attaching
3. What antibodies are present in a peripheral or rim pattern?
Anti-DNA, Anti-dsDNA, Anti-nDNA
4. What disease is associated with a peripheral/rim pattern?
5. What does a light speckled pattern look like?
Only a few speckles in each cell
6. What does a heavy speckled pattern look like?
Very dense fluorescence all throughout the cell
7. What other pattern can heavy speckled be confused with?
8.When is heavy speckled pattern the most helpful?
When we know only an anti-Sm is present, which is specific for Lupus
9.What does a nucleolar pattern look like?
Just the nucleoli of the cells are fluorescent
10. What antibody is present in a nucleolar pattern?
11. What diseases are associated with a nucleolar pattern?
Scleroderma and Sjogren's Syndrome
12.What is the purpose of ANA?
13. What is the next step if you get a positive ANA?
Get more specific-react patients serum with extractable antigens
14. What extractable antigen is associated with SLE(lupus)?
15. What 2 types of DNA can you test for in Lupus
DS-DNA and SS-DNA
16. What type of assay do you use to test for SS-DNA?
17. What percentage of patients with Lupus have Anti-Sm?
18. What antibody is associated with MCTD (mixed connective tissue disease)?
19. What is the treatment for Lupus patients?
20. What do corticosteroids do?
Reduce the immune process
21. What is another type of immunodiffusion test we can do for ANA?
22. What type of inflammatory disorder is Sjogren's Syndrome?
23. In Sjogren's Syndrome, what part of the body do antibodies target?
mucus secreting glands-salivary, tear ducts, etc.
24. Is Sjogren's Syndrome a life threatening condition?
No-but very annoying
25. What is the major symptom of Sjogren's Syndrome?
no saliva, no tears (ex:eat a cracker without saliva)
26. What gender and age group does Sjogren's Syndrome primarily affect?
women and elderly patients
27. Why does Sjogren's Syndrome primarily affect women?
Estrogen enhances immune responses
28. What is the characteristic symptom of Scleroderma?
hardening of the skin-tissue turns fibrotic
29. What disease state can Scleroderma progress to?
30. What substance gets deposited into the internal organs?
31. What organ is primarily affected?
esophagus-you can't swallow or eat
32. How severe is Scleroderma?
very-5 year survival rate is about 40%
33. What are common symptoms with Scleroderma?
Can't relax mouth or purse lips together, can't move hands(collagen deposits in joints), poor cirulation
What does the ANA pattern look like for polymyositis?
Which antibody is present for polymyositis?
What are the symptoms of the inflammatory myopathy of polymyositis?