Systemic Autoimmune Diseases Flashcards
(106 cards)
1
Q
SLE stands for
A
systemic lupus erythematsus
2
Q
SLE mainly by what HSR
A
III
3
Q
not a diagnostic autoantibody for
A
rheumatic diseases
4
Q
SLE is most common
A
autoimmune rheumatic disease
5
Q
why is complement pathway involved in SLE
A
they are involved in removing immune complexes
6
Q
what is enviornmental trigger of SLE
A
UV light
7
Q
deposition of immune complexes where in SLE
A
glomeruli & blood vessels
8
Q
deposition of blood vessels in immune complexes are what give rise to
A
the rash (well known)
9
Q
hemorrhaging of small blood vessels in skin give rise to what rash in SLE
A
butterfly/malar
10
Q
what does SLE affect
A
everywehre - could eventually lead to organ failure and even death
11
Q
symptoms of SLE
A
arthirtis, skin rashes (butterfly rash), pleurisy, kidney dysfunction, fever, weakness, anemia, thrombocytopenia, memory loss, headaches, confusion, seizures
12
Q
treatment of SLE
A
immunosuppressive drugs, but SLE is a relapsing and remitting disease, so treatment is variable
13
Q
what are triggers of SLE
A
UV
cigarette smoke
14
Q
how can you diagnose SLE
A
ANA - antinuclear antibody (but most rheumatic diseases have this)
anti-SnRNP or smith antigen
15
Q
ANA stand for
A
anti-nuclear antibody
16
Q
most individuals with rheumatic disease will have
A
ANA
17
Q
Rheumatoid Arthritis is chronic, episodic
A
joint inflammation
18
Q
Rheumatoid Arthritis is due to what HSR
A
III and IV
19
Q
what cells are responsible for inflammation in jionts in Rheumatoid Arthritis
A
neutrophils
20
Q
what are the autoreactive cells in Rheumatoid Arthritis
A
CD4+ Th1 cells & Th17 cells
21
Q
key cytokine in Rheumatoid Arthritis
A
TNF alpha
22
Q
key cytokines in Rheumatoid Arthritis
A
IL-1
TNF alpha
prostaglandins
leukotrienes
23
Q
Th17 cells will activate what that will be responsible for remodeling of bone in chronic Rheumatoid Arthritis
A
osteoclastst
24
Q
what autoantibody is in Rheumatoid Arthritis
A
rheumatoid factor
25
rheumatoid factor is what isotype
IgM
26
rheumatoid factor not good for diagnosis b/c
only 70% pts with Rheumatoid Arthritis have it
27
detection of rheumatoid factor is useful in Rheumatoid Arthritis b/c it can be useful in
predicting outcome/prognosis of disease
28
pts who have rheumatoid factor in serum with Rheumatoid Arthritis tend to have (RF+)
more aggressive disease
29
test to detect RF is based on
agglutination
30
describe test to detect RF
latex particles that chemically coated with IgG
add pts serum
if pt has RF in serum it will bind to IgG and nbe very good at agglutination b/c IgM has 10 binding sites
so if pt does have RF in serum will see agglutination of serum
31
what is target antigen of RF
IgG
32
RF stands for
rheumatoid factor
33
test to detect RF and autoantibodies against any given autoantigen relies on activation of
complement
34
complement fixation test does what
detects if pt has antibody for particular antigen
35
fixation is old term for
ACTIVATION OF COMPLEMENT
36
describe complement fixation test
mix Ag with serum
add complement, a fixed amount
if antibody has bound to antigen complement will be activated
once complement is activated, will use up the fixed amount
to determine if complement is used up add indicator cells (RBC coated with antibody)
if antibody has not bound to the antigen the complement will not be used up and will be available to activation by cells that are coated with antibody (RBC) so in other words if in original tube there is not antibody specific for antigen, the complmeent is available for the RBC and will get lysis of RBC
if it is positive test and individual has antibody against antigen, the complement will b used up and no complement available to be activated on indicator cells (RBC) so no lysis of RBC
amount of erythrocyte lysis tell you if you have antibody specific for antigen
37
the important thing about the complement fixation test is that you add
limited amount of complement
38
negative result in complement fixation test, what is result
lysis of RBC
39
positive result of complement fixation test
no lysis of RBC, fixation of RBC
40
initiating factor of Rheumatoid Arthritis
don't know :(
might be multiple things, different for different ppl
41
Rheumatoid Arthritis results in infltration f
Th1 and Th17 into joint
42
Th1 and Th17 activate what in Rheumatoid Arthritis
macrophages, fibroblasts, osteoclasts
43
during Rheumatoid Arthritis what happens to synovial membrane
thickened due to infiltration of inflammatory cells into membrane and into synovial fluid
44
outgrowth from synovial membrane in Rheumatoid Arthritis what is it called
pannus
45
what is in pannus* high yield
lots of neutrophils
46
how do diagnose Rheumatoid Arthritis
no single test - based on clinical assessment, X-Ray
RF test
new test more specific than RF to test for cyclic citrullinated peptides
47
treatments for Rheumatoid Arthritis
physiotherapy
immunosuppressive drugs
TNF alpha inhibitors
anti-CD20 mAb
48
rewview pg 20
20
49
CRP is ___ __ protein
acute phase
50
CRP levels increase during
inflamamtion
51
CRP decrease is consistent with decrease in
inflammation
52
pts with Rheumatoid Arthritis that don't respond to TNF alpha treatment will usually respond to
antiCD20 treatment
53
Sjogren's syndrome is probably what HSR
IV
54
sjogren's syndrome more common in women ormen
women
55
what is most comon rheumatic disease besides SLE
sjogren's
56
what causes sjogren's syndrome
autoimmune destruction of exocrine glands - often lacrimal and salivary glands
57
parotid gland of pt with sjogren's is full of
CD4+ T cells and b cells
58
symptoms of sjogren's syndrome depend on
exocrine glands affected
59
symptoms of sjogren's syndrome
Symptoms are highly variable
Dry eyes - decreased production of tears, red eyes
Dry mouth - decreased production of saliva, difficulty swallowing, cracking of lips and/or tongue
Dry skin, nose, throat, vagina
Joint pain, skin rash
60
diagnosis for sjogren's syndrome
Shirmer’s Test, to test tear production
Spit Test
Autoantibodies = ANA (anti-Ro, -La) and RF
Biopsy of enlarged gland
61
RF is present in
many auto-rheumatic diseases
62
autoantibodies most useful in detecting sjogren's
anti-Ro, -La
63
Granulomatosis with Polyangiitis is due to what response
Th1
64
autoantibody associated with Granulomatosis with Polyangiitis
anti-neutrophil cytoplasmic autoantibodies (ANCA)
65
antigen recognized by anti-neutrophil cytoplasmic autoantibodies (ANCA)
PR3 (proteinase 3)
66
they think might have some disease/infection before Granulomatosis with Polyangiitis why
b/c of the granuloma that forms
67
describe mechanism of Granulomatosis with Polyangiitis
uptake of autoantigen by dendritic cells
autoantigen seems to be proteinase 3
autoantigen taken to lymph which stimulates CD4 to differentiate into Th1
Th1 activate marophaegs at site of infection
formation of granuloma
T cells can provide help to b cells to make autoantibody
68
proteinase 3 is released when
when neutrophils undergo netosis
69
Vasculitis in GP what is initiating antigen
PR3
70
in Vasculitis in GP autoantibodies stimulate the ___ of neutrophils
degranulation
71
symptoms of Granulomatosis with Polyangiitis
Airway Symptoms: rhinitis, cough, chest pain,
sinusitis, congestion
Kidney disease, due to glomerulonephritis
Saddle nose, due to cartilage damage in bridge
72
diagnosis of Granulomatosis with Polyangiitis
detect serum anti-PRS ANCA - the most specific
can also do x0ray, etc
73
review pg 29
29
74
Ankylosing Spondylitis is unusual b/c
ratio of males to females - 90% male
75
Ankylosing Spondylitis has very strong association with
HLA-B27
76
features of Ankylosing Spondylitis
fusion of vertebrae at base of spine - lose S shape curvature
77
clinical freatures of Ankylosing Spondylitis
Pain and stiffness in lower back and hips
Chronic synovitis (esp. Achilles)
20% have other HLA-B27-associated arthritides: Reactive Arthritis or Reiter’s syndrome
78
treatment of Pain and stiffness in lower back and hips
Chronic synovitis (esp. Achilles)
20% have other HLA-B27-associated arthritides: Reactive Arthritis or Reiter’s syndrome
anti-TNF mAbs (or drugs to inhibit activity of TNF)
79
role of HLA-B27 in Ankylosing Spondylitis
4 different theories
presentation of disease associated peptides by B27 - proposes that disease associated B27 alleles present peptide and recogniztion of peptide is what leads to arthritis
disease associated alleles cannot present peptide, failure to respond against pathogen that leads to development of Ankylosing Spondylitis
misfolded HLA-B27 on cell surface and in ER
one theory says misfolded on cell surface reocnigzed by NK and T cells
other theory says misfolded on ER triggers secretion f proinflammatory cytokines (unfolded protein response)
they don'tk now how it contributes
80
Scleroderma blood vessels involved look like
chronic graft rejection
81
features of blood vessles in Scleroderma
```
proliferation of smooth muscle cells
infiltration of B and T cells
imune complexes
fibrosis of blood vessels
just like chornic graft rejection
```
82
features of Scleroderma
occlusion of blood vessels - called raynaud's syndrome
tightening of skin - sclerodactyll
digital ulcers
83
limited cutaneous form of Scleroderma
restricted to skin on hands, arms, face, feet
84
diffuse cutaneous form of scleroderma
more servere:
extensive skin + internal organ(s)
lung, kidney, heart, GI diseases
complications: pulmonary fibrosis; pulmonary hypertension
85
diagnosis of scleroderma
anti-nuclear autoantibodies (ANA)
| RF+
86
review slide 35
35
87
omab means
only mouse
88
ximab means
mouse/human chimera
89
zumab means
humanized - almsot all human except CDR
90
mumab means
fully human
91
what is the target for SLE
skin
joints
kidneys
nervous system
92
what type of HSR for SLE
III
93
what are the autoantibodies for SLE
Anti-Sm
Anti-Ro
Anti-La
94
what is the target for Rheumatoid arthritis
joints
| blood vessels
95
what is HSR for rheumatoid arthritis
III & IV
96
autoantibodies for rheumatoid arthritis?
Anti-CCP
| RF
97
what is the target for scleroderma
skin
lungs
kidneys
heart
98
what are the autoantibodies for scleroderma?
anti-centromere
anti-ro
anti-RNP
99
what is the target for Ankylosing spondylitis
axial skeleton
peripheral joints
eye
100
what is the HLA for Ankylosing spondylitis
B27
101
what is the target for Granulomatosis with polyangiitis (WG)
kidneys
lungs
joints
skin
102
what is HSR for Granulomatosis with polyangiitis (WG)
II
103
what are autoantibodies for Granulomatosis with polyangiitis (WG)
anti-proteinase 3
104
what is target for sjogren's syndrome
exocrine glands
105
what is HSR for sjogrens syndrome
IV
106
what are autoantibodies for sjogrens syndrome
anti-Ro
Anti-La
RF
