The Systemic Autoimmune Diseases Flashcards
1
Q
Central Tolerance
A
testing B/T cells for self affinity
- autoreactive T-cells: killed
- autoreactive B-cells: modified
2
Q
Function of AIRE protein
A
key protein in deleting autoreactive T-cells before birth
3
Q
Peripheral Tolerance
A
rendering of autoreactive lymphocytes after birth
- self reactive lymphocytes rendered by CTLA-4/PD-1
- regulatory t-cells (CD25, IL-2, FOXP3) suppress self-immunity
- autoreactive T-cells undergo apoptosis when they activate
4
Q
Most-often-mutated gene in autoimmunity
A
PTPN22
others – NOD2, IL-2, IL-7
5
Q
Molecular mimicry
A
antigens on microbes mimic self-Ag
- immune response can cross-react to normal tissue
6
Q
Microchimerism
A
increased number cells from mother in those with AI diseases
7
Q
ANA: rim pattern
A
Systemic lupus
- anti ds-DNA
8
Q
ANA: homogenous pattern
A
Drug-lupus
- anti-ssDNA, anti-histone
9
Q
ANA: speckled pattern
A
think anything….
10
Q
ANA: centromere pattern
A
CREST
11
Q
ANA: nucleolar pattern
A
scleroderma
12
Q
Anti-dsDNA
A
systemic lupus
- rim pattern on ANA
13
Q
Anti-ssDNA
A
drug-induced lupus
14
Q
Anti-histone
A
drug-induced lupus
15
Q
Anti-Sm
A
systemic lupus (1/3 of patients with SLE)
16
Q
Anti-Ro/SSA
A
Sjogren’s patients, neonatal lupus (heart block)
17
Q
Anti-LA/SSB
A
Sjogren’s patients
18
Q
Anti-U1RNP
A
mixed CT disease
19
Q
Anti-Scl70
A
scleroderma
20
Q
Anti-RNA polymerase III
A
scleroderma
21
Q
Anti-centromere
A
CREST (mild scleroderma)
22
Q
Anti-Jo
A
polymyositis/dermatomyositis
- severe disease, lung damage, arthritis
23
Q
Anti-Mi2
A
dermatomyositis
24
Q
Anti-SRP
A
severe polymyositis variant w/ severe necrotizing myopathy
25
Anti-P155/TIF1g and Anti-P140/MJ
juvenile and paraneoplasic dermatomyositis
26
Cardinal sign of SLE
butterfly rash
27
Systemic Lupus Erythematosus
antinuclear antibodies
- UV light triggers flareups
- females/kleinfelters more common
- Tissue injury from Type III, cell destruction from antibodies (Type II)
28
Models for SLE
- Drug reactions (hydralaine/procainamide/anti-aTNF, isoniazid)
- Genetic deficiences (C1q is used for removing apoptotic bodies)
29
How many criteria must a patient present with to be deemed SLE?
4/11 (now 4/17)
30
Hematoxylin bodies
stripped nuclei acted-on by ANA's in lupus
| - homogenized nuclei
31
LE cell
stripped nucleus opsonized by ANA
| - devoured by phagocytes
32
What mediates lupus vasculitis?
immune-complex mediated
33
What is the appearance of the spleen in lupus?
adventitial onionskinning
34
What is an immune complex deposit in the glomerulus called?
wire loop
35
Lupus band test
granular Ig and complement at dermal-epidermal junction
36
What causes mouth ulcers in lupus?
infarcts from vasculitis
37
What are Libman-Sacks endocarditis?
In lupus, sterile thrombi on the lines of closure of valves
| - can cause infarcts
38
Neonatal Lupus
develop rash, heart block (AV node) by mom's auto-Ab (anti-Ro)
39
Blood in SLE
hypercoaguable
-polyclonal gammopathy (high Ig), lysis of RBC, destruction of neutrophils/platelets, low lymphocyte count, lupus anticoagulants, DVT (w/out lupus anticoagulants), thrombotic thyrombocytopenic purpura
40
Subacute cutaneous lupus
Anti-Ro is seen
| - have rash with lupus (or sjorgren's or RA)
41
Discoid lupus
rash with patches of scales and lost hairs
- hyperkeratosis as plugs (with hydropic changes)
- lymphocytes in patches
42
Drug-induced lupus
hydralazine, procainamide, isoniazid
| - anti-histone Ab, and anti-dsDNA Ab (patients receiving anti-aTNF tx)
43
What glands does Sjogren's effect primarily?
Salivary, lacrimal, pancreatic glands
| - fill with lymphocytes
44
Symptoms of Sjorgren's
autoimmunity against glands
- dry eyes and teeth decay (no healthy saliva)
- Mikulicz's syndrome (enlarged glands)
- test for using schirmer test
- Potentially malignant lymphoma
45
Scleroderma
"systemic sclerosis"
- inflammation --> dense fibrosis (seen in hands that cannot flex/extend)
- abundant CD4+ T-cells, calcium deposits
- begins with Raynaud's
- causes telangiectasias (vessels become dilated tufts)
- fibrosis of esophagus, small bowel, "onionskinning" of small renal artery
46
Raynaud's
fingers begin to hurt and change color (red, blue, white)
| - seen in cold
47
Common cause of death in Scleroderma
pulmonary fibrosis/pulmonary vascular narrowing
| - pulmonary HTN
48
Why does scleroderma of small renal artery present a problem?
Kidney thinks pt is in shock --> releases renin --> increase blood pressure --> stroke/heart problems
49
Limited Scleroderma
not as rapid of onset/generalized distribution as systemic sclerosis
- CREST syndrome
50
CREST syndrome
```
- Limited scleroderma
Calcinosis (deposits on skin)
Raynaud's
Esophageal fibrosis
Sclerodactyly
Telangiectasias
```
51
Morphea
Scleroderma-like changes in a patch
| - CD4 rich --> fibrosis
52
Parry-Romberg
scar contracts over 1/2 of face and underlying skull is deformed
53
Eosinophilic fasciitis
tight skin with many eosinophils in subcutaneous and fascia
54
Polymyositis
CD8 lymphocytes into muscle (in the endomysium)
- sk. rather than sm. m
- high CK and aldolase
55
Dermatomyositis
CD4 T-cells, B-cells, plasma cells (within perimysium/around the blood vessels)
- MAC often seen in vessel wall
- muscle fibers at edges of fascicles are most likely atrophied
- Heliotrope rash (eyelashes turn purple)
- Gottron's rash (reddish-purple) on knuckles (characteristic rash)
56
What is mechanic's hands a sign of ?
Polymyositis/dermatomyositis/anti-synthetase (Jo1) syndrome
| - dark keratin
57
Mixed Connective Tissue Disease (MCTD)
overlapping syndromes (lupus, scleroderma, RA, polymyositis)
- Anti-U1-RNP is high-titer Ab
- starts as synovitis of fingers plus Raynaud's
- death is usually due to pulmonary vascular sclerosis/fibrosis (rash--> calcification)
58
IgG4-related disease
fibroinflammatory diseases
(Riedel's fibrosing thyroiditis, AI pancreatitis, retroperitoneal fibrosis/periaortitis, fibrosis of salivary/lacrimal glands)
-features: fibrosis w/ cartwheel pattern, IgG4 lesions (high levels)
