B&L Unit 4 Flashcards
(217 cards)
1
Q
What are tophi?
A
The deposits of crystalline uric acid and other things in joints, skin, or muscle
Usually part of gout
2
Q
What are the 4 stages of gouty arthritis?
A
Asymptomatic hyperuricemia
Acute gouty arthritis
Intercritical gout - asymptomatic intervals between acute attacks of gout
Chronic tophaceous gout - deposits of uric acid crystals
3
Q
What are the 2 ‘big picture’ causes of gout?
A
Underexcretion - 90% of gout patients
Overproduction
4
Q
What enzyme do humans lack that increases risk of gout?
A
Uricase
5
Q
Uric acid is a product of _______ metabolism?
A
Purines
6
Q
Pseudogout is acute episodic arthritis due to _________
A
CPPD (calcium pyrophosphate dihydrate) crystals
7
Q
In rheumatoid arthritis, what is th emajor cellular component of synovial fluid?
A
Neutrophils
8
Q
What are 3 pro-inflammatory cytokines we care about?
A
IL-1
IL-6
TNF-a
9
Q
What are rheumatoid factors?
A
Immunoglobulins that recognize epitopes in the Fc portion of IgG
10
Q
Which finger joint is often spared in rheumatoid arthritis?
A
DIP
11
Q
What is the term for when a joint hurts but there is no evidence of inflammation?
A
Arthralgia
12
Q
What are the 3 types of joints?
A
Synarthrosis - bones come together and interlock (skull)
Amphiarthrosis - bones are joined by fibrocartilage (ribs)
Diarthrosis - bone articulation is cushioned by hyaline cartilage, stabilized by ligaments, moved by muscles and tendons, nourished and lubricate by synovial tissues
13
Q
What type of collagen are ligaments made out of?
A
Type 1
14
Q
What are entheses?
A
Where ligaments and tendons insert into bone
15
Q
What is axial arthropathy?
A
Arthritis involving the spine
16
Q
What is ankylosis?
A
Joint fixation
17
Q
What is spondylitis?
A
Inflammation of vertebrae
18
Q
What is an osteophyte?
A
A bony outgrowth
19
Q
What is a syndesmophyte?
A
Calcification of a ligament or tendon at the site of bony insertion
20
Q
What type of collagen is hyaline cartilage made out of?
A
Type II
21
Q
What are the 2 types of cells in synovium?
A
Type A - macrophage-like
Type B - fibroblast-like
22
Q
What 3 cells are increased in pannus? What happens to the synovium?
A
Increases in type A, type B, and immune cells
The synovium becomes inflamed and thickened
23
Q
What is synovium?
A
The thin layer of cells and capsule that covers all intra-articular surfaces other than the cartilage
24
Q
What are 2 disease categories red joints can indicate?
A
Infection
Crystal disease
25
What are the synovial WBC levels for inflammatory arthritis?
>2000
26
What are the 2 nodes on the fingers in osteoarthritis?
Heberden's
| Bouchard's
27
What joints are not affected by rheumatoid arthritis? (3)
DIP
| Thoracic and lumbar spine
28
What's the deal with pseudogout?
Inflammatory synovial fluid with Ca2+ pyrophosphate crystals
29
What is the NK cell's special killing mechanism?
ADCC - antibody-dependent cell-mediated cytotoxicity
NK cells have receptors for the Fc ends of IgG.
When IgG is bound to a cell, the NK cell can deliver apoptosis signals to the cell
30
What type of cells are NK cells?
Large granular lymphocytes
31
How does the BiTE drug work?
Bi-specific T-cell Engager
2 single-chain antibodies are coupled back-to-back. One against an tumor antigen and another against a T cell receptor.
This somehow gets the immune system to specifically act against the tumor cells
32
What does Azathioprine do?
What is a related drug?
It decreases DNA synthesis and mRNA transcription
It is used in organ transplantation
Mycophenolate mofetil. It has less toxicity
33
What does cyclosporine do?
Decreases IL-2 production.
This downregulates macrophages as APCs and lessens stimulation of T cells
34
Which 2 drugs for organ transplant rejection bind FKBP-12?
Tacrolimus - used in synergy with cyclosporine-A
| Sirolimus/rapamycin
35
What are the 2 mechanisms of anemia in SLE?
Anemia of chronic inflammation
Autoimmune hemolytic anemia - IgG and complement to RBCs -> destruction by spleen and liver
36
What happens with SLE patients produce phospholipid antibodies?
Blocks prothrombin activation in the clotting cascade
| Leads to increased clotting
37
What is correlated with SLE morbidity?
Kidney damage
38
What 5 things characterize axial arthropathies?
```
Axial arthritis
Peripheral arthritis
Enthesitis
Mucocutaneous lesions
Association with HLA-B27
```
39
Are males or females affected more in ankylosing spondylitis?
Males
7:3
40
Are males or females affected more in reactive arthritis?
Males
| 5-10:1
41
What precedes reactive arthritis?
Infectious diarrhea or urethritis 2-4 weeks before onset of arthritis
42
What is the affected joint pattern of reactive arthritis?
Asymmetric
Oligoarticular
Usually knees and ankles
Sometimes spine
43
What sort of tendon inflammation often occurs with reactive arthritis?
Dactylitis - diffusely swollen toes (sausage digit)
44
What genetic finding is most common in ankylosing spondylitis
HLA-B27
45
What is the environmental trigger most suspect for anklyosing spondylitis?
Bowel bacteria
46
4 possible theories of how HLA-B27 can predispose a person to develop ankylosing spondylitis (and reactive arthritis)
Arthritogenic peptide hypothesis
Molecular mimicry
Free heavy chain hypothesis - HLA heavy chains can form stable homodimers on a cell surface and trigger NK activation through recognition via killer cell immunoglobulin-like receptors
Unfolded protein hypothesis - HLA can misfold -> unfoldein protein stress response
47
What are the first 2 drugs types used for spondyloarthropathies?
NSAIDs
| Indole derivatives
48
What is the difference between heberden's and bouchard's nodes?
Heberden's are distal - DIP
| Bouchards are proximal - PIP
49
What is the fancy word for bunion?
Hallux valgus
50
What is the fancy word for bow-leggedness
Genu varus
51
What is the most common arthropathy?
Osteoarthritis
52
What does IL-1 do to cartilage?
Stimulates chondrocytes to make matrix metalloproteases -> matrix degradation
Also stimulates other inflammatory activities like prostaglandin, NO, and IL-6 production
53
What does TNF-alpha do to acrtilage?
Stimulates chondrocytes to make matrix metalloproteases -> matrix degradation
54
What are the 2 (unusual) elevated things in rheumatoid arthritis?
Rheumatoid factor in 85%
| Anti-cyclic citrullinated peptide in 70%
55
What is the WBC for synovial fluid analysis in rheumatoid arthritis
>2000/uL with predominantly neutrophils
56
Where are rheumatoid nodules located?
Extensor surfaces and tendon sheaths
57
What genotype is most common in rheumatoid arthritis?
HLA-DR4 is present in 50% of cases
58
In the early stage of rheumatoid arthritis, synovial fluid contains predominantly ________ cells
Mononuclear
59
What are the 3 hypothesized causes of rheumatoid arthritis
1. Antibodies to some arthritogenic peptide
2. T cell selection
3. The class II peptide is an antigen itself
60
TNF-A, IL-1, and IL-17 can induce osteocyte lineage cells to express ________, which results in _________
RANKL (receptor activator of nuclear factor kB ligand)
Interacts with RANK receptor on osteoclast precursors -> activation -> osteoclastic resorption of bone
61
What competitively binds RANKL and modulates its activity?
Osteoprotegerin
62
What are the 4 stages of gout?
Asympromatic hyperuricemia - elevated serum uric acid levels
Acute gouty arthritis
Intercritical gout - asymptomatic intervals between acute attacks of gout
Chronic tophaceous gout
63
What do gout crystals look like compared to CPPD crystals?
Needle-shaped and negatively birefringent (yellow)
Rhomboid-shaped and positively birefringent (blue)
64
What channel is important for uric acid reabsorption?
URAT1
So, inhibiting URAT1 will increase excetion of uric acid
65
Humans lack the enzyme ___________, which oxidizes uric acid to ___________
Uricase
| Allantoin
66
Overproduction of uric acid can be due to superactivity of ___________or deficiencies of ___________
PRPP synthetase
| HGPRT
67
In gout, initial recognition of MSU crystals by ___________ is critical to the inflammatory response
Toll-like receptors
68
3 drugs that can ameliorate an acute gouty attack
NSAIDS - anti inflammatory
Colchicine - prevents PMN movement
Corticosteroids - anti inflammatory
69
Where does pyrophosphate (PPi) come from in CPDD?
Metabolism of nucleoside triphosphates, particularly from articular chondrocytes
70
Can CPDD be cured?
No. There is no way to remove CPDD crystald form the joints or to retard further progression
71
What is used to treat acute pseudogout?
Anti-inflammatory drugs
72
Most of the parts of the coagulation cascade are synthesized by the ___________
Liver
73
Most of the enzymes in the coagulation cascade are ___________
Serine proteases
74
Inactive precursor proteins that are activated through cleavage into active enzymes are ___________
Zymogens
75
7 zymogens in the clotting cascade that become active serine proteases
Prekallikrein
Prothrombin (factor II)
Factors 12,11,10,9,7
(XII,XI,IX,X,VII)
76
Vitamin ___________ is important in clotting
K
77
What does factor XIII do (it is not a serine protease)
A transglutaminase that covalently links fibrin molecules together to form a stable clot
78
What are the 2 things von Willebrand protein does?
It is the carrier protein for factor VIII in the plasma
It adheres platelets to exposed collagen (binds to GIb)
79
Coagulation can be initiated with vascular disruption that leads to exposure of plasma to ___________
Tissue factor
80
Tissue factor binds factor ___________ in the presence of ___________
VIIa
| Calcium
81
The 2 clotting pathways meet at factor ___________
X (marks the spot)
82
What are the 3 granules of the platelets?
Dense granules - ATP, ADP, serotonin, calcium
Alpha granules - procoagulant proteins, growth factors, factors for platelet activation
Lysosomal granules - acid hydrolases
83
What attaches to exposed subendothelium and triggers the clotting cascade?
von Willebrand factor
84
What is the most common congenital bleeding disorder?
von Willebrand disease
85
What is Bernard-Soulier syndrome?
Expression of GP1b on platelet surface is reduced, leading to defect in platelet adhesion
Autosomal recessive
86
What si gray platelet syndrome?
Deficiency of alpha-granules in platelets
87
What is Glanzmann thrombasthenia?
An autosomal recessive disorder caused by absent/defective GPIIb-IIIa
Platelets can adhere but not aggregate
Signs are petechiae and easy bruising
88
What is the normal platelet range?
150,000-400,000/uL
89
What are the 3 treatments for immune thrombocytopenic purpura? (3)
Corticosteroids - reduces B cell clone-making autoantibody
IVIg - blocks splenic Fc receptors so they don't bind to antibody-coated platelets
Splenectomy - so the spleen doesn't get rid of platelets
90
What is antithrombin? What are the 2 most important things it targets?
A serpin that targets lors of things in the clotting cascade (most importantly thrombin and factor Xa)
91
Heparin is a cofactors for _____________
Antithrombin
Accelerates rate of protease inactivation
92
What does heparin cofactor II inhibit?
Thrombin
93
What is the cofactor for activated protein C (APC)?
Protein S
94
What is Factor V Leiden?
An abnormal factor V in the clotting cascade
Makes it resistant to APC, so factor Va remains activated longer than normal
Pro-thrombotic
95
Tissue factor pathway inhibitor is expressed by ____________ and inhibits the ___________ part of the coagulation pathway
Endothelial cells
| Entrinsic
96
The key enzyme in fibrinolysis is ___________
Plasmin
97
What primarily activates plasmin? (2) What enzyme type are they? What makes them?
Tissue plasminogen activator
Serine protease
Made by endothelial cells
urokinase plasminogen activator
Serine protease
Made by endothelial cells and kidney cells
98
What does thrombin-activatable fibrinolysis inhibitor do?
Is made by binding to the thrombin-thrombomodulin complex
Removes basic amno acids from C-terminal, which decreases plasminogen-binding sites on fibrin
99
What is the primary inhibitor of plasminogen activation?
Plasminogen activator inhibitor-1 (PAI-1)
A serpin
It inhibits tissue plasminogen activator
100
What is the primary plasmin inhibitor in blood?
alpha2-antiplasmin
A serpin
Inhibits plasmin that is freely circulating
101
What 4 things do endothelial cells express that inhibit thrombosis?
Heparan sultfate - cofactor for antithrombin
Ddermatan sulfate - cofactor for heparin cofactor II
Thrombomodulin - cofactor w/ thrombin for activating protein C
TFPI - inhibits extrinsic Xase complex
102
What 3 things do endothelial cells express to prevent platelets from activating?
Prostacyclin, nitric oxide - prevent platelet adhesion
Enzyme that metabolizes ADP to AMP and adenosine (a platelet inhibitor)
103
Do RBCs have MHCs? Do platelets?
No
| Yes
104
Blood group antigens are ___________
Blood group substances are ___________
Glycolipids
| Glycoproteins
105
What % of people are blood group substance secretors? What does this mean?
80
| The glycoproteins are also found in body fluids
106
Whta are isohemagluttinins?
Antibodies against A or B antigens in blood
107
What is the most important Rh locus?
d/D
The other is c/C and e/E
Rh antigens are on proteins coded for at these loci
108
What is the genotype for Rh phenotypes?
Rh+ is DD or Dd
| Rh- is dd
109
What on platelets binds von Willebrand Factor?
GP1b
110
What does ADP do during platelet degranulation?
Promotes exposure of GPIIb/IIIa receptor, which is essential for platelet aggregation
111
What on platelets allows aggregation?
GPIIb/IIIa
112
Which Ig is implicated in immune thrombocytic purpura?
IgG
113
Why does IVIg work for immune thrombocytic purpura?
Because splenic macrophages eat it instead of antibody bound to RBCs
114
What happens to RBCs in microangiopathic hemolytic anemia?
Schistocytes
Because microthrombi cause shearing
This results in hemolysis
115
What are the 2 underlying causes of microangiopathic heolytic anemia?
Thrombotic thrombocytic purpura
| Hemolytic uremic syndrome
116
What cuases thrombotic thrombocytic purpura?
Decreased ADAMSTS13 enzyme
Can't degrade von Willebrand multimers
Extra platelet adhesion
117
What causes hemolytic uremic syndrome?
Endothelial damage by drugs or infection
Platelets adhere!
118
What is the factor for hemophiliaa A,B, and C?
A - factor 8
B - factor 9
C - factor 11
119
aPTT measures the activity of the entire clotting pathway except _______________
Factor 7 (VII)
120
Both PT and PTT start with _______________-treated plasma
Citrate
121
Why do we use the Protime/International Normalized Ratio for clotting time?
Thromboplastin varies by manufacturer, so we normalize by adjusting for known potency
122
What does thrombin time detect deficiencies in?
Low or abnormal fibrinogen, fibrin split products, heparin
123
What is the cause of death in hemophilia?
CNS bleeds
124
What is the abnormal screening test in hemophilia?
PTT (vs. PT in factor VII deficiency)
125
What is the abnormal screening test in factor VII deficiency?
PT (vs. PTT in hemophilia – VIII and IX deficiencies)
126
How bad is hemophilia C (factor 11 deficiency)
Mostly post-operative bleeding, often delayed, especially on sites like prostate, uterus, bladder
Spontaneous bleeding is rare
127
What are the genetics for hemophilia C?
Autosomal recessive
| Common in Ashkenazi Jews and certain Middle Eastern populaitons
128
What 2 drugs reduce vitamin K/reduce its utiilzation?
Warfarin (Coumadin)
| Rat poison
129
What is the lupus anticoagulant?
IgG against phospholipid
130
What is the bleeding lab finding for lupus anticoagulant presence?
Prolonged PTT, since it binds the phospholipid that is added to the test tube to start the reaction
Is a pro-thrombic state though
We can check by seeing if adding normal plasma corrects the problem (it does not in this case)
131
Whan PTT>PT, which disease should we think os?
DIC
132
Signs and symptoms of vasculitis
Skin lesions
Constitutional symptoms - fever, weight loss, anorexia, weakness, fatigue
Musculoskeletal symptoms - arthralgias, arthritis, myalgias, peripheral neuropathy
133
Laboratory features of vasculitis reflect ____________________
Systemic inflammation
```
Anemia of inflammatory disease
Thrombocytosis
Low albumin
Elevated sedimentation rate and C-reactive protein
Polyclonal gammopathy
Possibly elevated liver enzyme tests
Low complement levels
Cryoglobulins
```
134
What 2 things characterize polymyositis and dermatomyositis?
Chronic muscle weakness
Infiltration of muscle tissue by chronic inflammatory cells
135
Which muscles in dermatomuositis/polymyositis are affected first?
Proximal extremeties
| can't comb hair
Also difficulty standing, rising from chairs, climbing stairs
136
What are 3 myositis-specific antibodies in polymyositis/dermatomyositis
Anti-synthetase: aminoacyl-tRNA synthetases that help translation
Anti-Mi-2: nuclear helicase. Associated with a good prognosis
Anti-signal recognition particle: translocaiton of newly synthesized proteins into ER
137
What antibody do we use to detect B cells with flow?
CD19
138
What do we use single radial immunodiffusion for?
Levels of individual immunoglobulin classes
139
What is the best overall test for Th1 activity?
A skin test with combos of antigens most people will have positive reactions to
(like the tuberculosis skin test)
140
What can be done to promote visualization of immune complexes in the lab?
Chill it for 1-7 days
| Immune complexes in serum are often insoluble in cold and precipitate out
141
How is imunohistochemistry different from immunofluorescence?
Immunohistochemistry uses a final antibody labelled with an enzyme (vs. fluorophore) like peroxidase that turns the tissue black/brown
Immunohistochemistry lasts longer
142
Direct immunofluorescence is a test for _____________
| Indirect immunofluorescence is a test for _____________
Antigen
Antibody
143
What is a sandwich/capture ELISA?
Use it to find an antigen that is at least divalent
One antibody is on the plate
The other has a fluorophore
The antigen is trapped in the middle
144
What are the 3 parts of the Virchow triad?
Decreased blood flow
Altered vessels (inflammation, mechanical injury, hypoxia)
Altered coagulability
145
Arterial thrombi occur under conditions of _____________
High shear stress
146
What is the typical composition of arterial thrombi?
Aggregated platelets with only small amounts of fibrin and red cells
White thrombi
147
What is the typical composition of venous thrombi?
Large amounts of fibrin with lots of RBCs
Red thrombi
148
How are D-dimers formed in thrombosis?
What does testing their levels tell us?
When cross-linked fibrin is degraded by plasmin
An indirect measure of amt of clotting. A negative D-dimer result means thrombosis probably isn't happening
149
How do you detect pulmonary embolisms?
Inhale and inject radionucleotides
CT scan
Look for areas of mismatch (areas that ventilate well but are not perfused)
150
What 2 things are given for arterial thrombi in an acute setting?
Heparin - prevents further clotting
| Fibrinolytic agent like tPA - lyse existing clot
151
What is a nontransforming retrovirus?
It is a virus that carries no oncogene
152
What does lentivirus mean?
Cause slow, ultimately fatal illnesses
HIV is one
153
What genetic difference do HIV-positive long-term surfvivors have?
Elite controllers?
CCR5 mutation, which is an HIV receptor
HLA-B57 - become infected but don't get AIDS. They make effective CTL to HIV peptides presented on this MHC
154
How does the HIV virus get to the Th cells?
It adheres to the dendritic cells in a way that it is not harmed
Then it is taken to the lymph node
155
What is the most common test for HIV?
ELISA for antibody to HIV
AND
Western Blot
156
What do the 2 antiretriviral drugs do?
Nucleosides - competitive inhibitors and chain-terminators
Non-nucleoside - bind hydrophobic pocket that alters catalytic site
157
What does Maraviroc do as an antiretroviral?
CCR5 antagonist - blocks entry of virus intoTh cells
158
What does Raltegravir do as an antiretrovirsl
Integrase inhibitor
159
Which antiretroviral is a CCR5 antagonist?
Which one is an integrase inhibitor?
Maraviroc
| Raltegravir
160
What are 3 pieces of evidence for cance immune surveillance taking place?
1. People with immunodeficiencies have higher rates of tumors
2. There are lots of activated T cells that recognize tumor-associated antigens
3. Some tumors spontaneously regress (probs due to an immunologic response)
161
What are tumor antigens?
Antigens expressed by tumor cells that are not readily found on the corresponding normal cell
162
How does CTL and NK activity correlate with MHC class I expresion?
Binding of MHC class 1 suppresses NK cells but gets CTLs to do there thing. The opposite is also true
163
How do the time courses of ESR and CRP vary?
C-reactive protein is upregulated much faster (hours) than erythrocyte sedimentation rate
164
What is the term for calcium deposition in cartilage?
Chondrocalcinosis
165
Serine proteases cleave targets at _____________ residues
Arginine
166
Vitamin K is required for clotting proteins that have a _____________ domain
Gla
167
Extrensic tenase complex is made of _____________, _____________, and _____________
Tissue factor
Factor 7
Ca2+
Activates factor 10
168
Intrinsic tenase is made of _____________ and _____________
Factor 11a
Factor 8a
Activates factor 10
169
Prothrombinase is _____________, and ____________
```
Factor 10a
Factor 5a (a cofactor)
```
It catalyzes the conversion of prothrombin to thrombin
170
von Willebrand factor hangs out in ____________s of endothelial cells
Weibel-Palade bodies
171
What are the 4 vitamin-K dependent factors?
9
7
2 (thrombin)
10
They all go into ten!
172
What 2 proteins (not factors) are vitamin K-dependent
Protein C
| Protein S
173
Protein C is activated by ____________ and ____________
Protein C inactivates ____________ and ____________
Thrombind and thrombomodulintogether
Factor 5a, factor 8a
174
Thrombin activates what 5 things?
What inactivates it?
13, 11, 8, 5, 2
Antithrombin III
175
TAFI is activated by ____________ and ____________
Thrombin and thrombomodulin (make a complex)
176
TAFI is a ____________, PAI ____________, alpha2-antiplasmin ____________
(enzyme type)
Exopeptidase
Serpin
Serpin
177
Platelets adhere to the damaged vessel wall directly via ____________ or indirectly via ____________
Collagen
| von WIllebrand factor
178
What platelet levels see spontaneous hemorrhage and increased hemorrhage with trauma or surgery?
What platelet levels can see life-threatening spontaneous hemorrhage?
20K-50K
179
What is pseudothrombocytopenia?
When people look like they have low platelet counts because they clump and the automated counter doesn't see them
Make sure you look at the smear to identify if present
180
What are the 3 subtypes of von Willebrand disease?
1. partial quantitative
2. Qualitative - decreased or increased adhesion to platelets
3. Almost no vWF
1 is most common, 3 is least
181
What drug is given to treat con Willebrand disease?
DDAVP - arginine vasopressin
182
What drug is given for acute DVT/PE?
What is used to prevent additional clots?
Heparin
Warfarin
183
What are the 5 known hypercoagulable states (like deficiencies)
```
Factor 5 Leiden
Prothrombin mutation
Antithrombin III deficiency
Protein S deficiency
Protein C deficiency
```
184
The prothrombin gene mutation and antithrombin III deficiency are associated with ____________ thrombosis
Venous
185
Whatat are the 6 things antithrombin III inhibits?
2+7 =9
| And 10,11,12
186
What are the 3 types of heparin we give as drugs?
Unfractionated - many lengths
Low molecular weight heparin
Fondaprinux - the minimal sequence in heparin for binding antithrombin
187
By which route is heparin administered?
IV or subcutaneously
188
What is the main difference in heparin forms in bioavailability and half life?
Smaller heparins have longer half lives (and are thus more prediactable) and better bioavailability
189
What is the main difference in heparin forms in their binding?
Only heparin containing atleast 18 saccharide units can bind to the antithrombin/thrombin complex. The smaller heparins just bind antithrombin
190
What compound can neutralize heparin?
Protamine sulfate
191
What is heparin-induced thrombocytopenia syndrome?
The platelet count decreases (by like 50%) 5-10 days after heparin
Caused by the development of antibodies to platelet factor 4/heparin complexes that activates them
192
What does streptokinase do?
Complexes and activates plasminogen to make more plasmin
Which makes it a fibrinolytic agent
193
What are the 3 classes of anti-platelet drugs?
Aspirin - inhibit formation of platelet products
Prevent activation/aggregation - ADP receptor antagonists
GIIb/IIIa inhibitors - block adhesion proteins
194
What is the difference in lymphocyte behavior between polymyositis and dermatomyositis?
Lymphocyte infiltrate directly into the fasicles
Perifascilar lymphocyte infilatration (a bland vasculopathy)
195
What is the difference in lymphocyte type between polymyositis and dermatomyositis?
CD8
| CD4
196
What skin findings are present in dermatomyositis? (6)
Gottron's sign -"an erythematous, scaly eruption occurring in symmetric fashion over the MCP and interphalangeal joints"
Heliotrope rash
Shawl or V sign/Erythroderma/holster sign
Mechanic's hands
Periungual telangiectasias and erythema
Calcinosis cutis (usually in juvenile dermatomyositis)
197
Polymyositis and dermatomyositis often have anti-________
Synthetase
One example is anti-Jo-1
198
What are the EMG findings of dermatomyositis/polymyositis
Increased activity when the needle is stuck in
Spontaneous fibrillations
Decreased amplitude of motor unit action potentials
199
________ is overexpressed in myocytes in polymyositis
MHC class I
An unfolded protein response made by the ER may be important
200
Categorize the vasculitises
Large vessel: Takayasu's arteritis, termporal arteritis
Medium vessel: Vuerger's disease, cutaneous vasculitis, Kawasaki disease, polyarteritis nodosa
Small vessel: Chur-Strauss, microscopic polyangiitis, ganulomatous with polyangiitis, cryoglobulinemia
201
What defines 'large vessels'?
Their connective tissues have their own blood supplies
202
What is ANCA?
Anti-neutrophil cytoplasmic antibodies
203
What are the 2 types of ANCA?
p-ANCA (perinuclear) - myeloperoxidase - microscopic polyanggitis (sorta)
c-ANCA (cytoplasmic) - PR3 - granulomatous polyangiitis
204
What is the main difference between giant cell arteritis and Takayasu's arteritis?
Takayasu's generally affects young people from Asia and doesn't affect the temporal artery
Giant cell arteritis mostly affects people of northern european background
205
Is palpable purpura blanchable?
No
206
Which vasculitis is due to smoking?
Thromboangiitis obliterans
| Buerger disease
207
Which disease involves heberden's and bouchard's nodes?
Osteoarthritis
Heberden's - DIP
Bouchard's - PIP
208
Which joint does rheumatoid arthritis spare?
DIPs
209
Which autoantibodies are extremely specific for rheumatoid arthritis?
RF AND anti-CCP
| Though only like 30% of RA patients have them both
210
Are neutrophils in the synovium in RA?
No, just in the synovial fluid
211
What mutation can cause CPPD crystal arthropathy?
ANKH gene -> excess intracellular PPi egress from chondrocytes
212
Enthesitis characterizes which arthropathy?
Seronegative spondyloarthropathies (like ankylosing spondylitis)
213
What is upregulated in the synovium in seronegative spondyloarthropathies?
TNF-a
214
What joints are affected in reactive arthritis?
Lower extremity
215
What genes are lupus associated with?
HLA-DR3
| C4A null allele
216
Anti-synthetase syndrome is associated with PM/DM and what other disease?
Interstitial lung disease
217
What 2 receptors that, when bound, can downregulat CTLs?
CTLA-4
| PD-1
