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*med3* > Immunology - Autoimmune Disorders > Flashcards

Immunology - Autoimmune Disorders Flashcards

1
Q

what type of HS is SLE?

A

type III

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2
Q

Why is C3/C4 low in SLE?

A

Ab-Ag complexes activate complement

supply < demand => hypocomplementemia

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3
Q

what antibodies cause lupus?

A

anti-nuclear antibodies (ANA’s)

anti-double stranded DNA (anti-dsDNA)

anti-smith (anti-Sm)

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4
Q

ANA is present in the serum of _____ patients and in __% of normal patients

A

lupus

5

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5
Q

Is ANA sensitive or specific?

A

sensitive, meaning a negative result indicates the disease is very unlikely

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6
Q

why isn’t ANA specific for lupus?

A

because it is found in many other autoimmune disorders

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7
Q

what is considered a normal ANA?

A

< 1:160

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8
Q

what 2 antibodies are specific for SLE?

A

anti-dsDNA anti-Sm

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9
Q

what Ab can be used to monitor disease activity in SLE?

A

anti-dsDNA will increase during flares

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10
Q

what Ab is associated with renal involvement in SLE?

A

anti-dsDNA = glomerulonephritis

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11
Q

what is anti-Sm directed against?

A

small nuclear ribonucleoproteins (snRNPs)

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12
Q

what are snRNPs?

A

small nuclear ribonucleoproteins made up of RNA and protein combine with RNA transcripts –> form a spliceosome –> removes a portion of the RNA transcript

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13
Q

what are RNA transcripts?

A

pieces of RNA that have been synthesized from DNA

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14
Q

what are snRNPs used for?

A

modify RNA before it is translated into a protein

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15
Q

antibodies against snRNPs are seen in what?

A

SLE

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16
Q

anti-Sm antibodies are directed against what?

A

snRNPs

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17
Q

what is an ENA panel?

A

extractable nuclear antigens panel of blood tests against nuclear antigens

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18
Q

when would you order an ENA panel?

A

if you suspect an autoimmune disease

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19
Q

what antibodies are tested for on an ENA panel?

A

Anti-RNP Anti-Sm Anti-SS-A (Ro) Anti-SS-B (La) Scl-70 Anti-Jo-1

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20
Q

anti-RNP

A

MCTD SLE scleroderma

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21
Q

anti-Sm

A

specific for lupus

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22
Q

anti-SS-A (Ro)

A

Sjogren’s syndrome SLE scleroderma

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23
Q

anti-SS-B (La)

A

Sjogren’s syndrome SLE scleroderma

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24
Q

scl-70

A

specific for scleroderma

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25
Q

anti-Jo-1

A

polymyositis

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26
Q

SLE flare Sx

A

fever weight loss fatigue LAD

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27
Q

what causes Raynaud’s?

A

vasospasm in arteries supplying blood to the fingertips causing ischemia

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28
Q

what other Sx are seen in SLE?

A

oral/nasal ulcers arthritis serositis “penias” - anemia, thrombocytopenia, leukopenia - results from antibody attack of cells - type II HS where Ab directly attacks the cell

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29
Q

what is lupus cerebritis?

A

lupus affecting the brain resulting in cognitive dysfunction (confusion, memory loss) strokes seizures

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30
Q

lupus nephropathy presents as what syndrome?

A

nephritic or nephrotic syndrome or both

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31
Q

what is the most common lupus renal syndrome?

A

diffuse proliferative glomerular nephritis

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32
Q

what syndrome does diffuse proliferative glomerular nephritis present as?

A

nephritic syndrome

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33
Q

what syndrome dose membranous glomerular nephritis present as?

A

nephrotic syndrome

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34
Q

What is Libman-Sacks endocarditis?

A

aka marantic endocarditis nonbacterial inflammation of valves ***classically affects both sides of mitral valve

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35
Q

What is the main difference between Libman-Sacks and bacterial endocarditis?

A

bacterial endocarditis only affects one side of the mitral valve, typically the left atrial side Libman-Sacks endocarditis affects both sides of the mitral valve

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36
Q

anti-phospholipid antibodies occur in association with what?

A

lupus nb: can also occur as a primary problem

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37
Q

what are the 3 important clinical consequences of anti-phospholipid antibodies?

A

“antiphospholipid syndrome” - blood clots increased PTT false positive syphilis (RPR/VDRL)

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38
Q

what are anti-phospholipid antibodies?

A

Ab’s against proteins in phospholipids

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39
Q

what is antiphospholipid syndrome?

A

pt w/ antiphospholipid Ab’s develops venous or arterial thrombosis causing DVTs, stroke, fetal loss

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40
Q

what are the 3 clinically relevant antiphospholipid antibodies?

A

anti-cardiolipin “lupus anticoagulant’ anti-ß2 glycoprotein

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41
Q

what does anti-cardiolipin give a false positive for?

A

RPR/VDRL syphilis

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42
Q

why does anti-cardiolipin give a false positive for syphilis?

A

syphilis produces antibodies against the RPR/VDRL antigens but the anti-cardiolipin antibody also reacts to the RPR/VDRL antigens

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43
Q

what test does the lupus anticoagulant interfere with?

A

PTT test will be falsely elevated

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44
Q

What are the criteria for Dx’ing lupus?

A

need 4/11: 1. malar rash 2. discoid rash 3. photosensitivity 4. oral ulcers 5. arthritis 6. serositis 7. cerbritis/CNS involvement 8. renal disease 9. “Penias” 10. ANA 11. anti-dsDNA/anti-Sm/anti-phospholipid

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45
Q

what drugs cause drug-induced lupus?

A

SHIPP sulfonamide hydralazine INH (isoniazid) procainamide

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46
Q

isoniazid is used for what?

A

tuberculosis

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47
Q

what symptoms will you see in drug induced lupus?

A

rash arthritis penias ANA+

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48
Q

what antibodies are elevated in drug induced lupus?

A

anti-histone antibodies

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49
Q

How is SLE treated?

A

steroids other immunosuppressants avoid sunlight

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50
Q

what are causes of death in SLE?

A

renal failure infection (immunosuppression drugs) coronary disease (SLE –> increased risk)

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51
Q

what is neonatal lupus?

A

maternal Ab’s cross the placenta and affect the fetus

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52
Q

how often does neonatal lupus occur?

A

1-2% of babies born to moms with an autoimmune disease

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53
Q

what other autoimmune disease can you see neonatal lupus?

A

Sjogren’s

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54
Q

Why is Sjogren’s syndrome common among mothers?

A

the SSA/Ro or SSB/La antibodies are believed to be the ones that lead to this condition in the fetus and these are the antibodies that are elevated in Sjogren’s syndrome but can also be seen in SLE

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55
Q

elevated levels of what maternal antibodies puts a fetus at risk for neonatal lupus?

A

SSA/Ro SSB/La

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56
Q

when do the Sx of neonatal lupus present?

A

at birth or first few weeks of life

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57
Q

what is the characteristic feature of neonatal lupus?

A

Rash - multiple red, circular lesions on their face and scalp

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58
Q

what serious complication does neonatal lupus cause?

A

congenital complete heart block

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59
Q

rheumatoid arthritis most commonly affects what kind of joints?

A

synovial

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60
Q

what is the synovium and how is it involved in the pathophysiology of RA?

A

thin layer of tissue lines joints and tendon sheaths secretes hyaluronic acid to lubricate joint space

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61
Q

describe the pathophysiology of RA

A

inflammation of the synovium by an unknown trigger causing an overproduction of cytokines TNF and IL-6

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62
Q

what is synovial hypertrophy?

A

as a result of inflammation of the synovium, it thickens into a pannus

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63
Q

what is a pannus?

A

synovium that has been infiltrated with inflammatory cells and granulation tissue

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64
Q

what is characteristic of a pannus?

A

increase in synovial fluid

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65
Q

How can a pannus destroy a joint?

A

it can erode into the cartilage and bone

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66
Q

What type of HS is RA?

A

type III Ab-mediated

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67
Q

RA vs OA

A

RA - symmetric joint inflammation joint stiffness lasts more than 1hr after rising Sx improve with use systemic Sx (fever) affects MCP and PIP joints OA - starts with one side before moving to the next joint stiffness lasts less than 1hr after rising Sx worsen with use no systemic Sx affects the DIP joints

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68
Q

Sx of RA

A

gradual onset of pain, stiffness, swelling “morning stiffness” systemic Sx (fever)

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69
Q

morning stiffness is a common complaint of patients with what autoimmune disorder?

A

rheumatoid arthritis

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70
Q

what is “morning stiffness”

A

joint stiffness > 1hr after rising that improves with use

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71
Q

How does OA differ from RA?

A

morning stiffness in OA resolves in < 1hr and gets worse with use No systemic Sx seen in OA

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72
Q

what joints of the hand are spared in RA?

A

DIP joints

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73
Q

what joints of the hand are affected in RA?

A

MCP AND PIP

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74
Q

before RA could be treated how did it progress?

A

bones eroded and become deviated and very deformed

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75
Q

what are the 2 classic sequela of RA left untreated?

A

ulnar deviation swan neck deformity

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76
Q

why does ulnar deviation occur?

A

swelling of the MCP joints near the wrist causes the wrist to deviate towards the ulnar side

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77
Q

why does the swan neck deformity occur?

A

hyperextended PIP joint w/ flexed DIP joint

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78
Q

what are the systemic complications of RA?

A

baker’s cyst

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79
Q

what is a baker’s cyst?

A

aka popliteal cyst synovium-lined sac at back of knee that is continuous with the joint space can become swollen

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80
Q

where do you find baker’s cyst?

A

back of the knee in the popliteal fossa

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81
Q

what happens if a baker’s cyst ruptures?

A

pain all down the leg, mimics DVT

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82
Q

how does a baker’s cyst present?

A

pain and swelling in the back of the knee

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83
Q

what are subcutaneous nodules?

A

aka rheumatoid nodules palpable nodules seen in 20-35% of patients

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84
Q

Patients with RA that develop subcutaneous nodules are most likely positive for what?

A

Rheumatoid factor

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85
Q

what is the pathology of subcutaneous nodules?

A

central necrosis surrounded by macrophages/lymphocytes

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86
Q

how does RA affect the eye?

A

episcleritis scleritis uveitis

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87
Q

what is episcleritis?

A

inflammation of the episclera (surface layer of the eye) painful, red eye without discharge

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88
Q

what is scleritis?

A

often bilateral inflammation of the sclera dark, red eyes deep ocular pain on eye mvmt

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89
Q

why does it hurt to move your eyes in scleritis?

A

eye muscled inter onto the sclera so when that is inflamed it hurts to move your eyes

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90
Q

What is uveitis?

A

inflammation of the uvea - vascular layer of the eye

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91
Q

what is the difference between anterior and posterior uveitis?

A

posterior - floaters and vision loss anterior - similar to scleritis or episcelitis

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92
Q

what syndrome is commonly developed 2º to RA?

A

Sjogren’s syndrome

93
Q

what is Sjogren’s syndrome?

A

autoimmune disease that causes damage to the salivary and lacrimal glands

94
Q

what are the Sx of Sjogren’s?

A

dry eyes, dry mouth (sicca symptoms) sicca = dryness

95
Q

what 2 autoimmune diseases are commonly associated with Sjogren’s syndrome?

A

RA SLE

96
Q

what makes osteoporosis worse?

A

RA steroid treatment

97
Q

why is osteoporosis a big problem for women especially who have RA?

A

osteoporosis is accelerated by RA steroid treatment for RA worsens osteoporosis

98
Q

people with RA are at an increased risk for what?

A

30% increased risk of major fracture 40% increased risk of hip fracture

99
Q

what Ab’s are used to Dx RA?

A

rheumatoid factor Ab seen in 80% of pts antibodies to citrullinated peptides (ACPA) are specific for RA

100
Q

what is the RF antibody directed against?

A

Fc portion of IgG Ab

101
Q

what is “seropositive” RA?

A

people with RA who are positive for RF (80% of patients)

102
Q

what is the problem with the RF test?

A

poor specificity - positive in multiple conditions

103
Q

what conditions show a positive RF?

A

RA endocarditis Hep B Hep C Sjogren’s SLE

104
Q

what are citrullinated peptides?

A

peptides that have had citrulline added to them

105
Q

what is the specific marker for RA often used to confirm Dx?

A

ACPA - antibodies to citrullinated peptides

106
Q

what is the target of the rheumatoid factor (RF) antibody?

A

the Fc portion of IgG

107
Q

what is citrulline?

A

non-standard amino acid - not encoded by our genome intermediate of the urea cycle

108
Q

how does citrulline get into proteins?

A

post-translational modification of protein structures derived from our genome

109
Q

why is anti-citrulline peptide antibodies used in RA?

A

citrulline is incorporated into more proteins during inflammation especially RA up to 80% of patients w/ RA

110
Q

what HLA serotype is RA associated with?

A

HLA-DR4

111
Q

what is the treatment for RA?

A

NSAIDS steroids DMARDS

112
Q

What are DMARDS?

A

disease modifying anti rheumatic drugs have been shown in clinical trials to protect joints from joint destruction

113
Q

what drugs are considered DMARDS?

A

MTX azathioprine cyclosporine hydroxychloroquine sulfasalazine leflunomide TNF-α inhibitors (Ab’s α TNF-α)

114
Q

When sulfasalazine enters the colon what happens?

A

colonic bacteria split it into sulfapyridine and 5-aminosalicylic acid (5-ASA)

115
Q

The structure and function of 5-ASA is similar to what other molecule?

A

acetylsalicylic acid aka aspirin

116
Q

why is sulfasalazine used as an anti-inflammatory drug?

A

during the breakdown of it, 5-ASA is formed which acts like aspiring to reduce inflammation

117
Q

what is the active form of sulfasalazine?

A

5-ASA

118
Q

what is leflunomide?

A

immunosuppressant used in RA and psoriatic arthritis

119
Q

what is the MOA of leflunomide?

A

inhibits dihydroorotate dehydrogenase and therefore pyrimidine synthesis

120
Q

what rxn does dihydroorotate dehydrogenase catalyze?

A

dihydroorotic acid –> orotic acid

121
Q

what are the S/E’s of Leflunomide?

A

diarrhea abnormal LFTs low WBCs

122
Q

How does leflunomide suppress the immune system?

A

orotic acid will be low causing less UMP and therefore CMP and TMP will be low (pyrimidines)

123
Q

what is infliximab and what is it used to treat?

A

antibody against TNF-α RA and Chron’s

124
Q

what is an important risk for people taking infliximab?

A

risk of reactivation of TB PPD done before treatment

125
Q

before prescribing infliximab, what test should be done?

A

PPD

126
Q

what other infections are people who take infliximab at an increased risk for?

A

bacterial infections reactivation of herpes zoster

127
Q

what are other TNF-α inhibitors?

A

adalimumab golimumab etanercept

128
Q

what is different about the TNF-α inhibitor etanercept?

A

it is a recombinant protein of TNF-R “decoy receptor” binds TNF instead of TNF-R

129
Q

what are long term complications of RA?

A

increased risk for coronary disease - leading cause of death secondary (AA) amyloidosis

130
Q

apple green bifringence

A

amyloid

131
Q

what is Felty syndrome?

A

a rare complication of RA triad: RA, splenomegaly, neutropenia usually pt has severe RA - joint deformity, extra-articular disease improves with RA therapy

132
Q

how can you map a bacterial genome?

A

interrupt conjugation of high frequency strains at various time intervals to see which genetic material has transferred

133
Q

what is transduction?

A

transfer of DNA by a bacteriophage; virus picks up DNA and transfers it to another bacteria

134
Q

what is a bacteriophage?

A

virus that infects bacteria

135
Q

what are the 2 subtypes of transduction?

A

generalized specialized

136
Q

what is generalized transduction?

A

virus infects bacteria and multiplies inside the bacteria and randomly picks up host DNA and the host DNA gets transferred to other bacteria

137
Q

what is specialized transduction?

A

transfer of specific genes from one bacteria to another

138
Q

MOA of specialized transduction?

A

virus DNA inserts into host DNA (lysogeny) viral DNA lives in bacterial cell genome and later the bacteriophage DNA is excised and packaged into a virus with specific host DNA

139
Q

where does the specific host DNA that is packaged into virus during transduction come from?

A

the DNA that sat right next to the viral DNA when it was incorporated it is this host DNA that then gets transferred to other cells via viral infection vector

140
Q

what are the 2 ways that transduction can happen? (2 different bacteriophages)

A

lytic or lysogenic

141
Q

what is the lytic cycle of transduction?

A

generalized transduction nuclear material enters bacteria and multiplies, cell lyses and releases progeny viruses

142
Q

what is the lysogenic cycle of transduction?

A

nuclear material enters cell from virus and incorporates into host DNA may later become excised (enter lytic phase)

143
Q

What are phages that replicate only via the lytic cycle called?

A

virulent phages

144
Q

What are phages that replicate via both the lytic and lysogenic cycles called?

A

temperate phages

145
Q

virulent phages

A

phages that replicate only via the lytic cycle

146
Q

temperate phages

A

phages that replicate via both the lytic and lysogenic cycles

147
Q

how do lytic phages operate?

A

virus infects bacteria and uses cellular machinery to reproduce then lyses the cell usually generalized gene transfer

148
Q

how do lysogenic phages operate?

A

virus infects bacteria and incorporates phage DNA into bacterial DNA can remain dormant for long periods of time and then can be triggered by something (UV light) to induce genome excision resulting in a lytic cycle and release of phage particles

149
Q

what is a prophage?

A

virus puts genetic material into bacteria and that gets inserted into the bacterial genome mechanism by which a new bacteria can acquire abilities that it didn’t previously have

150
Q

why does lysogeny matter in medicine?

A

because the genes for some bacterial toxins are transferred to non-toxic strains via lysogeny (i.e. C. diphtheria)

151
Q

how does a corynebacteria cell become toxic?

A

it has to be infected by a phage (corynephage) which imparts the ability to synthesize the toxin and then the diphtheria bacterial cell will become toxic

152
Q

Is the gene for diphtheria toxin part of the bacterias normal genome?

A

no

153
Q

what are the main lysogenic toxins? (toxins that are imparted by viruses to bacteria via lysogeny)

A

diphtheria toxin erythrogenic toxin (S. pyogenes; Scarlet fever) shiga-like toxin (E. coli; EHEC) cholera toxin botulinum toxin

154
Q

what are transposons?

A

DNA segments within bacterial DNA that can be excised and reintegrated in new locations in DNA or sometimes moved to a plasmid (mechanism of transfer of resistance to antibiotics)

155
Q

what is the mechanism of transfer of resistance to antibiotics?

A

bacteria #1 is resistant and the transposon segment carries the resistance gene which can jump around the genome and potentially be moved to a plasmid, the plasmid can then be transferred to other bacteria

156
Q

what is scleroderma?

A

systemic autoimmune disorder that causes stiff, hardened tissue (sclerosis)

157
Q

what causes scleroderma?

A

endothelial cell damage (trigger unclear) causes Ab’s to be produced and cytokines to be released resulting in tissue damage

158
Q

what becomes over activated in scleroderma?

A

fibroblasts (unusual for an autoimmune disorder) causes collagen deposition = sclerosis

159
Q

what are the 2 clinical syndromes that scleroderma can present as?

A

diffuse scleroderma limited scleroderma (CREST)

160
Q

what is diffuse scleroderma?

A

skin thickening throughout the body

161
Q

what commonly presents prior to a diagnosis of scleroderma?

A

raynauds phenomenon can present by itself for some time and scleroderma will develop later

162
Q

what is Raynaud’s phenomenon?

A

vasoconstriction & ischemia of the hands

163
Q

why is diffuse scleroderma so serious?

A

early involvement of visceral organs

164
Q

what complications arise in diffuse scleroderma

A

renal disease - renal failure GI - dysmotility, heartburn <3 - pericarditis, myocarditis, conduction disease (L/R BBB, AV node block) joints/muscles - arthralgia, myalgias pulmonary HTN interstitial lung disease

165
Q

what is the most lethal feature of scleroderma?

A

pulmonary HTN

166
Q

why is pulmonary HTN so lethal?

A

bc it leads to R <3 failure

167
Q

How will a pt present with RHF?

A

RV heave elevated jugular veins pitting edema

168
Q

how are patients with pulmonary HTN monitored?

A

echocardiography - allows you to estimate the pulmonary P

169
Q

why are pts w/ pulmonary HTN monitored?

A

early & aggressive treatment can be life saving

170
Q

what is interstitial lung disease?

A

when fibrous tissue develops in the lungs

171
Q

what is scleroderma renal crisis?

A

life threatening complication of diffuse scleroderma acute worsening of renal function often early in the disease

172
Q

how will a pt with scleroderma renal crisis present?

A

feeling very unwell w/ marked HTN

173
Q

what is the treatment of choice for scleroderma renal crisis?

A

ACE inhibitors

174
Q

before the development of _______ scleroderma renal crisis was fatal

A

ACE inhibitors

175
Q

what is limited scleroderma?

A

CREST syndrome sclerosis is limited to the hands, sometimes distal forearm, face or neck

176
Q

CREST syndrome

A

5 Sx of limited scleroderma: Calcinosis Raynauds phenomenon Esophageal dysmotility Sclerodactyly Telangiectasias

177
Q

Crest syndrome

A

Calcinosis calcium deposits in subcutaneous tissue bumps on elbows, knees and fingers can break skin and leak white fluid x-rays of hands may show soft tissue calcifications

178
Q

cRest syndrome

A

Raynauds phenomenon white/blue fingertips painful on exposure to cold - vessels constrict more vasospasm of the artery –> ischemia can lead to fingertip ulcers often the 1st Sx of scleroderma

179
Q

crEst syndrome

A

Esophageal dysmotility difficulty swallowing from collagen deposition in the esophagus reflux/heartburn from LES hypotonia

180
Q

What is a characteristic feature of esophageal dysmotility in scleroderma?

A

LES hypotonia

181
Q

what is the LES

A

muscular sphincter on the bottom of the esophagus that keeps the esophagus tight so that stomach acids don’t reflux up into the esophagus

182
Q

How does scleroderma cause LES hypotonia?

A

collagen deposition disrupts the muscular fibers of the sphincter allowing stomach acids to reflux back into the esophagus

183
Q

how can you measure LES tonicity?

A

esophageal manometry

184
Q

On esophageal manometry, a pt w/ esophageal dysmotility from scleroderma will show ________, in contrast, _______ will show LES hypertonia

A

LES hypotonia achalasia

185
Q

creSt syndrome

A

Sclerodactyly fibrosis of the hands begins as puffy fingers that are hard to bend later, skin often becomes shiny skin thickened skin (can’t pinch) loss of wrinkles severe: hands feel like claws/wooden

186
Q

cresT syndrome

A

Telangiectasias skin lesions caused by dilated capillaries face hands mucous membranes (inside mouth on cheeks)

187
Q

what is the main source of mortality for diffuse scleroderma?

A

heart & kidneys which are rarely involved in limited

188
Q

what is the main risk for ppl with CREST syndrome?

A

pulmonary disease - leading cause of death pulmonary HTN interstitial lung disease

189
Q

what antibodies are used to Dx scleroderma?

A

Anti-nuclear antibody (ANA) - not specific Anti-topoisomerase I (anti-Scl-70) Ab - diffuse Anti-centromere antibody - limited Anti-RNA polymerase III Ab - diffuse

190
Q

what is the anti-centromere Ab?

A

specific for CREST scleroderma (think Crest = Centromere)

191
Q

anti-Scl-70

A

aka anti-topoisomerase I indicates diffuse scleroderma

192
Q

anti-RNA polymerase III Ab

A

indicates diffuse scleroderma associated with rapidly progressive skin involvement

193
Q

People with diffuse scleroderma will have an increased risk for scleroderma renal crisis if they are positive for what Ab?

A

anti-RNA polymerase III

194
Q

how is scleroderma treated?

A

treat the Sx GI tract (esophagus) - PPI to treat the heartburn Raynauds - Calcium Ch blocker Pulm - Pulmonary HTN drugs

195
Q

what is different about the treatment of scleroderma as an autoimmune disease?

A

it is rarely treated with anti-inflammatory or immunosuppressant drugs treatment is aimed at the organ system affected

196
Q

What other autoimmune disease is associated with primary biliary cirrhosis?

A

**scleroderma - high yield to know they are related Sjogren’s - up to 1/2 of pts w/ PBC SLE RA hashimotos thyroiditis

197
Q

5-15% of patients with _________ will also have scleroderma

A

primary biliary cirrhosis

198
Q

what is primary biliary cirrhosis? presentation? complications?

A

T-cell destruction of small bile ducts jaundice, fatigue, itching cirrhosis and liver failure

199
Q

what labs are elevated in primary biliary cirrhosis?

A

conjugated bilirubin alkaline phosphatase (ALP)

200
Q

What is Sjogren’s syndrome?

A

autoimmune disorder resulting in destruction of salivary and lacrimal glands

201
Q

What are the clinical features of Sjogren’s syndrome?

A

keratoconjunctivitis sicca xerostomia

202
Q

keratoconjunctivitis sicca

A

dry eyes from AI destruction of lacrimal glands “dryness of the surface of the eye” presents as feeling dirt/debris in eyes

203
Q

xerostomia

A

dry mouth difficulty chewing dry foods cavities bad breath

204
Q

what are the extra glandular Sx of Sjogren’s syndrome?

A

xerosis - dry, scaly skin arthralgias/arthritis raynauds

205
Q

Why can’t you make a diagnosis of Sjogren’s based on symptoms alone?

A

many elderly patients have “sicca symptoms” dry mouth/eyes due to aging Ab test and Bx will come back normal

206
Q

what are “sicca symptoms”

A

dry eyes & mouth from aging, presents the same as Sjogren’s syndrome w/o + Ab/Bx results

207
Q

What type of HS is Sjogren’s syndrome?

A

type IV lymphocyte mediated

208
Q

lymphocytic sialadenitis

A

lymphocytic infiltration of the salivary gland seen in Sjogren’s syndrome **BUZZWORD**

209
Q

what other autoimmune diseases is Sjogren’s syndrome associated with?

A

RA SLE PBC

210
Q

40-65% or patients with ___________ have Sjogren’s syndrome

A

PBC - AI destruction of the bile ducts primary biliary cirrhosis

211
Q

What antibodies are seen in Sjogren’s syndrome?

A

ANA - not specific but a negative result makes Dx less likely rheumatoid factor - seen in 1º and 2º anti-SS-A (Ro) anti-SS-B (La)

212
Q

what antibodies are associated with neonatal lupus?

A

anti-SS-A (Ro) anti-SS-B (La)

213
Q

what is the schirmer test and how is it done?

A

tests reflex tear production 1. filter paper is placed near lower eyelid 2. pt closes eye 3. measure amt of wetting over 5 min

214
Q

What tests are done for salivary testing?

A

salivary gland scintigraphy whole sialometry

215
Q

salivary gland scintigraphy

A

nuclear medicine test where you administer a radio tracer and look at the uptake of the radio tracer by the salivary glands

216
Q

what would you see on salivary gland scintigraphy in a pt w/ Sjogren’s syndrome?

A

if the salivary glands have been destroyed by an AI process there will be low uptake of the radio-tracer

217
Q

whole sialometry

A

measurement of saliva production pt collects all saliva over 15 minutes sample weighed

218
Q

what is the criteria to Dx Sjogren’s syndrome?

A

need 4/6 eye Sx oral Sx ocular signs (Schirmer test) oral signs (salivary testing) Bx: lymphocytic sialadenitis anti-SSa or anti-SSb

219
Q

what is the treatment for Sjogren’s syndrome?

A

good oral hygiene artificial saliva Muscarinic agonists: pilocarpine steroids for extraglandular disease

220
Q

MOA pilocarpine

A

stimulates Muscarinic Ach-R’s which will increase flow of fluid from the salivary glands and the lacrimal ducts treats SS

221
Q

what is a serious complication of Sjogren’s syndrome?

A

B cell lymphoma develops from the chronic inflammation of glandular tissue

222
Q

patients with Sjogren’s have increased risk for what cancer?

A

B cell lymphoma

223
Q

how would B cell lymphoma present in someone who has Sjogren’s?

A

persistent unilateral swollen gland may mimic past swelling that resembles a flare but persistent and larger

224
Q

There is an increased risk that a fetus will get neonatal lupus if the mom has what? What is this due to?

A

an autoimmune disease, mainly Sjogren’s/SLE maternal Ab’s cross the placenta

225
Q

what Ab’s are associated with neonatal lupus? What other autoimmune disease are they associated with?

A

+SSA/Ro +SSB/La Sjogren’s syndrome Sometimes lupus

226
Q

If a pregnant woman with SLE is +SSA-Ro or +SSB-La, what is their baby at risk of developing?

A

neonatal lupus

227
Q

S/Sx neonatal lupus

A

shows at birth or 1st few weeks of life rash - 1st symptom, most noticeable congenital complete heart block - bradycardia 50bpm

228
Q

How does the rash seen in neonatal lupus differ from the butterfly rash seen in SLE?

A

multiple red, circular lesions on face and scalp

229
Q

if a baby is born with neonatal lupus and it is suspected that there is a congenital complete heart block, what will the treatment be?

A

the baby will need a pace maker for the rest of their life

*med3* (21 decks)

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