Immunology - Autoimmune Disorders Flashcards
(229 cards)
1
Q
what type of HS is SLE?
A
type III
2
Q
Why is C3/C4 low in SLE?
A
Ab-Ag complexes activate complement
supply < demand => hypocomplementemia
3
Q
what antibodies cause lupus?
A
anti-nuclear antibodies (ANA’s)
anti-double stranded DNA (anti-dsDNA)
anti-smith (anti-Sm)
4
Q
ANA is present in the serum of _____ patients and in __% of normal patients
A
lupus
5
5
Q
Is ANA sensitive or specific?
A
sensitive, meaning a negative result indicates the disease is very unlikely
6
Q
why isn’t ANA specific for lupus?
A
because it is found in many other autoimmune disorders
7
Q
what is considered a normal ANA?
A
< 1:160
8
Q
what 2 antibodies are specific for SLE?
A
anti-dsDNA anti-Sm
9
Q
what Ab can be used to monitor disease activity in SLE?
A
anti-dsDNA will increase during flares
10
Q
what Ab is associated with renal involvement in SLE?
A
anti-dsDNA = glomerulonephritis
11
Q
what is anti-Sm directed against?
A
small nuclear ribonucleoproteins (snRNPs)
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
13
Q
what are RNA transcripts?
A
pieces of RNA that have been synthesized from DNA
14
Q
what are snRNPs used for?
A
modify RNA before it is translated into a protein
15
Q
antibodies against snRNPs are seen in what?
A
SLE
16
Q
anti-Sm antibodies are directed against what?
A
snRNPs
17
Q
what is an ENA panel?
A
extractable nuclear antigens panel of blood tests against nuclear antigens
18
Q
when would you order an ENA panel?
A
if you suspect an autoimmune disease
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
20
Q
anti-RNP
A
MCTD SLE scleroderma
21
Q
anti-Sm
A
specific for lupus
22
Q
anti-SS-A (Ro)
A
Sjogren’s syndrome SLE scleroderma
23
Q
anti-SS-B (La)
A
Sjogren’s syndrome SLE scleroderma
24
Q
scl-70
A
specific for scleroderma
25
anti-Jo-1
polymyositis
26
SLE flare Sx
fever weight loss fatigue LAD
27
what causes Raynaud's?
vasospasm in arteries supplying blood to the fingertips causing ischemia
28
what other Sx are seen in SLE?
oral/nasal ulcers arthritis serositis "penias" - anemia, thrombocytopenia, leukopenia - results from antibody attack of cells - type II HS where Ab directly attacks the cell
29
what is lupus cerebritis?
lupus affecting the brain resulting in cognitive dysfunction (confusion, memory loss) strokes seizures
30
lupus nephropathy presents as what syndrome?
nephritic or nephrotic syndrome or both
31
what is the most common lupus renal syndrome?
diffuse proliferative glomerular nephritis
32
what syndrome does diffuse proliferative glomerular nephritis present as?
nephritic syndrome
33
what syndrome dose membranous glomerular nephritis present as?
nephrotic syndrome
34
What is Libman-Sacks endocarditis?
aka marantic endocarditis nonbacterial inflammation of valves \*\*\*classically affects both sides of mitral valve
35
What is the main difference between Libman-Sacks and bacterial endocarditis?
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
36
anti-phospholipid antibodies occur in association with what?
lupus nb: can also occur as a primary problem
37
what are the 3 important clinical consequences of anti-phospholipid antibodies?
"antiphospholipid syndrome" - blood clots increased PTT false positive syphilis (RPR/VDRL)
38
what are anti-phospholipid antibodies?
Ab's against proteins in phospholipids
39
what is antiphospholipid syndrome?
pt w/ antiphospholipid Ab's develops venous or arterial thrombosis causing DVTs, stroke, fetal loss
40
what are the 3 clinically relevant antiphospholipid antibodies?
anti-cardiolipin "lupus anticoagulant' anti-ß2 glycoprotein
41
what does anti-cardiolipin give a false positive for?
RPR/VDRL syphilis
42
why does anti-cardiolipin give a false positive for syphilis?
syphilis produces antibodies against the RPR/VDRL antigens but the anti-cardiolipin antibody also reacts to the RPR/VDRL antigens
43
what test does the lupus anticoagulant interfere with?
PTT test will be falsely elevated
44
What are the criteria for Dx'ing lupus?
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
45
what drugs cause drug-induced lupus?
SHIPP sulfonamide hydralazine INH (isoniazid) procainamide
46
isoniazid is used for what?
tuberculosis
47
what symptoms will you see in drug induced lupus?
rash arthritis penias ANA+
48
what antibodies are elevated in drug induced lupus?
anti-histone antibodies
49
How is SLE treated?
steroids other immunosuppressants avoid sunlight
50
what are causes of death in SLE?
renal failure infection (immunosuppression drugs) coronary disease (SLE --\> increased risk)
51
what is neonatal lupus?
maternal Ab's cross the placenta and affect the fetus
52
how often does neonatal lupus occur?
1-2% of babies born to moms with an autoimmune disease
53
what other autoimmune disease can you see neonatal lupus?
Sjogren's
54
Why is Sjogren's syndrome common among mothers?
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
55
elevated levels of what maternal antibodies puts a fetus at risk for neonatal lupus?
SSA/Ro SSB/La
56
when do the Sx of neonatal lupus present?
at birth or first few weeks of life
57
what is the characteristic feature of neonatal lupus?
Rash - multiple red, circular lesions on their face and scalp
58
what serious complication does neonatal lupus cause?
congenital complete heart block
59
rheumatoid arthritis most commonly affects what kind of joints?
synovial
60
what is the synovium and how is it involved in the pathophysiology of RA?
thin layer of tissue lines joints and tendon sheaths secretes hyaluronic acid to lubricate joint space
61
describe the pathophysiology of RA
inflammation of the synovium by an unknown trigger causing an overproduction of cytokines TNF and IL-6
62
what is synovial hypertrophy?
as a result of inflammation of the synovium, it thickens into a pannus
63
what is a pannus?
synovium that has been infiltrated with inflammatory cells and granulation tissue
64
what is characteristic of a pannus?
increase in synovial fluid
65
How can a pannus destroy a joint?
it can erode into the cartilage and bone
66
What type of HS is RA?
type III Ab-mediated
67
RA vs OA
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
68
Sx of RA
gradual onset of pain, stiffness, swelling "morning stiffness" systemic Sx (fever)
69
morning stiffness is a common complaint of patients with what autoimmune disorder?
rheumatoid arthritis
70
what is "morning stiffness"
joint stiffness \> 1hr after rising that improves with use
71
How does OA differ from RA?
morning stiffness in OA resolves in \< 1hr and gets worse with use No systemic Sx seen in OA
72
what joints of the hand are spared in RA?
DIP joints
73
what joints of the hand are affected in RA?
MCP AND PIP
74
before RA could be treated how did it progress?
bones eroded and become deviated and very deformed
75
what are the 2 classic sequela of RA left untreated?
ulnar deviation swan neck deformity
76
why does ulnar deviation occur?
swelling of the MCP joints near the wrist causes the wrist to deviate towards the ulnar side
77
why does the swan neck deformity occur?
hyperextended PIP joint w/ flexed DIP joint
78
what are the systemic complications of RA?
baker's cyst
79
what is a baker's cyst?
aka popliteal cyst synovium-lined sac at back of knee that is continuous with the joint space can become swollen
80
where do you find baker's cyst?
back of the knee in the popliteal fossa
81
what happens if a baker's cyst ruptures?
pain all down the leg, mimics DVT
82
how does a baker's cyst present?
pain and swelling in the back of the knee
83
what are subcutaneous nodules?
aka rheumatoid nodules palpable nodules seen in 20-35% of patients
84
Patients with RA that develop subcutaneous nodules are most likely positive for what?
Rheumatoid factor
85
what is the pathology of subcutaneous nodules?
central necrosis surrounded by macrophages/lymphocytes
86
how does RA affect the eye?
episcleritis scleritis uveitis
87
what is episcleritis?
inflammation of the episclera (surface layer of the eye) painful, red eye without discharge
88
what is scleritis?
often bilateral inflammation of the sclera dark, red eyes deep ocular pain on eye mvmt
89
why does it hurt to move your eyes in scleritis?
eye muscled inter onto the sclera so when that is inflamed it hurts to move your eyes
90
What is uveitis?
inflammation of the uvea - vascular layer of the eye
91
what is the difference between anterior and posterior uveitis?
posterior - floaters and vision loss anterior - similar to scleritis or episcelitis
92
what syndrome is commonly developed 2º to RA?
Sjogren's syndrome
93
what is Sjogren's syndrome?
autoimmune disease that causes damage to the salivary and lacrimal glands
94
what are the Sx of Sjogren's?
dry eyes, dry mouth (sicca symptoms) sicca = dryness
95
what 2 autoimmune diseases are commonly associated with Sjogren's syndrome?
RA SLE
96
what makes osteoporosis worse?
RA steroid treatment
97
why is osteoporosis a big problem for women especially who have RA?
osteoporosis is accelerated by RA steroid treatment for RA worsens osteoporosis
98
people with RA are at an increased risk for what?
30% increased risk of major fracture 40% increased risk of hip fracture
99
what Ab's are used to Dx RA?
rheumatoid factor Ab seen in 80% of pts antibodies to citrullinated peptides (ACPA) are specific for RA
100
what is the RF antibody directed against?
Fc portion of IgG Ab
101
what is "seropositive" RA?
people with RA who are positive for RF (80% of patients)
102
what is the problem with the RF test?
poor specificity - positive in multiple conditions
103
what conditions show a positive RF?
RA endocarditis Hep B Hep C Sjogren's SLE
104
what are citrullinated peptides?
peptides that have had citrulline added to them
105
what is the specific marker for RA often used to confirm Dx?
ACPA - antibodies to citrullinated peptides
106
what is the target of the rheumatoid factor (RF) antibody?
the Fc portion of IgG
107
what is citrulline?
non-standard amino acid - not encoded by our genome intermediate of the urea cycle
108
how does citrulline get into proteins?
post-translational modification of protein structures derived from our genome
109
why is anti-citrulline peptide antibodies used in RA?
citrulline is incorporated into more proteins during inflammation especially RA up to 80% of patients w/ RA
110
what HLA serotype is RA associated with?
HLA-DR4
111
what is the treatment for RA?
NSAIDS steroids DMARDS
112
What are DMARDS?
disease modifying anti rheumatic drugs have been shown in clinical trials to protect joints from joint destruction
113
what drugs are considered DMARDS?
MTX azathioprine cyclosporine hydroxychloroquine sulfasalazine leflunomide TNF-α inhibitors (Ab's α TNF-α)
114
When sulfasalazine enters the colon what happens?
colonic bacteria split it into sulfapyridine and 5-aminosalicylic acid (5-ASA)
115
The structure and function of 5-ASA is similar to what other molecule?
acetylsalicylic acid aka aspirin
116
why is sulfasalazine used as an anti-inflammatory drug?
during the breakdown of it, 5-ASA is formed which acts like aspiring to reduce inflammation
117
what is the active form of sulfasalazine?
5-ASA
118
what is leflunomide?
immunosuppressant used in RA and psoriatic arthritis
119
what is the MOA of leflunomide?
inhibits dihydroorotate dehydrogenase and therefore pyrimidine synthesis
120
what rxn does dihydroorotate dehydrogenase catalyze?
dihydroorotic acid --\> orotic acid
121
what are the S/E's of Leflunomide?
diarrhea abnormal LFTs low WBCs
122
How does leflunomide suppress the immune system?
orotic acid will be low causing less UMP and therefore CMP and TMP will be low (pyrimidines)
123
what is infliximab and what is it used to treat?
antibody against TNF-α RA and Chron's
124
what is an important risk for people taking infliximab?
risk of reactivation of TB PPD done before treatment
125
before prescribing infliximab, what test should be done?
PPD
126
what other infections are people who take infliximab at an increased risk for?
bacterial infections reactivation of herpes zoster
127
what are other TNF-α inhibitors?
adalimumab golimumab etanercept
128
what is different about the TNF-α inhibitor etanercept?
it is a recombinant protein of TNF-R "decoy receptor" binds TNF instead of TNF-R
129
what are long term complications of RA?
increased risk for coronary disease - leading cause of death secondary (AA) amyloidosis
130
apple green bifringence
amyloid
131
what is Felty syndrome?
a rare complication of RA triad: RA, splenomegaly, neutropenia usually pt has severe RA - joint deformity, extra-articular disease improves with RA therapy
132
how can you map a bacterial genome?
interrupt conjugation of high frequency strains at various time intervals to see which genetic material has transferred
133
what is transduction?
transfer of DNA by a bacteriophage; virus picks up DNA and transfers it to another bacteria
134
what is a bacteriophage?
virus that infects bacteria
135
what are the 2 subtypes of transduction?
generalized specialized
136
what is generalized transduction?
virus infects bacteria and multiplies inside the bacteria and randomly picks up host DNA and the host DNA gets transferred to other bacteria
137
what is specialized transduction?
transfer of specific genes from one bacteria to another
138
MOA of specialized transduction?
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
where does the specific host DNA that is packaged into virus during transduction come from?
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
what are the 2 ways that transduction can happen? (2 different bacteriophages)
lytic or lysogenic
141
what is the lytic cycle of transduction?
generalized transduction nuclear material enters bacteria and multiplies, cell lyses and releases progeny viruses
142
what is the lysogenic cycle of transduction?
nuclear material enters cell from virus and incorporates into host DNA may later become excised (enter lytic phase)
143
What are phages that replicate only via the lytic cycle called?
virulent phages
144
What are phages that replicate via both the lytic and lysogenic cycles called?
temperate phages
145
virulent phages
phages that replicate only via the lytic cycle
146
temperate phages
phages that replicate via both the lytic and lysogenic cycles
147
how do lytic phages operate?
virus infects bacteria and uses cellular machinery to reproduce then lyses the cell usually generalized gene transfer
148
how do lysogenic phages operate?
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
what is a prophage?
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
why does lysogeny matter in medicine?
because the genes for some bacterial toxins are transferred to non-toxic strains via lysogeny (i.e. C. diphtheria)
151
how does a corynebacteria cell become toxic?
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
Is the gene for diphtheria toxin part of the bacterias normal genome?
no
153
what are the main lysogenic toxins? (toxins that are imparted by viruses to bacteria via lysogeny)
diphtheria toxin erythrogenic toxin (S. pyogenes; Scarlet fever) shiga-like toxin (E. coli; EHEC) cholera toxin botulinum toxin
154
what are transposons?
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
what is the mechanism of transfer of resistance to antibiotics?
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
what is scleroderma?
systemic autoimmune disorder that causes stiff, hardened tissue (sclerosis)
157
what causes scleroderma?
endothelial cell damage (trigger unclear) causes Ab's to be produced and cytokines to be released resulting in tissue damage
158
what becomes over activated in scleroderma?
fibroblasts (unusual for an autoimmune disorder) causes collagen deposition = sclerosis
159
what are the 2 clinical syndromes that scleroderma can present as?
diffuse scleroderma limited scleroderma (CREST)
160
what is diffuse scleroderma?
skin thickening throughout the body
161
what commonly presents prior to a diagnosis of scleroderma?
raynauds phenomenon can present by itself for some time and scleroderma will develop later
162
what is Raynaud's phenomenon?
vasoconstriction & ischemia of the hands
163
why is diffuse scleroderma so serious?
early involvement of visceral organs
164
what complications arise in diffuse scleroderma
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
what is the most lethal feature of scleroderma?
pulmonary HTN
166
why is pulmonary HTN so lethal?
bc it leads to R \<3 failure
167
How will a pt present with RHF?
RV heave elevated jugular veins pitting edema
168
how are patients with pulmonary HTN monitored?
echocardiography - allows you to estimate the pulmonary P
169
why are pts w/ pulmonary HTN monitored?
early & aggressive treatment can be life saving
170
what is interstitial lung disease?
when fibrous tissue develops in the lungs
171
what is scleroderma renal crisis?
life threatening complication of diffuse scleroderma acute worsening of renal function often early in the disease
172
how will a pt with scleroderma renal crisis present?
feeling very unwell w/ marked HTN
173
what is the treatment of choice for scleroderma renal crisis?
ACE inhibitors
174
before the development of _______ scleroderma renal crisis was fatal
ACE inhibitors
175
what is limited scleroderma?
CREST syndrome sclerosis is limited to the hands, sometimes distal forearm, face or neck
176
CREST syndrome
5 Sx of limited scleroderma: Calcinosis Raynauds phenomenon Esophageal dysmotility Sclerodactyly Telangiectasias
177
Crest syndrome
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
cRest syndrome
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
crEst syndrome
Esophageal dysmotility difficulty swallowing from collagen deposition in the esophagus reflux/heartburn from LES hypotonia
180
What is a characteristic feature of esophageal dysmotility in scleroderma?
LES hypotonia
181
what is the LES
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
How does scleroderma cause LES hypotonia?
collagen deposition disrupts the muscular fibers of the sphincter allowing stomach acids to reflux back into the esophagus
183
how can you measure LES tonicity?
esophageal manometry
184
On esophageal manometry, a pt w/ esophageal dysmotility from scleroderma will show \_\_\_\_\_\_\_\_, in contrast, _______ will show LES hypertonia
LES hypotonia achalasia
185
creSt syndrome
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
cresT syndrome
Telangiectasias skin lesions caused by dilated capillaries face hands mucous membranes (inside mouth on cheeks)
187
what is the main source of mortality for diffuse scleroderma?
heart & kidneys which are rarely involved in limited
188
what is the main risk for ppl with CREST syndrome?
pulmonary disease - leading cause of death pulmonary HTN interstitial lung disease
189
what antibodies are used to Dx scleroderma?
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
what is the anti-centromere Ab?
specific for CREST scleroderma (think Crest = Centromere)
191
anti-Scl-70
aka anti-topoisomerase I indicates diffuse scleroderma
192
anti-RNA polymerase III Ab
indicates diffuse scleroderma associated with rapidly progressive skin involvement
193
People with diffuse scleroderma will have an increased risk for scleroderma renal crisis if they are positive for what Ab?
anti-RNA polymerase III
194
how is scleroderma treated?
treat the Sx GI tract (esophagus) - PPI to treat the heartburn Raynauds - Calcium Ch blocker Pulm - Pulmonary HTN drugs
195
what is different about the treatment of scleroderma as an autoimmune disease?
it is rarely treated with anti-inflammatory or immunosuppressant drugs treatment is aimed at the organ system affected
196
What other autoimmune disease is associated with primary biliary cirrhosis?
\*\*scleroderma - high yield to know they are related Sjogren's - up to 1/2 of pts w/ PBC SLE RA hashimotos thyroiditis
197
5-15% of patients with _________ will also have scleroderma
primary biliary cirrhosis
198
what is primary biliary cirrhosis? presentation? complications?
T-cell destruction of small bile ducts jaundice, fatigue, itching cirrhosis and liver failure
199
what labs are elevated in primary biliary cirrhosis?
conjugated bilirubin alkaline phosphatase (ALP)
200
What is Sjogren's syndrome?
autoimmune disorder resulting in destruction of salivary and lacrimal glands
201
What are the clinical features of Sjogren's syndrome?
keratoconjunctivitis sicca xerostomia
202
keratoconjunctivitis sicca
dry eyes from AI destruction of lacrimal glands "dryness of the surface of the eye" presents as feeling dirt/debris in eyes
203
xerostomia
dry mouth difficulty chewing dry foods cavities bad breath
204
what are the extra glandular Sx of Sjogren's syndrome?
xerosis - dry, scaly skin arthralgias/arthritis raynauds
205
Why can't you make a diagnosis of Sjogren's based on symptoms alone?
many elderly patients have "sicca symptoms" dry mouth/eyes due to aging Ab test and Bx will come back normal
206
what are "sicca symptoms"
dry eyes & mouth from aging, presents the same as Sjogren's syndrome w/o + Ab/Bx results
207
What type of HS is Sjogren's syndrome?
type IV lymphocyte mediated
208
lymphocytic sialadenitis
lymphocytic infiltration of the salivary gland seen in Sjogren's syndrome \*\*BUZZWORD\*\*
209
what other autoimmune diseases is Sjogren's syndrome associated with?
RA SLE PBC
210
40-65% or patients with ___________ have Sjogren's syndrome
PBC - AI destruction of the bile ducts primary biliary cirrhosis
211
What antibodies are seen in Sjogren's syndrome?
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
what antibodies are associated with neonatal lupus?
anti-SS-A (Ro) anti-SS-B (La)
213
what is the schirmer test and how is it done?
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
What tests are done for salivary testing?
salivary gland scintigraphy whole sialometry
215
salivary gland scintigraphy
nuclear medicine test where you administer a radio tracer and look at the uptake of the radio tracer by the salivary glands
216
what would you see on salivary gland scintigraphy in a pt w/ Sjogren's syndrome?
if the salivary glands have been destroyed by an AI process there will be low uptake of the radio-tracer
217
whole sialometry
measurement of saliva production pt collects all saliva over 15 minutes sample weighed
218
what is the criteria to Dx Sjogren's syndrome?
need 4/6 eye Sx oral Sx ocular signs (Schirmer test) oral signs (salivary testing) Bx: lymphocytic sialadenitis anti-SSa or anti-SSb
219
what is the treatment for Sjogren's syndrome?
good oral hygiene artificial saliva Muscarinic agonists: pilocarpine steroids for extraglandular disease
220
MOA pilocarpine
stimulates Muscarinic Ach-R's which will increase flow of fluid from the salivary glands and the lacrimal ducts treats SS
221
what is a serious complication of Sjogren's syndrome?
B cell lymphoma develops from the chronic inflammation of glandular tissue
222
patients with Sjogren's have increased risk for what cancer?
B cell lymphoma
223
how would B cell lymphoma present in someone who has Sjogren's?
persistent unilateral swollen gland may mimic past swelling that resembles a flare but persistent and larger
224
There is an increased risk that a fetus will get neonatal lupus if the mom has what? What is this due to?
an autoimmune disease, mainly Sjogren's/SLE maternal Ab's cross the placenta
225
what Ab's are associated with neonatal lupus? What other autoimmune disease are they associated with?
+SSA/Ro +SSB/La Sjogren's syndrome Sometimes lupus
226
If a pregnant woman with SLE is +SSA-Ro or +SSB-La, what is their baby at risk of developing?
neonatal lupus
227
S/Sx neonatal lupus
shows at birth or 1st few weeks of life rash - 1st symptom, most noticeable congenital complete heart block - bradycardia 50bpm
228
How does the rash seen in neonatal lupus differ from the butterfly rash seen in SLE?
multiple red, circular lesions on face and scalp
229
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?
the baby will need a pace maker for the rest of their life
