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MSK > Sjogren's Syndrome, SSc, MCTD and APS > Flashcards

Sjogren's Syndrome, SSc, MCTD and APS Flashcards

1
Q

Sjogrens syndrome is an autoimmune condition characterised by ____________ infiltrates in ________ organs

A

Sjogrens syndrome is an autoimmune condition characterised by LYMPHOCYTIC infiltrates in EXOCRINE organs

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2
Q

what typical things does Sjogrens cause?

A 
eye and mouth dryness (sicca)
arthralgia
fatigue
vaginal dryness
parotid gland swelling
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3
Q

Sjogrens can occur secondary to what 2 other autoimmune conditions?

A

RA and SLE

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4
Q

peripheral neuropathy and interstitial lung disease may occur in those with Sjogrens

true or false?

A

true

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5
Q

what is there an increased risk of in those with Sjogrens?

A

lymphoma

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6
Q

name the test for ocular dryness

A

schirmers test

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7
Q

what biopsy is performed to diagnose Sjogrens?

A

lip biopsy

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8
Q

name the 2 antibodies found in Sjogrens

A

anti-Ro and anti-La

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9
Q

ocular dryness, oral dryness, ocular signs, oral signs, positive minor salivary gland biopsy and positive anti-Ro and anti-La are all diagnostic of what?

A

Sjogrens

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10
Q

Sjogrens treatment?

A 
lubricating eyedrops
saliva replacement products
dental care
pilocarpine
hydroxychloroquine
immunosuppression in organ involvement
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11
Q

name the systemic connective tissue disease that is characterised by vasomotor disturbances (Raynauds), fibrosis and subsequent atrophy of the skin and subcutaneous tissue

A

systemic sclerosis

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12
Q

deposition of what in skin and organs causes changes seen in SSc?

A

excessive collagen disposition in SSc

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13
Q

name the 2 organs affected in SSc that causes death

A

kidneys and lung

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14
Q

name the common early cutaneous finding in SSc

A

raynauds phenomenon

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15
Q

name the 3 phases of cutaneous involvement in SSc

A

(1) oedematous
(2) indurative
(3) atrophic

skin becomes thickened and tight

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16
Q

what is seen in MAJOR SSc cutaneous presentation?

A

centrally located skin sclerosis that affects the arms, face, and/or neck

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17
Q

what is seen in MINOR SSc cutaneous presentation?

A

sclerodactyly and atrophy of the fingertips and bilateral lung fibrosis

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18
Q

what is seen on the face of those with SSc?

A

pinching of skin of nose and tightening of skin around mouth

telangiectasia

calcinosis

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19
Q

name the the things that go wrong in the 2 main organs involved in SSc

A

lungs - pulmonary hypertension, pulmonary fibrosis and accelerated hypertension

kidneys - renal crisis due to pulmonary hypertension

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20
Q

what happens in the gut of those with SSc?

A

dysphagia, malabsorption and bacterial overgrowth of the small bowel

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21
Q

inflammatory arthritis and myositis may be seen in SSc

true or false?

A

true

22
Q

what can SSc be subdivided into?

A

limited and diffuse

23
Q

limited or diffuse SSc:

skin involved tends to be confined to face, hands and forearms and feet

organ involvement tends to occur later

A

limited

24
Q

limited or diffuse SSc:

skin changes develop more rapidly and can involve the trunk

early significant organ involvement

A

diffuse

25
Q

what antibody is associated with the following:

(a) limited SSc
(b) diffuse SSc

A

(a) limited - anti-centromere antibody association

(b) diffuse - anti-Scl-70 antibody association

26
Q

investigations in SSc?

A

pulmonary function testing
echo
monitoring of renal function

27
Q

treatment of the following in SSc:

raynauds/digital ulcers

A

calcium channel blockers

iloprost

bosentan

28
Q

treatment of the following in SSc:

renal involvement

A

ACE inhibitors

29
Q

treatment of the following in SSc:

GI involvement

A

PPIs for reflux

30
Q

treatment of the following in SSc:

interstitial lung disease

A

immunosuppression - cyclophosphamide

31
Q

what is defined as a condition which features symptoms also seen in other connective tissue diseases (CTDs)?

A

mixed connective tissue disease (MCTD)

32
Q

name the symptoms seen in MCTD

A 
raynauds
arthralgia/arthritis
myositis
sclerodactyly
pulmonary hypertension
interstitial lung disease
33
Q

name the antibody that MCTD is associated with

A

anti-RNP

34
Q

because of the risk of __________ _____________, regular echocardiograms (annually) are suggested for MCTD

screening for ILD with _________ ________ ______ should also take place

A

because of the risk of PULMONARY HYPERTENSION, regular echocardiograms (annually) are suggested for MCTD

screening for ILD with PULMONARY FUNCTION TESTS should also take place

35
Q

what drug is good for treatment of raynauds?

A

calcium channel blockers

36
Q

name the disorder that manifests clinically as recurrent venous or arterial thrombosis and/or fetal loss

A

anti-phospholipid syndrome (APS)

37
Q

what other diseases can APS occur in association with?

A

SLE or another rheumatic or autoimmune disorder

38
Q

what does APS increase the frequency of?

A

stroke or MI

39
Q

name the special type of endocarditis seen in APS

A

Libman-sacks (sterile) endocarditis

40
Q

recurrent pulmonary emboli or thrombosis can lead to life-threatening what in APS?

A

pulmonary hypertension

41
Q

name the rare, serious, and often fatal manifestation of APS (mortality rate of approximately 50%) characterized by multiorgan infarctions over a period of days to weeks

A

CAPS (catastrophic APS)

42
Q

loss of what in women is common is APS?

A

loss of fetes in 2nd/3rd trimester - recurrent petal loss is also possible

43
Q

is migraine common in APS?

A

yes

44
Q

common cutaneous finding in APS?

A

livedo-reticularis

45
Q

APS investigation?

A

thrombocytopenia and prolongation of APTT

46
Q

antibodies seen in APS?

A

lupus anticoagulant
anti-cardiolipin antibodies
anti-beta 2 glycoprotein

47
Q

APS treatment for thrombosis?

A

anti-coagulant

48
Q

APS treatment for those with recurrent pregnancy loss?

A

LMWH

49
Q

do patients who are found to have positive antibodies but who have never had had an episode of thrombosis require anti-coagulation in APS?

A

no!

50
Q

what drug should not be used in pregnant people with APS?

A

warfarin!

MSK (12 decks)

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