Sjogren's Syndrome, SSc, MCTD and APS

Question 1

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

Answer 1

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

Question 2

what typical things does Sjogrens cause?

Answer 2

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

Question 3

Sjogrens can occur secondary to what 2 other autoimmune conditions?

Answer 3

RA and SLE

Question 4

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

true or false?

Answer 4

true

Question 5

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

Answer 5

lymphoma

Question 6

name the test for ocular dryness

Answer 6

schirmers test

Question 7

what biopsy is performed to diagnose Sjogrens?

Answer 7

lip biopsy

Question 8

name the 2 antibodies found in Sjogrens

Answer 8

anti-Ro and anti-La

Question 9

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?

Answer 9

Sjogrens

Question 10

Sjogrens treatment?

Answer 10

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

Question 11

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

Answer 11

systemic sclerosis

Question 12

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

Answer 12

excessive collagen disposition in SSc

Question 13

name the 2 organs affected in SSc that causes death

Answer 13

kidneys and lung

Question 14

name the common early cutaneous finding in SSc

Answer 14

raynauds phenomenon

Question 15

name the 3 phases of cutaneous involvement in SSc

Answer 15

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

skin becomes thickened and tight

Question 16

what is seen in MAJOR SSc cutaneous presentation?

Answer 16

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

Question 17

what is seen in MINOR SSc cutaneous presentation?

Answer 17

sclerodactyly and atrophy of the fingertips and bilateral lung fibrosis

Question 18

what is seen on the face of those with SSc?

Answer 18

pinching of skin of nose and tightening of skin around mouth

telangiectasia

calcinosis

Question 19

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

Answer 19

lungs - pulmonary hypertension, pulmonary fibrosis and accelerated hypertension

kidneys - renal crisis due to pulmonary hypertension

Question 20

what happens in the gut of those with SSc?

Answer 20

dysphagia, malabsorption and bacterial overgrowth of the small bowel

Question 21

inflammatory arthritis and myositis may be seen in SSc

true or false?

Answer 21

true

Question 22

what can SSc be subdivided into?

Answer 22

limited and diffuse

Question 23

limited or diffuse SSc:

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

organ involvement tends to occur later

Answer 23

limited

Question 24

limited or diffuse SSc:

skin changes develop more rapidly and can involve the trunk

early significant organ involvement

Answer 24

diffuse

Question 25

what antibody is associated with the following: (a) limited SSc (b) diffuse SSc

Answer 25

(a) limited - anti-centromere antibody association | (b) diffuse - anti-Scl-70 antibody association

Question 26

investigations in SSc?

Answer 26

pulmonary function testing echo monitoring of renal function

Question 27

treatment of the following in SSc: raynauds/digital ulcers

Answer 27

calcium channel blockers iloprost bosentan

Question 28

treatment of the following in SSc: renal involvement

Answer 28

ACE inhibitors

Question 29

treatment of the following in SSc: GI involvement

Answer 29

PPIs for reflux

Question 30

treatment of the following in SSc: interstitial lung disease

Answer 30

immunosuppression - cyclophosphamide

Question 31

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

Answer 31

mixed connective tissue disease (MCTD)

Question 32

name the symptoms seen in MCTD

Answer 32

``` raynauds arthralgia/arthritis myositis sclerodactyly pulmonary hypertension interstitial lung disease ```

Question 33

name the antibody that MCTD is associated with

Answer 33

anti-RNP

Question 34

because of the risk of __________ _____________, regular echocardiograms (annually) are suggested for MCTD screening for ILD with _________ ________ ______ should also take place

Answer 34

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

Question 35

what drug is good for treatment of raynauds?

Answer 35

calcium channel blockers

Question 36

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

Answer 36

anti-phospholipid syndrome (APS)

Question 37

what other diseases can APS occur in association with?

Answer 37

SLE or another rheumatic or autoimmune disorder

Question 38

what does APS increase the frequency of?

Answer 38

stroke or MI

Question 39

name the special type of endocarditis seen in APS

Answer 39

Libman-sacks (sterile) endocarditis

Question 40

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

Answer 40

pulmonary hypertension

Question 41

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

Answer 41

CAPS (catastrophic APS)

Question 42

loss of what in women is common is APS?

Answer 42

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

Question 43

is migraine common in APS?

Answer 43

yes

Question 44

common cutaneous finding in APS?

Answer 44

livedo-reticularis

Question 45

APS investigation?

Answer 45

thrombocytopenia and prolongation of APTT

Question 46

antibodies seen in APS?

Answer 46

lupus anticoagulant anti-cardiolipin antibodies anti-beta 2 glycoprotein

Question 47

APS treatment for thrombosis?

Answer 47

anti-coagulant

Question 48

APS treatment for those with recurrent pregnancy loss?

Answer 48

LMWH

Question 49

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

Answer 49

no!

Question 50

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

Answer 50

warfarin!