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Flashcards in Connective Tissue Disease Deck (56):
1

How does antiphospholipid syndrome manifest clinically?

As arterial or venous thrombosis (common cause of these in people under 50)
Pregnancy loss with no other explanation or pre-term birth because of eclampsia, severe pre-eclampsia or with signs of placental insufficiency.l
Pregnancy loss is commonly late, in the second or third trimester but can be at any time

2

What do we need to diagnose antiphospholipid syndrome?

One positive lab test and one clinical feature present in the right patient context.

3

What lab tests can be used as part of the diagnosis for antiphospholipid syndrome?

Positive anti-cardiolipin (anti-phospholipid) antibodies.
And/or lupus anticoagulant activity
And/or anti-beta2-glycoprotein

These must be present on 2 occasions at least 12 weeks apart.

4

Is antiphospholipid syndrome more common in males or females?

Females X 3.5

5

What other types of people can have anti-cardiolipin antibodies other than APS patients?

Around a third of those with SLE.
Some in people with other CTD's
Around 1-5% of the healthy population

6

What features other than pregnancy loss and arterial/venous thrombosis do we see in APS?

Superficial thrombophlebitis and livedo reticularis
Mild/moderate thrombocytopenia
Neurological features e.g. Migraine and transverse myelitis
Libman Sachs endocarditis

7

What CTD should we be suspicious of if we see strokes or MI's in younger patients?

APS

8

What can strokes develop secondary to in APS?

In situ thrombosis or Libman sacks valvular lesions.

9

What kind of lesions are found in Libman sacks endocarditis?

Sterile.

10

What can recurrent PE or thrombosis lead to in APS patients?

Life threatening pulmonary hypertension.

11

What is CAPS?

Catastrophic APS. Characterised by multi organ infarctions seen over days to weeks.

12

What serum results can we find in APS?

Thrombocytopenia and prolonged APTT (anti partial thromboplastin time)

13

What are the treatments for APS?

Thrombosis (lifelong anticoagulation)
Pregnancy loss is given aspirin and heparin
Attention is paid to vascular risk factors.

14

Why is warfarin not given to APS sufferers during pregnancy?

It is teratogenic.

15

Is Sjogrens a primary or secondary condition?

It can be either

16

What is Sjorgens and what does it cause?

An autoimmune condition resulting in lymphocyte infiltration of the exocrine glands. This causes xerostomia and keratoconjunctivitis sicca (commonly in the lacrimal glands and mouth.

17

What is xerostomia?

Dryness in the mouth.

18

What are the 6 classification criteria of Sjogrens?

Subjective ocular symptoms (daily for over 3 months)
Subjective oral symptoms (daily >3 months)
Objective evidence of ocular dryness
Objective evidence of salivary gland involvement
Either Ro La or both
Lip gland biopsy with evidence of lymphocytic infiltrate

19

What must be present in order to diagnose Sjogrens?

4 of 6 of the classification criteria which must include either immunology or biopsy evidence.

20

What is Schirmers test?

A test for Sjogrens where paper is put in the lower eyelid and left for 5 mins. Tears should come down to at least 10mm

21

What are some of manifestations of Sjogrens other than eye and mouth involvement?

Fatigue
Arthralgia
Raynauds
Salivary swelling (especially parotid)
Lymphadenopathy (predominantly cervical)
Skin and vaginal dryness
Interstitial lung disease (far more common in other CTD's)
Neuropathy (in the glove stocking pattern)
Lymphoma
Renal tubular acidosis
Neonatal complete heart block (anti Ro)

22

What is the peak age for primary Sjogrens?

40-60 years old

23

Does Sjogrens affect males or females more?

Females x9

24

What are the treatments for Sjogrens?

Mostly to stress symptoms
Eye drops and punctual plugs
Regular dental care
Saliva replacement or stimulation (pilocarpine stimulates but may cause flushing)
Hydroxychloroquine can help with arthralgia and fatigue
Steroids and immunosuppression
CVS risk factor monitoring

25

What is Scleroderma?

Excessive fibrosis of organs and tissues due to excessive collagen production.

26

What organs does scleroderma commonly affect?

Skin, GI tract, heart, lungs, kidneys, arteries and MSK

27

What is the peak age for Scleroderma?

25-55

28

Does scleroderma affect males or females more?

Females x4

29

What syndrome is scleroderma associated with?

CREST syndrome

30

What does CREST syndrome comprise of?

Calcinosis
Reynauds
Oesophageal dysfunction
Sclerodactyly
Telangectasia

31

What two categories of scleroderma are there?

Localised and systemic

32

What subtypes of systemic scleroderma are their?

Limited and diffuse

33

What is sclerodactyly?

Localised thickening and tightening of the skin of the fingers or toes

34

Other than CREST features what is also found in around 30% of limited systemic sclerosis patients?

Pulmonary hypertension

35

What antibodies are associated with limited systemic sclerosis?

Anti-centromere antibodies

36

Where does the skin involvement tend to occur in limited systemic sclerosis?

Face, hands, forearms and feet

37

Is organ involvement early or late in limited systemic sclerosis?

Late

38

What are the features of diffuse systemic sclerosis?

Skin changes within one year of raynauds
Truncal and acral skin involvement
Early significant organ involvement
Anti-scl-70 antibodies

39

What can scleroderma cause in the heart?

Pericarditis and myocardial fibrosis

40

What can scleroderma cause in the MSK system?

Polyarteritis and myositis

41

What are the three phases of cutaneous involvement in scleroderma?
What are the results of these?

Oedematous
Indurative
Atrophic

Skin becomes thickened and tight and can result in decreased joint movement

42

How is a diagnosis made for systemic sclerosis?

If a patient has 1 major and 2 minor classification features.

Major = proximal sclerosis/induration of the skin

43

What major cutaneous features are seen in systemic sclerosis?

On the face beaking occurs when the skin of the nose pinches. Tightening of the skin around the mouth.

Telangectasia

Calcinosis (subcutaneous deposits)

44

What GI symptoms does systemic sclerosis?

Oesophageal hypomobility
Small bowel hypomobility and bacterial overgrowth
Pancreatic insufficiency
Rectal hypomobility
Dysphagia
Malabsorption

45

What Respiratory symptoms does systemic sclerosis?

Interstitial lung disease
Pulmonary hypertension
Chest wall restriction
Pulmonary fibrosis

46

What renal issues does systemic sclerosis cause?

Hypertensive renal crisis
Ischaemic issues

47

What cardiovascular issues does systemic sclerosis cause?

Raynauds with digital ulceration
Atherosclerotic disease
Hypertensive cardiomyopathy

48

What investigations do we do for systemic sclerosis?

Anti centromere antibody and a to Scl-70

Organ screening e.g.pulmonary function tests, echo and urinalysis

49

What are the treatments for systemic sclerosis?

Tailored to symptoms.

Raynauds/digital ulcers = calcium channel blockers e.g. Iloprost and bosentan.
Renal involvement = ACE inhibitors
GI = PPI's
Interstitial lung disease = immunosuppression usually with cyclophosamide.

50

What usually causes death in systemic sclerosis?

Renal failure secondary to malignant hypertension
Sever respiratory compromise
Cor pulmonale
Cardiac failure or arrhythmias secondary to myocardial fibrosis

51

What are the common features of connective tissue disease that we should know to allow us to recognise a CTD even if we don't know the type?

Multi system including joints, skin and subcut tissues.
Women affected more than men
Immunological abnormalities are common
Usually respond to anti inflammatory drugs

52

What are the 6 major criteria for mixed connective tissue disease?

severe myositis
pulmonary involvement
Raynauds
observed swollen hands
sclerodactyly
anti-U1-RNP over 1 in 10000

53

what are the 11 minor criteria for mixed connective tissue disease?

alopecia
leukopenia
anaemia
pleuritis
pericarditis
arthritis
trigeminal neuralgia
malar rash
thrombocytopaenia
mild myositis
history of swollen hands

54

what screening must we do for mixed connective tissue disease and why?

Due to the risk of pulmonary hypertension we must do regular echos and pulmonary function tests.

55

what treatments do we use for connective tissue disease?

according to symptoms e.g. calcium channel blockers for Raynauds. if there is significant muscle or lung involvement, immunosuppressants may be needed.

56

what is undifferentiated connective tissue disease?

a syndrome that has parts of different diseases but doesnt fit into one. it usually has a better prognosis and some antibody positivity can be expected.