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Int Spec (Rheumatology) > Connective Tissue Diseases > Flashcards

Connective Tissue Diseases Flashcards

1
Q

How should mild cases (i.e. cutaneous manifestations and arthralgia) of SLE be managed?

A

Simple analgesia, topical steroids and hydroxychloroquine

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2
Q

Who is Sjogren’s syndrome most likely to occur in?

A

Women in their 50s/60s

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3
Q

How should moderate cases (i.e. inflammatory arthritis and minimal organ involvement) of SLE be managed?

A

Simple analgesia, oral steroids, hydroxychloroquine, azathioprine/methotrexate

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4
Q

How should severe cases (i.e. major organ involvement) of SLE be managed?

A

Simple analgesia, IV steroids, hydroxychloroquine, cyclophosphamide +/- rituximab

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5
Q

How is Sjogren’s syndrome diagnosed?

A

Positive Schirmer’s test (confirming dry eyes), typical features on lip gland biopsy and the presence of anti-Ro/anti-La antibodies

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6
Q

What type of hypersensitivity reaction is SLE and why?

A

Type III- it is immune complex mediated

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7
Q

Which connective tissue disease may present with unexplained dental caries?

A

Sjogren’s syndrome

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8
Q

How are each of the following manifestations of systemic sclerosis managed: a) Raynaud’s? b) renal involvement? c) GI involvement? d) interstitial lung disease?

A

a) Ca++ channel blocker b) ACE inhibitor c) PPI d) cyclophosphamide

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9
Q

Apart from the malar rash, name some cutaneous features of SLE?

A

Oral/nasal ulceration, Raynaud’s phenomenon, alopecia

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10
Q

What are some important organ manifestations of systemic sclerosis?

A

Pulmonary hypertension, pulmonary fibrosis and accelerated hypertension (leading to renal crisis)

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11
Q

Having Sjogren’s syndrome increases a person’s risk of developing which other disease?

A

Lymphoma

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12
Q

More than 90% of cases of SLE occur in women- when does it usually present?

A

At childbearing age (typically 20s and 30s)

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13
Q

How is Sjogren’s syndrome managed?

A

Generally with lubricating eye drops and saliva replacement; hydroxychloroquine may be helpful for arthralgia/fatigue

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14
Q

Why are a CXR and PFTs performed on any patient with suspected SLE?

A

To look for evidence of any interstitial lung disease

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15
Q

What investigations should be performed if someone is suspected to have SLE?

A

Routine bloods, antibody screen, C3/4 levels, urinalysis, CXR and PFTs, ECHO

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16
Q

Which connective tissue disease is characterised by lymphocytic infiltrates in exocrine organs?

A

Sjogren’s syndrome

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17
Q

In addition to anti-dsDNA, what is another antibody which is specific for SLE but has low sensitivity?

A

Anti-Sm

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18
Q

What is the risk of being Anti-Ro positive during pregnancy?

A

Can cause foetal heart block, this must be monitored with in-utero ECHOs

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19
Q

Name some general features of most connective tissue diseases?

A

Fever, weight loss, fatigue

20
Q

Describe the features of limited systemic sclerosis?

A

Skin involvement of the face and distal arms/legs; organ involvement occurs late

21
Q

Limited systemic sclerosis can sometimes be known as CREST syndrome- what are the features of this?

A

Calcinosis, Raynaud’s, oesophageal dysmotility, sclerodactyly, telangectasia

22
Q

Describe the features of diffuse systemic sclerosis?

A

Skin involvement may include the proximal limbs and torso, significant organ involvement occurs early

23
Q

When investigating for anti-phospholipid syndrome, as well as antibodies, what other features may be seen on blood tests?

A

Thrombocytopenia and prolonged APTT

24
Q

What is the most important investigation in someone with suspected SLE and why?

A

Urinalysis- to detect evidence of glomerulonephritis as this shows no clinical features

25
Q

What are the anti-phospholipid antibodies?

A

Anti-cardiolipin, lupus anticoagulant and anti-beta 2 glycoprotein

26
Q

What are the main features of anti-phospholipid syndrome?

A

Recurrent venous/arterial thromboses and/or foetal loss

27
Q

What antibody is specific for SLE?

A

Anti-dsDNA

28
Q

Why is an ECHO performed on any patient with suspected SLE?

A

To look for evidence of any pulmonary hypertension

29
Q

If a patient with SLE wanted to get pregnant, which antibody should be checked for and why?

A

Anti-phospholipid antibodies- if these are present then they should be given LMWH and aspirin during pregnancy until 6 weeks post-partum

30
Q

Which antibody is present in > 95% of patients with SLE but is not specific?

A

ANA

31
Q

It is unusual to have systemic sclerosis without having what feature?

A

Raynaud’s phenomenon

32
Q

What is a common cutaneous finding of patients with anti-phospholipid syndrome?

A

Livedo reticularis

33
Q

What happens to complement (C3/4) levels in active SLE?

A

They decrease

34
Q

Organ screening is performed regularly in patients with systemic sclerosis- what does this involve?

A

PFTs, ECHO and urinalysis

35
Q

Mixed connective tissue disease is mostly associated with which antibodies?

A

Anti-RNP

36
Q

What investigations can be used to monitor patients with SLE?

A

Anti-dsDNA levels, C3/4 levels, urinalysis, evaluation of CV risk with BP and cholesterol levels

37
Q

Do patients who have positive anti-phospholipid antibodies but no previous thrombosis require treatment?

A

No

38
Q

For APS to be diagnosed, there must be persistent lab evidence of anti-phospholipid antibodies- what is meant by this?

A

These must be positive on two occasions, at least 12 weeks apart

39
Q

What is the mainstay of treatment for APS?

A

Anticoagulation

40
Q

If someone with suspected SLE has haematuria or proteinuria on urinalysis, what investigation should be done next?

A

Renal biopsy

41
Q

What advice would you give to someone with SLE who wanted to get pregnant?

A

Ideally, they would be free from active disease for at least 6 months before getting pregnant

42
Q

Systemic sclerosis is diagnosed when a patient has 1 major and 2 minor features- what are these?

A

Minor = sclerodactyly, atrophy of the fingertips, bilateral lung fibrosis / major = skin sclerosis affecting the arms, face and/or neck

43
Q

What are the most common symptoms of Sjogren’s disease?

A

Dry eyes and mouth (parotid gland swelling and vaginal dryness may also occur)

44
Q

A malar (butterfly) rash is typically seen in which condition? What are some features of this rash which differentiate it from other conditions?

A

SLE - it is usually photosensitive and always spares the naso-labial folds

45
Q

What autoantibody is suggestive of drug-induced lupus?

A

Anti-histone antibodies

Int Spec (Rheumatology) (6 decks)

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