4: Connective tissue diseases 1 Flashcards

1
Q

Most connective tissue diseases are ___ diseases.

A

autoimmune

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2
Q

Connective tissue diseases often affect multiple ___.

A

systems

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3
Q

What are some examples of CTDs?

A

SLE

Sjogren’s syndrome

Systemic sclerosis

Dermatomyositis

Polymyositis

Anti-phospholipid syndrome

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4
Q

Do connective tissue diseases affect connective tissue?

A

No

autoimmune disease - antibodies

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5
Q

In connective tissues diseases, the immune system attack cells (in one place / all over the body).

A

all over the body

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6
Q

Connective tissue diseases are more common in (men / women).

A

women

as with all autoimmune diseases

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7
Q

What four factors are thought to cause connective tissue diseases?

A

Genetics

Environment

Faulty immune system

Hormones

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8
Q

Which hormone is linked to the onset of CTDs?

A

Oestrogen

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9
Q

Which virus is associated with lupus?

A

Epstein-Barr virus

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10
Q

What environmental factors are linked to lupus?

A

UV exposure

Cigarette smoking

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11
Q

Although lupus is less common in men, it tends to be more ___.

A

severe

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12
Q

Which immune cells are antigens presented to?

A

T cells

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13
Q

In lupus, apoptotic cells aren’t cleaned up and their contents float around for longer than normal.

What is the consequence of this?

A

Immune system recognises nuclear material as foreign antigen

Nuclear material presented to T cells, which activate B cells

Production of autoantibodies

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14
Q

In lupus, the immune system treats self nuclear material as an antigen.

Which processes occur?

A

Cytokines released

Complement released

Inflammation

Necrosis

Scarring

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15
Q

Which system is asymptomatic when it’s being messed up by lupus?

A

Renal

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16
Q

What are deposited in the kidneys and the skin in lupus?

A

Immune complexes

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17
Q

In lupus, immune complexes get lodged in endothelial walls - what happens next?

A

Immune response - complement released, acute inflammation

Endothelial damage

Leaky vessels

Oedema

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18
Q

What is a very common symptom of lupus?

A

Rash

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19
Q

What are general symptoms of lupus?

A

Fever

Weight loss

Fatigue

Anorexia

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20
Q

What does a malar rash look like?

A

Butterfly rash on face associated with UV exposure

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21
Q

What aspect of the face is spared by lupus?

A

Naso-labial folds

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22
Q

___ - the tendency to burn easily - is a skin symptom of SLE.

A

Photosensitivity

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23
Q

What hair condition is associated with lupus?

A

Alopecia

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24
Q

Which lesions appear in the oral and nasal mucosa in some cases of SLE?

A

Ulcers

25
Q

An MSK symptom of lupus is ___ ___ with no actual joint damage.

A

joint pain

26
Q

What is serositis?

A

Inflammation of a lining e.g pleura, pericardium…

27
Q

Serositis secondary to lupus often presents as what?

A

Pericarditis / Pericardial effusion

Pleurisy / Pleural effusion

28
Q

What is a renal sign of lupus?

A

Proteinuria

elevated levels of protein in the urine

29
Q

After a positive urinalysis, what is done to confirm that someone has renal disease secondary to lupus?

A

Renal biopsy

30
Q

Lupus can also cause what brain-related disease?

A

Neurological disease

31
Q

Name three haematological signs of lupus you might see on a FBC.

A

Anaemia - low RBCs

Thrombocytopaenia - low platelets

Leukopaenia - low white blood cells (i.e immune cells)

32
Q

Which structures are often enlarged in lupus?

A

Lymph nodes

33
Q

Which syndrome, associated with lupus, causes hypercoagulability and may result in thrombosis and recurrent miscarriage?

A

Anti-phospholipid syndrome

34
Q

People with lupus are more susceptible to ___.

Why?

A

infection

through the haem effects of the disease itself AND the immunosuppressive drugs used to treat it

35
Q

How is suspected SLE diagnosed?

A

Autoantibody tests

36
Q

Most people with lupus are positive for which antibody?

A

Anti-nuclear antibody (ANA)

37
Q

ANA is a (specific / non-specific) antibody for lupus.

A

non-specific

found in other autoimmune diseases, RA, HIV, Hep C….

38
Q

Apart from ANA, what other autoantibodies are associated with lupus?

A

Anti-dsDNA (anti double stranded DNA antibody)

Anti-Sm

Anti-Ro (non-specific for lupus but uncommon)

Anti-RNP

39
Q

Which antibodies are highly specific for lupus?

A

Anti-dsDNA

Anti-Sm

40
Q

The higher the titre of anti-dsDNA, the more (active / inactive) the disease.

A

active

41
Q

Babies of mothers who are anti-Ro positive may present with what?

A

Neonatal lupus

of which heart block is a significant feature

42
Q

Which autoantibodies are found in anti-phospholipid syndrome?

A

Anti-cardiolipin antibody

Lupus anticoagulant

Anti-beta 2 glycoprotein

43
Q

Once you’ve diagnosed someone with lupus, what must you do?

A

Screen every other system

44
Q

What’s the most appropriate first screening test in someone newly diagnosed with lupus?

A

Urinanalysis

for renal disease

45
Q

What are the two inflammatory markers which rise in people with rheumatoid conditions?

A

CRP

PV

46
Q

Does CRP rise in flare ups of lupus?

A

NO

47
Q

When may CRP be raised in a lupus patient?

A

Infection

Co-existing inflammatory disease e.g RA

48
Q

Which changes in two markers indicate a flare up of lupus?

A

INCREASED anti-dsDNA (antibody associated with active disease)

DECREASED in C4 (complement being used up)

49
Q

In a flare up of lupus, the level of anti-dsDNA (increases / decreases).

A

increases

50
Q

In a flare up of lupus, the level of complement (increases / decreases).

A

decreases

51
Q

SLE tends to get (better / worse) during pregnancy.

A

worse

52
Q

Patients with lupus are advised to avoid excessive __ __.

A

sun exposure

because it causes photosensitivity

53
Q

Which drugs are used to treat lupus?

A

1. NSAIDs, analgesics, HYDROXYCHLOROQUINE

2.

Steroids

Immunosuppressants (mycophenolate mofetil, cyclophosphamide, methotrexate, azathioprine)

Biologic agents

54
Q

If all your drugs aren’t controlling lupus symptoms, which drugs can you use?

A

Biologic agents

55
Q

Which DMARD is used first to treat lupus?

A

Hydroxychloroquine

56
Q

What are the immediate tests for someone who has suspected SLE?

A

Urinalysis

Antibodies and complement

57
Q

Does a positive ANA guarantee lupus?

A

No

58
Q

What is the most common presentation of SLE and who has it?

A

Fever, joint pain and a rash

In a woman of childbearing age

59
Q

What is the typical distribution of joint pain seen in SLE?

A

Symmetrical polyarthritis affecting the small joints of hands and feet