CTD Flashcards

1
Q

pathogenesis of SLE

A
loss of immune regulation
increased and defective apoptosis
nuclear material act as autoantigens
B and T cells stimulated 
autoantibodies produced
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what kidney problems can occur in SLE

A

Glomerulonephritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

how might glomerulonephritis due to SLE present

A
Proteinuria
Urine sediments
Urine RBC and casts
Hypertension
Acute renal failure
Chronic renal failure
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

investigations for SLE

A
FBC
U&Es
CRP, ESR
Complement levels
Renal function tests

Anti-nuclear antibody (ANA)
Anti-double stranded DNA antibodies (dsDNA)
Extractable nuclear antigens (ENA) - Anti-Sm, Anti-RNP, Anti-RO

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

why does a positive ANA not mean it is SLE

A

it is found in other conditions

e.g. RA, HIV, Hep C

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

when should a positive ANA be taken seriously

A
when other anti-nuclear antibodies are positive 
Anti-dsDNA
Anti-Sm
Anti-Ro
Anti-RNP
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what antibody is highly specific for SLE

A

Anti-double stranded DNA antibody (Anti-dsDNA)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what is Anti-Ro associated with

A

anti-la
Cutaneous manifestations
secondary sjogre’s features
congenital heart block

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

which antibody is high specific for SLE and associated with neurological involvement

A

Anti-Sm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

which antibody in SLE correlates with activity

A

Anti-dsDNA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

SLE general management

A

lifestyle changes

dietary advice, smoking cessation, sun protection, and exercise

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

SLE drug treatment

A

NSAIDs - normally Naproxen
Analgesia
When NSAID ineffective;
Anti-malarial - hydroxychloroquine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what is used in SLE when NSAIDs and hydroxychloroquine ineffective

A

steroids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

what is the last line of treatment for SLE

A

immunosuppressives

e.g. Azathioprine, Methotrextae

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

cons of immunosuppressies

A

All can cause bone marrow suppression
All can cause increased susceptibility to infection
Potentially teratogenic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what do patients with SLE die of

A

Infection

CV disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

what are key features of anti-phospholipid syndrome

A

Hx of 1 or more episodes of venous, arterial, or microvascular thrombosis
Hx of pregnancy loss
Hx of pregnancy-associated morbidity e.g. pre-eclampsia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

what is the antibody associated with anti-phospholipid syndrome

A

Positive anti-cardiolipin

19
Q

what other CTD is anti-phospholipid syndrome associated with

A

SLE

20
Q

what investigations can be done to confirm anti-phospholipid syndrome

A

lupus anticoagulant activity - positive on 2 occasions
anticardiolipin antibodies - positive on 2 occasions
anti-beta2-glycoprotein I antibodies - positive on 2 occasions

21
Q

what are the features/symtoms of anti-phospholipid syndrome

A
CLOTS
Coagulation defect
Livedo reticularis 
Obstetric (recurrent miscarriages)
Thrombocytopenia (low platelets)
22
Q

what is the treatment of anti-phospholipid syndrome

A

Thrombosis – LMWH
Pregnancy loss – aspirin + LMWH during pregnancy

Management of risk factors

23
Q

what is Sjogren’s syndrome

A

Lymphocyte infiltration of exocrine glands causing xerostomia (dry mouth) and keratoconjunctivitis sicca (dry eyes)

24
Q

what test can be done in Sjogren’s Syndrome to prove dry mouth

A

Schirmer test

25
Q

what are other symptoms of Sjogren’s syndrome

A
Fatigue
Joint pain
Raynauds
Lymphadenopathy
Skin/Vaginal dryness
26
Q

antibodies associated with Sjogren’s syndrome

A

Anti-Ro and La

27
Q

treatment of sjogren’s syndrome

A

eye drops/punctal plugs e.g. hypromellose
salvia replacement
pilocarpine - treats Dry mouth

Hydroxychloroquine - for joint pain
steroids
immunosuppression - in severe cases

28
Q

what is systemic sclerosis

A

autoimmune

excess deposition of collagen in skin and internal organs causing fibrosis

29
Q

what was limited systemic sclerosis previously known as

A
CREST
C - calconosis 
R - Raynauds
E - esophageal dysmotility
S - sclerodactyly (localized thickening and tightness of the skin of the fingers or toes)
T - telangiectasia
30
Q

what common symptoms is not mentioned in CREST in LIMITED systemic sclerosis

A

Pulmonary hypertension

31
Q

what antibodies is LIMITED systemic sclerosis associated with

A

anti-centromere antibodies

32
Q

what are the two types of scleroderma

A

localised

systemic

33
Q

what are the 3 subtypes of systemic scleroderma

A

Limited
Diffuse
Systemic

34
Q

what are the features of DIFFUSE systemic sclerosis

A

skin changes within 1 year of Raynaud’s
Truncal and acral skin involvement
Early significant organ involvement (kidneys, lungs, gut, muscle, joints, heart)

35
Q

what is the antibody associated with diffuse systemic sclerosis

A

Anti-Scl-70

36
Q

what are cutaneous features of Systemic sclerosis

A
Raynaud's phenomenon
Ulcers
Swelling of hands and feet
Skin thickening 
Sclerodactyly
37
Q

what are organ manifestations of systemic sclerosis

A

GI

  • Oesophageal hypomobility
  • Small bowel hypo mobility
  • Pancreatic insufficiency
  • Rectal hypomobility

Resp
- interstitial lung disease
- pulmonary hypertension
chest wall restriction

Renal

  • hypertensive renal crisis
  • ischaemic

CV

  • Raynaud’s with digital ulceration
  • Atherosclerotic disease
  • Hypertensive cardiomyopathy
38
Q

treatment for systemic sclerosis

A
ACE inhibitors 
CCB
Prednisolone
Immunosuppression
Biological agents
39
Q

what antibody is associated with mixed connective tissue disease

A

Anti-RNP

40
Q

what antibody is associated with polymyositis

A

Anti-Jo-1

41
Q

what are the major symptoms of mixed connective tissue disease

A
Severe myositis.
Pulmonary involvement.
Raynaud's phenomenon.
Swollen hands observed.
Sclerodactyly.
42
Q

what are minor features of MCTD

A
Alopecia
Leukopenia
Anaemia
Pleuritis
Pericarditis
Arthritis
Trigeminal neuralgia
Malar rash
Thrombocytopenia
Mild myositis
History of swollen hands
43
Q

all antibodies associated with SLE

A

ANA, Anti-DNA binding, Anti-Sm, Anti-Ro and La

44
Q

Criteria for diagnosing SLE

need more than 4 out of a 11 to be diagnosed with SLE

A

1 - Malar/Butterfly Rash
2 - Discoid rash
3- Photosensitivity
4 - Oral Ulcers
5 - Non-erosive arthritis
6 - Serositis (either pleuritis or pericarditis)
7 - Renal disorder (Glomerulonephritis, persistent proteinuria)
8 - CNS disorder (seizures, psychosis)
9 - Haematological disorder (haemolytic anaemia, thrombocytopenia)
10 - Immunological disorder (presence of antibody)
11 - ANA positive