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Maternal Medicine > Connective Tissue Disease > Flashcards

Connective Tissue Disease Flashcards

1
Q

What is the shift in immunity in pregnancy?

A

Shift away from cell-mediated immunity (T-helper / Th1 response)
To humoral immunity (Th2 response)

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2
Q

What is Rheumatoid arthritis?

What are the clinical features of it?

A

Chronic inflammatory disease affecting primarily the synovial joints

Deforming polyarthritis with synovitis of joint and tendon sheaths, articular cartilage loss and erosion of juxta-articular bone

Symmetrical
Particularly MCP, proximal IP, wrist joints

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3
Q

What are extra-articular features of Rheumatoid Arthritis?

A 
Fatigue
Vascularise
Subcutaneous rheumatoid nodules
Haematological abnormalities 
Pulmonary granulomas
Effusions and fibrosis
Cardiac involvement
Amyloidosis 
Scleritis, scleromalacia, secondary Sjögren’s syndrome
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4
Q

What antibodies are present in Rheumatoid arthritis?

A

Anti-nuclear antibodies (30%)
Rheumatoid factor (80-90%)
Anti-CCP: biomarker that predicts aggressive disease

Anti-Ro and Anti-La
Antiphospholipid antibodies, but APS is unusual

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5
Q

What is the effect of pregnancy on Rheumatoid Arthritis?

A

50% improve
- usually during the first trimester
25% will have substantial disability

90% suffer postpartum exacerbations within the first 4 months

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6
Q

What is the effect of Rheumatoid arthritis on pregnancy?

A

PTB
SGA
Anti-Ro / La: cutaneous lupus, heart block

Atlanto-axial subluxation can complicate a GA
Limitation of hip addition can impede vaginal delivery

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7
Q

What is the management of Rheumatoid Arthritis in pregnancy?

A

Analgesia
Steroids
Azathioprine
Hydroxychloroquine
Sulfasalazine (with concomitant folate 5mg)
Biological therapy: TNF-alpha antagonists

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8
Q

What is the incidence of SLE

A

1:1000

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9
Q

What are the clinical features of SLE?

A

Systemic connective tissue disease
Characteristised by periods of disease activity and remissions
Heterogenous
Joint involvement (90%) - peripheral joints
Skin involvement (80%) - malar rash, photosensitivity, vasculitic lesions, Raynau’ds
Serositis
Renal involvement
Neurological involvement
Haematological involvement

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10
Q

How is SLE diagnosed?

A 
Raised ESR, normal CRP
Low / falling C3 C4
ANA (96%)
DsDNA
ENAs: Anti-Ro/La
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11
Q

What is the effect of pregnancy on SLE?

A

Increased likelihood of flare (from 40 to 60%)

May be difficult to diagnose

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12
Q

What is the effect of SLE on pregnancy?

A 
Spontaneous miscarriage
Fetal death
PET
PTB
FGR

Related to the presence of :

  • APS - aCL / LA,
  • lupus nephritis or hypertension,
  • active disease at the time of conception
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13
Q

In Ro-positive mothers, what are the neonatal / fetal risks?

A

Transient neonatal cutaneous lupus (5%)

Congenital heart block (2%)

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14
Q

Describe cutaneous form of neonatal lupus

A

Typical erythematous geographical skin lesions
Annular wheels
Face and scalp, which are photosensitive

Usually manifests in the first 2 weeks of life
Disappears spontaneously within 4-6 months

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15
Q

Describe congenital heart block, from Anti-Ro / La

A

Appears in utero, is permanent, and has 15-20% mortality
Normally detected at 18-28/40
Inflammation and fibrosis of the conducting system

Most infants need pacemakers either in infancy, or in their teens

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16
Q

What are the clinical criteria for APS?

A

Elevated ACL or LA Antibodies (IgG or IgM) on two occasions ≥12 weeks apart

AND

At least 1 clinical feature of:
>/= 3 consecutive miscarriages <10/40
>/= 1 FDIU > 10/40
>/= 1 PTB < 34/40 due to PET or severe placental insufficiency
- Second two only with normal fetal morphology

Venous thrombosis
Arterial thrombosis
Small vessel thrombosis

17
Q

What are the biochemical criteria for APS?

A

2 x positive readings 12/52 apart of any of one of Anticardiolipin Ab OR Lupus anticoagulant
(IgM or IgG) High titre

18
Q

What is the pathogenesis of APS-associated loss?

A

Massive infarction and thrombosis of the placental and decidual vessels
Probably secondary to spiral artery vascular achy

19
Q

What is the effect of APS on pregnancy?

A 
Miscarriage
Second/third trimester fetal death
PET
FGR
Placental abruption
20
Q

What is the treatment for APS in pregnancy?

A

LDA
Consideration of Clexane
- definitely if previous VTE
- possibly in most other clinical contexts

BP and urinalysis monitoring
Uterine artery Doppler at 20-24/40
Monthly growth scan with UAPI if indicated

21
Q

What is Ehlers Danlos Syndrome?

A

Autosomal dominant Inherited defects of collagen metabolism

Fragile skin and blood vessels, easy bruising, skin hyperelasticity, joint hypermobility

22
Q

What are four types of EDS, and which ones carry the highest risk in pregnancy?

A

I - Classic or Gravis (high risk)
II - Mitis
III - Joint hypermobility syndrome (commonest). May be associated with POTS
IV - Ecchymotic or vascular (high risk), maternal mortality 20-25%

Types I and IV carry most risk in pregnancy

23
Q

What is vascular (type IV) EDS associated with a risk of?

A

Aortic dissection/rupture
Uterine rupture
PTB
Skin fragility and poor healing

24
Q

What is joint hypermobility syndrome (Type III EDS) associated with in pregnancy?

A

PPROM and PTL
Precipitous labour
PPH
Skin fragility and poor healing

25
Q

For what type of EDS is TOP advised?

A

Type IV: vascular

If pregnancy continued, recommend CS at 34/40 to reduce the risk of uterine and aortic rupture towards the end of the third trimester

26
Q

How is osteoporosis defined?

A

Bone density <2.5 (T score) standard deviations below the mean

27
Q

What are the clinical manifestations of APS?

Apart from the clinical criteria

A

Epilepsy
Retinal vein thrombosis

Pulmonary HTN, cardiac valvular disease,

Livedo reticularis (mottled discolouration of the skin)
Osteonecrosis
Vascular dementia

28
Q

In APS, what are risk factors that predict poor pregnancy outcome?

A

Systemic autoimmune disease esp SLE
Previous thrombotic event
Reduced complement levels
Lupus anticoagulant positivity
Triple antibody positivity
REduced blood flow in the uterine arteries
- indirect indicator of the development of placental insufficiency and/or pre-eclampsia

Maternal Medicine (18 decks)

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