Connective Tissue Disease Flashcards
What is the shift in immunity in pregnancy?
Shift away from cell-mediated immunity (T-helper / Th1 response)
To humoral immunity (Th2 response)
What is Rheumatoid arthritis?
What are the clinical features of it?
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
What are extra-articular features of Rheumatoid Arthritis?
Fatigue Vascularise Subcutaneous rheumatoid nodules Haematological abnormalities Pulmonary granulomas Effusions and fibrosis Cardiac involvement Amyloidosis Scleritis, scleromalacia, secondary Sjögren’s syndrome
What antibodies are present in Rheumatoid arthritis?
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
What is the effect of pregnancy on Rheumatoid Arthritis?
50% improve
- usually during the first trimester
25% will have substantial disability
90% suffer postpartum exacerbations within the first 4 months
What is the effect of Rheumatoid arthritis on pregnancy?
PTB
SGA
Anti-Ro / La: cutaneous lupus, heart block
Atlanto-axial subluxation can complicate a GA
Limitation of hip addition can impede vaginal delivery
What is the management of Rheumatoid Arthritis in pregnancy?
Analgesia
Steroids
Azathioprine
Hydroxychloroquine
Sulfasalazine (with concomitant folate 5mg)
Biological therapy: TNF-alpha antagonists
What is the incidence of SLE
1:1000
What are the clinical features of SLE?
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
How is SLE diagnosed?
Raised ESR, normal CRP Low / falling C3 C4 ANA (96%) DsDNA ENAs: Anti-Ro/La
What is the effect of pregnancy on SLE?
Increased likelihood of flare (from 40 to 60%)
May be difficult to diagnose
What is the effect of SLE on pregnancy?
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
In Ro-positive mothers, what are the neonatal / fetal risks?
Transient neonatal cutaneous lupus (5%)
Congenital heart block (2%)
Describe cutaneous form of neonatal lupus
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
Describe congenital heart block, from Anti-Ro / La
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
What are the clinical criteria for APS?
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
What are the biochemical criteria for APS?
2 x positive readings 12/52 apart of any of one of Anticardiolipin Ab OR Lupus anticoagulant
(IgM or IgG) High titre
What is the pathogenesis of APS-associated loss?
Massive infarction and thrombosis of the placental and decidual vessels
Probably secondary to spiral artery vascular achy
What is the effect of APS on pregnancy?
Miscarriage Second/third trimester fetal death PET FGR Placental abruption
What is the treatment for APS in pregnancy?
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
What is Ehlers Danlos Syndrome?
Autosomal dominant Inherited defects of collagen metabolism
Fragile skin and blood vessels, easy bruising, skin hyperelasticity, joint hypermobility
What are four types of EDS, and which ones carry the highest risk in pregnancy?
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
What is vascular (type IV) EDS associated with a risk of?
Aortic dissection/rupture
Uterine rupture
PTB
Skin fragility and poor healing
What is joint hypermobility syndrome (Type III EDS) associated with in pregnancy?
PPROM and PTL
Precipitous labour
PPH
Skin fragility and poor healing
For what type of EDS is TOP advised?
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
How is osteoporosis defined?
Bone density <2.5 (T score) standard deviations below the mean
What are the clinical manifestations of APS?
Apart from the clinical criteria
Epilepsy
Retinal vein thrombosis
Pulmonary HTN, cardiac valvular disease,
Livedo reticularis (mottled discolouration of the skin)
Osteonecrosis
Vascular dementia
In APS, what are risk factors that predict poor pregnancy outcome?
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
