Rheumatic and connective tissues disorders in pregnancy

Question 1

Rheumatoid arthritis clinical manifestations

Answer 1

• Clinical manifestations: morning stiffness, pain, swelling of peripheral joints; erosive arthritis of wrist, knees, shoulders, hand joints
• Rheumatoid nodules (20%) on extensor surfaces of forearm
• Rarely: pericarditis, myocarditis, endocarditis, or vasculitis
• Unlike SLE, rare to have renal involvement
• 90% ultimately test positive for rheumatoid factor

Question 2

Rheumatoid arthritis- mainstay medications

Answer 2

Question 3

Rheumatoid arthritis and pregnancy

Answer 3

Interaction with pregnancy:
o Improves in pregnancy (50-75% of patients); relapse in pp (75%); no long term effect on progression
o No adverse effect on pregnancy; no need for increased fetal surveillance

Question 4

Diagnosis of SLE

Answer 4

Question 5

Risks of SLE in pregnancy

Answer 5

Risks in pregnancy
o Maternal: exacervation, nephritis, PEC
o Fetal: SAB, IUFD, IUGR, PTD, PPROM, neonatal lupus

• SSA and SSB:
  o SSA 25% of SLE
  o SSB 12% of SLE
  o Risk of heart block (most cases occur 16-24 weeks)
   SSA = 2%
   Both positive = 3%
   Previous child with heart block = 16%
  o SSA primarily associated with neonatal lupus (1/15,000 births)
   SSA  20% risk of neonatal lupus
   Neonatal lupus  75-90% mothers have SSA; primarily dermatologic until antibodies clear up (6 months)
• Recurrence rate = 25%

Question 6

What is SLE nephritis and what are the complications in pregnancy

Answer 6

• Complications of nephritis:
  o Cr > 1.5 – relative contraindication to pregnancy; Cr > 2 = absolute contraindication to pregnancy
  o Effect of pregnancy on renal function:
   Transient deterioration 17%; permanent deterioration 8%
  o PEC: 2/3 develop PEC (vs 20% with no renal disease and SLE)
   Differentiation:
• PEC = elevated LFTs, decreased AT III
• SLE/LN flare: cellular casts and hematuria
  o Very low complement levels (vs mild decrease with PEC)

Question 7

Management of See in pregnancy and therapies

Answer 7

Management:
o Serology: LAC< ACA, anti SSA and SSB – if SSA/SSB positive – echo in 2nd trimester
o Urine: 24 hour; serially if nephritis
o Growth q 4 weeks; NST at 28 weeks
o Visits q 1-2 weeks; then weekly after 24 weeks

Question 8

Management of See in pregnancy and therapies

Answer 8

Management:
o Serology: LAC< ACA, anti SSA and SSB – if SSA/SSB positive – echo in 2nd trimester
o Urine: 24 hour; serially if nephritis
o Growth q 4 weeks; NST at 28 weeks
o Visits q 1-2 weeks; then weekly after 24 weeks

Question 9

Scleroderma and pregnancy

Answer 9

• Skin fibrosis, Raynaud phenomenon
• Fetal risk: SAB, PTB, IUGR
• Surveillance: simiarl to SLE
• Check SSA/SSB

Question 10

Dermatomyosities and pregnancy

Answer 10

• Findings: periorbital heliotrope rash; proximal muscle weakness, can affect joints, esophagus, lungs; increased risk for malignancy after age 60
   Risk in pregnancy no significantly elevated; Serial US/NSTs in 3rd trimester
  o Treatment in pregnancy:
   Myositis: steroids, azathioprine
   Skin: sun avoidance, hydroxychloroquine

Question 11

Poyarteritis Nodosa

Answer 11

• Findings: necrotizing vasculitis primary of medium vessels; 10% have hepatitis B
• Maternal risk: not increased unless new diagnosis
• Treatment: corticosteroids used in mild disease; cytoxan (cyclophosphamide) in severe cases – contraindicated in pregnancy

Question 12

What percentage of patients with polyateritis nodes have hepatitis B?

Answer 12

10%

Question 13

APLS LAC

Answer 13

• LAC: most specific; less sensitive
  o Antibodies to phospholipids
  o In vitro: increase clotting time; in vivo: increase clotting
  o Assays: LAC sensitive aPTT, dilue Russell’s viper venom time (DRVVT)
• 5% of healthy controls have ACA
• 35% patients with SLE have ACA

Question 14

APLS B2-glycoprogein antibody

Answer 14

• Beta 2 glycoprotein I (B2GLPI)
  o Antibodies to it exert lupus anticoagulant activity

Question 15

APLS B2-glycoprogein antibody

Answer 15

• Beta 2 glycoprotein I (B2GLPI)
  o Antibodies to it exert lupus anticoagulant activity

Question 16

What are the risks of APLS and management in pregnancy

Answer 16

• OB risks: IUGR (30%), GHTN (30%), PTD (30%)
• Risk of thrombosis in pregnancy with APS is 5-12%; lifetime prevalence of thrombosis in APS = 30%
• Management:
  o Anticoagulation: antepartum = prophylactic heparin + baby ASA; postpartum = treat for 6 weeks
  o Delivery at 39 weeks; serial US; NSTs at 32 weeks
  o LMWH not shown to reduce pregnancy loss vs baby ASA alone; USE HEPARIN

Question 17

What are the risks of APLS and management in pregnancy

Answer 17

• OB risks: IUGR (30%), GHTN (30%), PTD (30%)
• Risk of thrombosis in pregnancy with APS is 5-12%; lifetime prevalence of thrombosis in APS = 30%
• Management:
  o Anticoagulation: antepartum = prophylactic heparin + baby ASA; postpartum = treat for 6 weeks
  o Delivery at 39 weeks; serial US; NSTs at 32 weeks
  o LMWH not shown to reduce pregnancy loss vs baby ASA alone; USE HEPARIN

Question 18

ITP

Answer 18

• Pregnancy dose not cause or worsen ITP
• Autoimmune destruction; IgG binds platelets targeting them for destruction by RE system
• Testing for antibodies not recommended; does nto distinguish ITP from gest thrombo or PEC
• Gestational Thrombo
  o Accounts for 70% thrombocytopenia; 99% have plt > 100K; no sequelae
• No risk if plt > 50K
  o Treat if plt < 30K or bleeding antenatally
  o Near term, treat if < 50K or bleeding
• Maternal therapy does not prevent fetal thrombocytopenia
  o Cordocentesis, scalp sampling, CD do not prevent neonatal bleeding; avoid operative vag delivery

Question 19

What is treatment of ITP in pregnancy

Answer 19

Question 20

What is the risk of recurrent heart block?

Answer 20

16%