Systemic lupus erythematosus

During pregnancy

Aboubakr Elnashar

Benha university, Egypt

Incidence Women > men (ratio 9:1)

During the child-bearing years (ratio 15:1).

Incidence: 1 in 1000 women

Aboubakr Elnashar

Clinical features Systemic connective tissue disease

Heterogeneous: variety of clinical& antibody

patterns.

Other autoimmune disorders: 6%.

Periods of disease activity (flares)& remissions.

Average age at diagnosis: 30 y

Aboubakr Elnashar

Commonest clinical feature (90%):

Arthritis:

non­erosive, peripheral, tenderness& swelling.

Other features:

Skin: (80%):

malar rash

Photosensitivity

vasculitic lesions on the "fingertips& nail folds,

Raynaud's phenomenon

Discoid lupus.

Aboubakr Elnashar

Serositis:

pleuritis, pericarditis

Renal:

glomeru­lonephritis with proteinuria& cellular casts

Neurological:

psychosis, seizures or chorea.

Haematological:

haemolytic anaemia, thrombocytopenia &

lymphopenia or leukopenia.

Aboubakr Elnashar

Pathogenesis Cause: Un known

Genetic predisposition

Environmental triggers e.g. ultraviolet light or viral

infection.

Immunological:

polyclonal B-cell activation

impaired T-cell regulation of the immune response

failure to remove immune complexes.

Circulating non-organ-specific autoantibodies.

Deposition of immune complexes: vasculitis.

Aboubakr Elnashar

Diagnosis American Rheumatic Association criteria

Many patients have a lupus-like illness without

fulfilling these.

1. CBC:

Normochromic normocytic anaemia

Neutropenia

Thrombocytopenia.

2. ESR:

raised {high immunoglobulin levels}

3. CRP:

Normal

4. 3rd or 4th components of complement:

low or falling: active disease. Aboubakr Elnashar

5. Auto-antibody

a. The most common:

Antinuclear antibody (ANA): (96%)

Titres do not change with disease activity.

b. The most specific

antibodies to double-stranded DNA (78%) and

Smith (Sm).

Glomerulonephritis occurs more frequently in

women with these antibodies.

c. Other

anti-Ro and anti-La: (30%)

anticardiolipin antibodies (aPLs): (40%).

Aboubakr Elnashar

Effect of pregnancy on SLE

Flares:

Increases:

from 40% to 60%.

When:

at any stage of pregnancy or the puerperium

more likely immediately postpartum: little evidence

Prediction:

not possible

more likely if disease has been active within 6 ms of

conception.

Aboubakr Elnashar

Diagnosis during pregnancy:

difficult

{many features such as hair loss, oedema, palmar &

facial erythema, fatigue, anaemia, raised ESR &

musculoskeletal pain also occur in normal

pregnancy}.

most commonly involving the skin& joints

Prevention:

prophylactic steroids or routine increases of dose:

no effect & not recommended

Aboubakr Elnashar

Women with lupus nephritis

SLE nephropathy may manifest for the first time in

pregnancy.

Risk of deterioration

Moderate renal impairment (serum creatinine 125-

200 umol/I): uncomplicated pregnancies.

High baseline serum creatinine: Inc risk of

deterioration

Aboubakr Elnashar

Preconception counseling.

1. Delay pregnancy until at least 6 ms after a lupus

nephritis flare.

2. Prediction of the risks to the woman& fetus:

anti-Ro/La

aPLs

Renal function

Blood pressure

Aboubakr Elnashar

Effect of SLE on pregnancy 1. The increased risks

spontaneous miscarriage

fetal death

pre-eclampsia

preterm delivery

IUGR

Related to

1. Age, parity

2. anticardiolipin antibodies

3. lupus anticoagulant

4. lupus nephritis

5. hypertension

6. active disease at the time of conception or

7. first presentation of SLE during pregnancy. Aboubakr Elnashar

During pregnancy, lupus

improves in a third of women

remains unchanged in a third

worsens in the remaining third.

Clinical condition can worsen or flare without

warning (Khamashta and colleagues, 1997).

Risk of major morbidity during pregnancy

7% (Petri, 1998)

Lupus can be life threatening to both the mother

and her fetus-infant.

Aboubakr Elnashar

Good pregnancy outcome if:

1. Lupus activity has been quiescent for at least 6

months before conception

2. No active renal involvement manifest by

proteinuria or renal dysfunction

3. Superimposed preeclampsia does not develop

4. No evidence of antiphospholipid antibody

activity.

Aboubakr Elnashar

Renal lupus:

increased risk of fetal loss, PET, IUGR

particularly if there is hypertension or proteinuria.

Women in remission, but without hypertension,

renal involvement or aPLs:

Risk of pregnancy loss& PET is not higher than in

the general population.

2. Chorea

very rare complication of pregnancy in women with

SLE or aPLs.

Aboubakr Elnashar

Management Preconception counseling

1. Prediction of the risks to the woman& fetus:

anti-Ro/La

aPLs

Renal

Blood pressure.

2. Outcome is improved if conception occurs during

disease remission.

Aboubakr Elnashar

Multidisciplinary team in combined clinics:

Physicians& obstetricians

Monitor

1. disease activity

2. Fetal growth

3. uterine artery Doppler blood flow at 20-24 w

4. umbilical artery blood flow from 24 w

Aboubakr Elnashar

Baseline values in early pregnancy:

1. FBC

2. KFT:

U, creatinine, uric acid, quantify any proteinuria.

3. LFT:

4. anti DNA

5. Complement titres

5. Electrolytes

Serial measurements

at intervals dependent on disease severity

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Disease flare

Symptoms:

Arthralgia, pleuritic pain, skin rash

Lab:

A. Urine: Red blood cells or cellular casts

B. Rising anti-DNA antibody titre

C. Fall in complement levels

>25% fall in C3 or C4 suggests active SLE.

N.B. elevation of complement split products,

particularly Ba & Bb, often accompanies flares,

so high ratios of CH50: Ba may differentiate

PET from active lupus.

Aboubakr Elnashar

Treatment

Corticosteroids are the drugs of choice.

Aboubakr Elnashar

Hydroxychloroquine (Plaquenil)

should be continued {stopping may precipitate

flare}.

For control of hypertension:

Drug of choice: methyldopa

2nd -line agents: nifedipine or hydralazine

Although long-term hydralazine& methyldopa use

may rarely induce a SLE-like syndrome, they

are not contraindicated in SLE.

Aboubakr Elnashar

Differentiation of active renal lupus from PET

Difficult

1. The two conditions may be superimposed.

2. Hypertension, proteinuria, thrombocytopenia&

even renal impairment are all features of PET

3. A doubling of baseline proteinuria may be

expected in pregnancy but more than this would be

indicative of either worsening lupus nephritis or

PET}

Aboubakr Elnashar

How:

1. Hyperuricaemia & abnormal LFT point more

towards PET.

2. Renal biopsy

The only definitive investigation

Rarely undertaken in pregnancy.

More likely to be appropriate prior to fetal viability

Aboubakr Elnashar

TT of active lupus nephritis

Increase oral prednisolone

Pulsed IV methyl predisolone

Azathioprine (Imuran).

Cyclophos­phamide: Rarely used

Aboubakr Elnashar

If lupus flare & PET cannot be dd beyond 24-28

w, when the fetus is viable:

Delivery

{cure PET

allow administration of cyclophosphamide}

Aboubakr Elnashar

Conclusions There is an increased rate of flare during

pregnancy.

Disease flares must be actively managed with

corticosteroids.

Adverse pregnancy outcome is related to the

presence of renal involvement, hypertension,

antiphospholipid antibodies& disease activity at

the time of conception.

These factors increase the risks of spontaneous

miscarriage, fetal death, PET, PTL& IUGR.

Aboubakr Elnashar

Pregnancy care is best undertaken in combined

clinics allowing close monitoring of disease

activity, fetal growth& well-being.

In Ro-positive mothers

risk of transient neonatal cutaneous lupus: 5%

risk of CHB: 2%.

Aboubakr Elnashar

Aboubakr Elnashar

Thanks