SLE is a chronic autoimmune systemic inflammatory autoimmune connective disease characterized by periods of disease activity (flares) and remission with a variety of clinical and antibody patterns.
Clinical Features: Joint involvement is common (90%) but also skin (butterfly rash), Raynaud phenomen,, renal (glomerulonephritis), pleuritis, pericarditis and hematological manifestations including hemolytic anemia, thrombocytopenia, lymphopenia, or leukopenia are common.
Antiphospholipid antibodies (aPL) are found in 50% of patients, being itself not diagnostic for antiphospholipid syndrome (APS) without clinical manifestation but it increases the risk of thrombosis and 50% develops APS in the future. The principal aPL are anticardiolipin and lupus anticoagulans.
Antibodies:
ANA is found in 98% of SLE patients, 96% sensitivity and specificity. ANA is good for screening but is not enough for the diagnosis. DNA are the most specific (78% of patients) and the DNA level is associated with the activity of the disease. They may also have antibodies against extractable nuclear antigens, for example cytoplasmic ribonucleoproteins, anti RO/SSA (30% of SLE) and anti LA/SSB or to phospholipids, i.e. anticardiolipin and lupus anticoagulant (50% of patients).
0,5% prevalence anti RO/SSA in asympthomatic pregnancie.
Exacerbation of SLE (Flares) occur in up to 60% of pregnancies but better management with use of steroids may have decreased this percentage.
Risk of preeclampsia early in pregnancy.
Lupus nephritis:
Risk of deterioration is greater, the higher the baseline serum creatinine.
Woman should avoid pregnancy for 6 months after a lupus nephritis flare.
Risk: Spontaneous miscarriage 20%, fetal death, preeclampsia, and intrauterine growth retardation 45% and preterm delivery 45% are related, anticardiolipin antibodies, lupus anticoagulant, lupus nephritis or hypertension or active disease.
Exacerbation of SLE (Flares) occur in up to 60% of pregnancies but better management with use of steroids may have decreased this percentage.
Risk of preeclampsia especially early in pregnancy.
Pregnancy in women with lupus nephritis is associated with an increase risk of fetal loss (up to 75%) and worsning of renal and extrarenal manifestations.
Monitoring:
Blood pressure, renal function, blood count and RO/SSA and anti La/SSB antibodies, LA and aCL assay, anti DNA-antibodies complement (CH50 or C3 and C4)
Management:
Flares must be actively managed. NSAID and Aspirin can interfere with implantation. The woman should receive profylatic Heparin and 75 mg Aspirin if she has antiphosfolipid syndrome to prevent pregnancy loss. Corticosteroids are the drugs of choice. Prednison, and metylprednisolon cross the placenta at very low concentration, whereas dexamethason and betametason reach the fetus in higher concentrations.
Azathioprine may be used very cautiously. Cyclophosphamide and Methotrexate should be avoided, because of a high risk of causing birth defects. Antimalaria drugs is probably safe. Hydroxychloroquine should not be stopped as it may precipitate flares.
Differential Diagnosis:
Preeclampsia, lupus nephritis is often associated with proteinuria and/or active urine sediment. Lupus has rising anti-DNA antibodies and red blood cells or cellular casts in urine. A fall in complement levels and increase in complement split products particularly Ba and Bb. High rate of CH50/Ba may thus differentiate preeclampsia from those with active lupus as complement are usually but not always increased in pregnancy.
In preeclampsia, there are more common thrombocytopenia, elevated liver enzymes and uric acid.
Preeclampsia with SLE called superimposed preeclampsia (SLE (15%) are more likely to develop renal function, abnormal liver dysfunction and hyperuricemia.
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