KEY POINTS
• Immunologic diseases are common in pregnancy.
• The complications encountered during pregnancy are related to the type of autoimmune disease.
• Most patients can have a successful pregnancy outcome with a multidisciplinary team approach to their care and careful monitoring.
SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)
Background
• A disease of fluctuating immune dysfunction.
• Disease manifestations vary within a patient as well as across populations of patients.
• Pregnancy complicated by systemic lupus erythematosus (SLE) is not uncommon as SLE has a predilection for females of childbearing years.
• Ten times more common in women than men.
• More common in African Americans than Caucasians.
Evaluation
• If a patient with SLE is contemplating pregnancy or is pregnant, assessment of her disease is important. Ideally, preconceptional assessment and counseling should be performed because of the potential for adverse impact on pregnancy outcome.
• Renal insufficiency is an important risk factor for maternal and fetal complications.
• Renal function is more important than precise renal histology.
• Urinalysis, creatinine clearance, and 24-hour urine are important for counseling and pregnancy prognosis.
• Presence or absence of hypertension should be documented.
• Autoimmune antibodies associated with SLE flares and adverse outcomes should be assessed preconceptionally and in the first trimester.
• ANA
• Anti-dsDNA
• Lupus anticoagulant
• Anticardiolipin antibodies
• Anti-SS-A
• Anti-SS-B
• Baseline complement levels (C3, C4, and CH50) should be drawn.
• Important to evaluate the complete blood count and platelets in the first trimester (1).
Diagnosis
• Rarely diagnosed for the first time during pregnancy.
• Diagnosis requires a thorough history, physical exam, and laboratory tests.
• Clinical manifestations: malar rash, discoid rash, photosensitivity, oral ulcers, polyarthritis, serositis, renal disorder, neurologic disorder (seizures, psychosis, organic brain syndrome, and stroke), and hematologic disorders (hemolytic anemia, leukopenia, lymphopenia, and thrombocytopenia).
• Immunologic tests associated with SLE: antinuclear antibodies, anti-SSA (Rho), anti-SSB (La), anti-RNP, anti-Sm, anti-dsDNA, and antiphospholipid antibodies.
• Four or more of the above clinical or laboratory features must be present to establish the diagnosis of SLE (2).
• The natural history of the disease is for flares to occur.
• Diagnosis of a flare in pregnancy may be difficult (3).
• Fatigue, joint aches, or changes in the skin may mark a flare or may be symptoms of normal pregnancy.
• Some laboratory findings used to assess disease activity, such as sedimentation rate, platelet counts, and hemoglobin, are altered by pregnancy.
• May be confused with preeclampsia.
Treatment
• Is the same in pregnancy as in the nonpregnant state with a few caveats.
• Caution should always be exercised when using drugs in pregnancy because limited data are available for many drugs.
• The benefit of treatment should clearly outweigh any potential risk to the fetus.
• Immunosuppressive agents
• Glucocorticoids/corticosteroids, such as prednisone and hydrocortisone:
Are inactivated by the placenta.
Are associated with cleft lip in animals but never documented in humans
Place patients at higher risk for gestational diabetes and hypertension (3)
• Glucocorticoids/corticosteroids, such as dexamethasone and betamethasone:
Cross the placenta and should be used only if there is the intent to treat the fetus
Should be avoided because of the concern of fetal growth
• Azathioprine:
Is generally considered safe to use
Has been associated with fetal cytopenias and intrauterine growth restriction in some reports (3)
• Antimalarial agents, such as hydroxychloroquine:
In the past, these drugs have been discontinued during the pregnancy due to concerns of ophthalmologic damage.
More recent reports have not revealed any congenital, ophthalmologic, or auditory abnormalities.
Discontinuation in pregnancy may precipitate a flare (4).
• Cyclophosphamide
An alkylating agent.
First trimester use should be avoided if possible as it may be teratogenic.
Second and third trimester usage appears safer but may be associated with intrauterine growth restriction (1).
• Methotrexate
An abortifacient
Associated with specific malformations such as craniosynostosis, cleft palate, and ear and eye abnormalities (3)
• Nonsteroidal anti-inflammatory agents (NSAIDs):
• Do not appear to be teratogenic
• May be used in very limited amounts and for a short duration but should particularly be avoided in the third trimester
• Associated with premature closure of the ductus arteriosus and may lead to primary pulmonary hypertension
• Associated with fetal oliguria and subsequent oligohydramnios (1)
• Antihypertensive agents
• Important to have good blood pressure control during pregnancy, especially if the patient has preexisting renal disease.
• Antihypertensive therapy is discussed in detail in Chapter 12.
• Angiotensin-converting enzyme (ACE) inhibitors should be stopped once pregnancy is diagnosed.
• Use of ACE inhibitors in the second and third trimesters is associated with severe and irreversible fetal renal abnormalities (1).
Complications
Maternal
• Pregnancy may be inadvisable in situations such as uncontrollable hypertension, rapid deterioration of renal function, or severe neurologic or cardiopulmonary involvement due to the increased risk of maternal morbidity and mortality.
• Whether or not flares are more common in pregnancy is still debatable (1,3,5).
• Features of SLE that appear to be protective of flares and subsequent progression of disease in pregnancy are quiescent disease for 6 to 12 months preconceptionally, normal renal function, nephritis in complete remission, and normal blood pressure before conception.
• A serum creatinine of greater than 1.6 mg/dL is associated with an increased risk of hypertension, proteinuria, and further renal impairment (6).
• Multidisciplinary management is often necessary to ensure the best maternal and fetal outcome.
Pregnancy
• Preterm delivery
• Rate is 24% to 59% compared to 5% to 15% in the general population.
• Most common causes are preeclampsia, nonreassuring fetal status, and possibly a higher incidence of premature rupture of the membranes.
• Major cause of perinatal morbidity and mortality.
• Increases the risk of cesarean section (5).
• Preeclampsia
• Two-thirds of patients with preexisting renal disease will develop preeclampsia compared to only 14% of patients with SLE without renal involvement.
• It may be impossible to distinguish a lupus flare from preeclampsia.
• Clinical signs of active SLE, such as inflammatory arthritis, rash, adenopathy, and rising anti-dsDNA titers, favor the diagnosis of lupus nephritis (1).
Fetal
• Increased fetal loss rate
• Includes both first spontaneous abortions and intrauterine fetal demise.
• Median loss rate is 29% in SLE patients compared to 15% in the general population.
• Creatinine greater than 1.6 mg/dL is associated with a fetal loss rate of 45%.
• Antiphospholipid antibodies are also associated with increased fetal wastage (6).
• Late second- and third-trimester losses may be reduced with antenatal fetal testing (see Chapter 32).
• Educate patients regarding fetal kick counts at 28 weeks (1).
• Intrauterine growth restriction
• May be detected by the use of serial growth ultrasounds.
• Congenital heart block
• Most strongly associated with anti-SS-A (Ro) (1).
• Affects less than 2% of the offspring of antibody-positive patients (5).
• Can be detected by auscultation with Doppler or ultrasound.
• Characterized by persistent fetal bradycardia leading to congestive heart failure and hydrops fetalis (7).
• Mortality is at least 20%.
• Surviving infants will require a pacemaker.
• Considered the only nonreversible component of neonatal lupus erythematosus.
Neonatal
• Neonatal lupus erythematosus
• Clinical manifestations include photosensitive rash, thrombocytopenia, and liver function abnormalities.
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