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Autoimmune Disorders in Pregnancy

By

Lara A. Friel

, MD, PhD, University of Texas Health Medical School at Houston, McGovern Medical School

Reviewed/Revised Sep 2023
View PATIENT EDUCATION

Systemic Lupus Erythematosus in Pregnancy

Systemic lupus erythematosus (SLE) Systemic Lupus Erythematosus (SLE) Systemic lupus erythematosus is a chronic, multisystem, inflammatory disorder of autoimmune etiology, occurring predominantly in young women. Common manifestations may include arthralgias and... read more Systemic Lupus Erythematosus (SLE) may first appear during pregnancy; women who have had an unexplained 2nd-trimester stillbirth, a fetus with growth restriction, preterm delivery, or recurrent spontaneous abortions may later be diagnosed with SLE.

The course of preexisting SLE during pregnancy cannot be predicted, but SLE may worsen, particularly immediately postpartum. Outcomes are better if conception can be delayed until the disorder has been inactive for at least 6 months, the drug regimen has been adjusted in advance, and blood pressure and renal function are normal.

Significant preexisting renal or cardiac complications increase risk of maternal morbidity and mortality. Diffuse nephritis, hypertension, or the presence of circulating antiphospholipid antibodies (usually anticardiolipin antibody or lupus anticoagulant) increases risk of perinatal mortality. Neonates may have anemia, thrombocytopenia, or leukopenia; these disorders tend to resolve during the first weeks after birth when maternal antibodies disappear.

If hydroxychloroquine was used before conception, it may be continued throughout pregnancy. SLE flares are usually treated with low-dose prednisone, IV pulse methylprednisolone, hydroxychloroquine, and/or azathioprine. High-dose prednisone and cyclophosphamide increase obstetric risks and are thus reserved for severe lupus complications.

Reference

Antiphospholipid Syndrome in Pregnancy

APS is caused by autoantibodies to certain phospholipid-binding proteins that would otherwise protect against excessive coagulation activation.

Diagnosis

  • Measurement of circulating antiphospholipid antibodies

  • Clinical criteria

Antiphospholipid syndrome is suspected in women with a history of any of the following:

  • ≥ 3 unexplained embryonic losses (before 10 weeks gestation) or ≥ 1 unexplained fetal losses (after 10 weeks)

  • Prior unexplained arterial or venous thromboembolism

  • New arterial or venous thromboembolism during pregnancy

Antiphospholipid syndrome is diagnosed by measuring levels of circulating antiphospholipid antibodies (anticardiolipin, beta-2 glycoprotein I, lupus anticoagulant) with positive results on ≥ 2 occasions 12 weeks apart.

Diagnosis of antiphospholipid syndrome requires ≥ 1 clinical criterion in addition to ≥ 1 of the laboratory criteria above. Clinical criteria can be vascular (prior unexplained arterial or venous thromboembolism in any tissue) or pregnancy-related. Pregnancy-related criteria include the following (1 Diagnosis reference Autoimmune disorders are 5 times more common among women, and incidence tends to peak during reproductive years. Thus, these disorders commonly occur in pregnant women. Systemic lupus erythematosus... read more ):

  • ≥ 1 unexplained deaths of a morphologically normal (via ultrasonography or direct examination) fetus at ≥ 10 weeks gestation

  • ≥ 1 premature births of a morphologically normal neonate at ≤ 34 weeks gestation because of eclampsia or severe preeclampsia or with features of placental insufficiency

  • ≥ 3 unexplained consecutive spontaneous pregnancy losses at < 10 weeks gestation, with maternal anatomic and hormonal abnormalities and paternal and maternal chromosomal causes excluded

Diagnosis reference

  • 1. Miyakis S, Lockshin MD, Atsumi T, et al: International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 4(2):295-306, 2006. doi:10.1111/j.1538-7836.2006.01753.x

Treatment

  • Prophylaxis with anticoagulants and low-dose aspirin

Women with antiphospholipid syndrome are usually treated prophylactically with anticoagulants and with low-dose aspirin during pregnancy and for 6 weeks postpartum.

Patients should be monitored closely and referred to a maternal-fetal medicine specialist as needed.

Immune Thrombocytopenia in Pregnancy

Immune thrombocytopenia (ITP) Immune Thrombocytopenia (ITP) Immune thrombocytopenia (ITP) is a bleeding disorder usually without anemia or leukopenia. Typically, it is chronic in adults, but it is usually acute and self-limited in children. Spleen size... read more Immune Thrombocytopenia (ITP) , mediated by maternal antiplatelet IgG, tends to worsen during pregnancy and increases risk of maternal morbidity. ITP is characterized by isolated thrombocytopenia in the absence of other etiologies, making it a diagnosis of exclusion.

Corticosteroids reduce IgG levels and cause remission in most women, but improvement is sustained in only 50%. Immunosuppressive therapy and plasma exchange further reduce IgG, increasing platelet counts. Rarely, splenectomy is required for refractory cases; it is best done during the 2nd trimester, when it causes sustained remission in about 80%.

IV immune globulin increases platelet count significantly but briefly, so that labor can be induced in women with low platelet counts. Platelet transfusions are recommended only when

Although antiplatelet IgG can cross the placenta, it only very rarely causes fetal or neonatal thrombocytopenia. Maternal antiplatelet antibody levels (measured by direct or indirect assay) cannot predict fetal involvement. Risk of neonatal intracranial hemorrhage due to maternal ITP is not affected by the mode of delivery nor by birth trauma. Accordingly, the current accepted practice is vaginal delivery, without routinely determining the fetal platelet count, and cesarean delivery only for obstetric indications (3 References Autoimmune disorders are 5 times more common among women, and incidence tends to peak during reproductive years. Thus, these disorders commonly occur in pregnant women. Systemic lupus erythematosus... read more , 4 References Autoimmune disorders are 5 times more common among women, and incidence tends to peak during reproductive years. Thus, these disorders commonly occur in pregnant women. Systemic lupus erythematosus... read more , 5 References Autoimmune disorders are 5 times more common among women, and incidence tends to peak during reproductive years. Thus, these disorders commonly occur in pregnant women. Systemic lupus erythematosus... read more ).

References

  • 1. Kaufman RM, Djulbegovic B, Gernsheimer T, et al: Platelet transfusion: a clinical practice guideline from the AABB. Ann Intern Med 162(3):205-213, 2015. doi:10.7326/M14-1589

  • 2. Bussel JB, Hou M, Cines DB: Management of primary immune thrombocytopenia in pregnancy. N Engl J Med 389(6):540-548, 2023. doi:10.1056/NEJMra2214617

  • 3. ACOG Practice Bulletin No. 207: Thrombocytopenia in Pregnancy. Obstet Gynecol 133(3):e181-e193, 2019. doi:10.1097/AOG.0000000000003100

  • 4. Provan D, Stasi R, Newland AC, et al: International consensus report on the investigation and management of primary immune thrombocytopenia. Blood 115(2):168-186, 2010. doi:10.1182/blood-2009-06-225565

  • 5. Neunert C, Lim W, Crowther M, et al: The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood 117(16):4190-4207, 2011. doi:10.1182/blood-2010-08-302984

Rheumatoid Arthritis in Pregnancy

Rheumatoid arthritis (RA) Rheumatoid Arthritis (RA) Rheumatoid arthritis is a chronic systemic autoimmune disease that primarily involves the joints. Rheumatoid arthritis causes damage mediated by cytokines, chemokines, and metalloproteases.... read more Rheumatoid Arthritis (RA) may begin during pregnancy or, even more often, during the postpartum period. Preexisting RA generally abates temporarily during pregnancy. The fetus is not directly affected, but delivery may be difficult if the woman’s hip joints or lumbar spine is affected. Cesarean delivery is significantly more common in women who have moderate or high disease activity in pregnancy compared to those with low disease activity. Furthermore, a postpartum flare can impair the ability of women with rheumatoid arthritis to take care of themselves and their infant.

If a woman develops an RA flare during pregnancy, first-line treatment usually begins with prednisone. For refractory cases, other immunosuppressants may be required (1 Reference Autoimmune disorders are 5 times more common among women, and incidence tends to peak during reproductive years. Thus, these disorders commonly occur in pregnant women. Systemic lupus erythematosus... read more ).

Reference

  • 1. ACOG Committee Opinion No. 776: Immune Modulating Therapies in Pregnancy and Lactation. Obstet Gynecol 133(4):e287-e295, 2019. doi:10.1097/AOG.0000000000003176

Myasthenia Gravis in Pregnancy

Myasthenia gravis Myasthenia Gravis Myasthenia gravis is characterized by episodic muscle weakness and easy fatigability caused by autoantibody- and cell-mediated destruction of acetylcholine receptors. It is more common among... read more varies in its course during pregnancy and even between pregnancies in the same woman. The diagnosis is made after clinical and physical examination reveals muscle weakness and is confirmed by serum immunoassays of autoantibody levels.

Frequent acute myasthenic episodes may require increasing doses of anticholinesterase medications (eg, neostigmine), which may cause symptoms of cholinergic excess (eg, abdominal pain, diarrhea, vomiting, increasing weakness); atropine may then be required. Sometimes myasthenia becomes refractory to standard therapy and requires corticosteroids or immunosuppressants.

During labor, women may need assisted ventilation and are extremely sensitive to medications that depress respiration (eg, sedatives, opioids, magnesium sulfate). Therefore, regional anesthesia is preferred to intravenous medications for pain control during labor and delivery. Because the IgG responsible for myasthenia crosses the placenta, transient myasthenia occurs in 20% of neonates (1 Reference Autoimmune disorders are 5 times more common among women, and incidence tends to peak during reproductive years. Thus, these disorders commonly occur in pregnant women. Systemic lupus erythematosus... read more ), even more if mothers have not had a thymectomy. Although vaginal delivery is recommended, an assisted vaginal delivery may be required due to weakness of striated muscle.

Reference

Drugs Mentioned In This Article

Drug Name Select Trade
Plaquenil, Quineprox, SOVUNA
Deltasone, Predone, RAYOS, Sterapred, Sterapred DS
A-Methapred, Depmedalone-40, Depmedalone-80 , Depo-Medrol, Medrol, Medrol Dosepak, Solu-Medrol
Azasan, Imuran
Cyclophosphamide, Cytoxan, Neosar
Anacin Adult Low Strength, Aspergum, Aspir-Low, Aspirtab , Aspir-Trin , Bayer Advanced Aspirin, Bayer Aspirin, Bayer Aspirin Extra Strength, Bayer Aspirin Plus, Bayer Aspirin Regimen, Bayer Children's Aspirin, Bayer Extra Strength, Bayer Extra Strength Plus, Bayer Genuine Aspirin, Bayer Low Dose Aspirin Regimen, Bayer Womens Aspirin , BeneHealth Aspirin, Bufferin, Bufferin Extra Strength, Bufferin Low Dose, DURLAZA, Easprin , Ecotrin, Ecotrin Low Strength, Genacote, Halfprin, MiniPrin, St. Joseph Adult Low Strength, St. Joseph Aspirin, VAZALORE, Zero Order Release Aspirin, ZORprin
BLOXIVERZ, Prostigmin
Atreza, Atropine Care , Atropisol , Isopto Atropine, Ocu-Tropine, Sal-Tropine
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NOTE: This is the Professional Version. CONSUMERS: View Consumer Version
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