SLE and antiphospholipid syndrome in pregnancy

A 32-year-old with lupus nephritis walks into your booking clinic at eight weeks. She is on mycophenolate because her rheumatologist "wanted her stable before she got pregnant." She is now pregnant, frightened, and asking you what to do. The next ten minutes decide whether this pregnancy is planned and protected or drifts into a flare, a fetal loss, or a baby with congenital heart block. This topic is those ten minutes.^[4]

Overview and definition

SLE is a multisystem autoimmune disease of women of reproductive age, and pregnancy turns up the volume on every risk it carries — flare, thrombosis, nephritis, preeclampsia, fetal loss, growth restriction, and a unique neonatal syndrome caused by antibody transfer across the placenta.^[4][5]

Antiphospholipid syndrome (APS) is the thrombophilic cousin defined by antibodies to phospholipid-binding proteins that cause vascular thrombosis and the characteristic pattern of pregnancy loss. APS may be primary (no underlying autoimmune disease) or secondary to SLE — but the antibody, the criteria and the obstetric risk are the same.^[1][2]

One fact frames the whole topic: outcomes track disease activity at conception. A lupus pregnancy conceived in quiescent disease on pregnancy-safe drugs is, for most women, a successful pregnancy. A lupus pregnancy conceived in active disease, on teratogenic drugs, or without hydroxychloroquine is a high-risk pregnancy from day one. Pre-conception planning is not a nicety — it is the intervention.^[4][14]

References14ShowHide

1. [1]Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS) J Thromb Haemost, 2006.PMID 16420554
2. [2]Tektonidou MG, Andreoli L, Limper M, Amoura Z, Cervera R, Costedoat-Chalumeau N, et al. EULAR recommendations for the management of antiphospholipid syndrome in adults Ann Rheum Dis, 2019.PMID 31092409
3. [3]Sammaritano LR, Bermas BL, Chakravarty EE, Chambers C, Clowse MEB, Lockshin MD, et al. 2020 American College of Rheumatology Guideline for the Management of Reproductive Health in Rheumatic and Musculoskeletal Diseases Arthritis Rheumatol, 2020.PMID 32090480
4. [4]Lateef A, Petri M Systemic Lupus Erythematosus and Pregnancy Rheum Dis Clin North Am, 2017.PMID 28390564
5. [5]Lucas A, Eudy AM, Gladman D, Petri M, Urowitz M, Wyatt CM, Clowse ME The association of lupus nephritis with adverse pregnancy outcomes among women with lupus in North America Lupus, 2022.PMID 36017607
6. [6]Kim MY, Buyon JP, Guerra MM, Rana S, Zhang D, Laskin CA, Petri M, Lockshin MD, Sammaritano LR, Branch DW, Porter TF, Merrill JT, Stephenson MD, Gao Q, Karumanchi SA, Salmon JE Angiogenic factor imbalance early in pregnancy predicts adverse outcomes in patients with lupus and antiphospholipid antibodies: results of the PROMISSE study Am J Obstet Gynecol, 2016.PMID 26432463
7. [7]Levy RA, Vilela VS, Cataldo MJ, et al. Hydroxychloroquine (HCQ) in lupus pregnancy: double-blind and placebo-controlled study Lupus, 2001.PMID 11434574
8. [8]Izmirly PM, Costedoat-Chalumeau N, Pisoni CN, Khamashta MA, Kim MY, Saxena A, Friedman D, Llanos C, Piette JC, Buyon JP Maternal use of hydroxychloroquine is associated with a reduced risk of recurrent anti-SSA/Ro-antibody-associated cardiac manifestations of neonatal lupus 2012.PMID 22626746
9. [9]Izmirly P, Kim M, Friedman DM, Costedoat-Chalumeau N, Clancy R, Copel JA, Phoon CKL, Cuneo BF, Cohen RE, Robins K, Masson M, Wainwright BJ, Zahr N, Saxena A, Buyon JP Hydroxychloroquine to Prevent Recurrent Congenital Heart Block in Fetuses of Anti-SSA/Ro-Positive Mothers J Am Coll Cardiol, 2020.PMID 32674792
10. [10]Izmirly PM, Saxena A, Sahl SK, et al. Assessment of fluorinated steroids to avert progression and mortality in anti-SSA/Ro-associated cardiac injury limited to the fetal conduction system Ann Rheum Dis, 2016.PMID 26835701
11. [11]Ziakas PD, Pavlou M, Voulgarelis M Heparin treatment in antiphospholipid syndrome with recurrent pregnancy loss: a systematic review and meta-analysis Obstet Gynecol, 2010.PMID 20502298
12. [12]Hladunewich MA, Bramham K, Jim B, et al. Managing glomerular disease in pregnancy Nephrol Dial Transplant, 2017.PMID 28391337
13. [13]Blom K, Odutayo A, Bramham K, Hladunewich MA Pregnancy and Glomerular Disease: A Systematic Review of the Literature with Management Guidelines Clin J Am Soc Nephrol, 2017.PMID 28522651
14. [14]Sims CA, Eudy AM, Doss J, et al. The impact of pregnancy planning and medical readiness on reproductive outcomes in women with systemic lupus erythematosus Lupus, 2023.PMID 37966369