Abstract
There is a scarcity of data on the management of primary Sjögren’s syndrome (pSS) in pregnancy. As there is a higher risk of complications in pregnant women with pSS, including the possibility of neonatal lupus, these women should be managed by a multidisciplinary team. At the preconceptional stage itself, there is a need to ensure that the disease activity is well controlled and the patient is not on any teratogenic drugs. It is also important to verify that there is no severe organ involvement that could adversely impact maternal and fetal health. Disease activity needs to be monitored throughout pregnancy. Baseline antibody profile (anti-Ro52, anti-Ro60, and anti-La) should be performed in all women planning pregnancy and if they are seropositive, appropriate counseling about possible risks of neonatal lupus should be done without imparting undue anxiety. Weekly fetal echocardiography is done from 16th to 28th weeks of gestation and less frequently till term hoping that impending complete heart block can be detected and treated, though evidence to support this protocol is weak. Fluorinated steroids are associated with adverse effects and have not been found to have survival advantage or decrease requirement for pacing. Routine use of fluorinated steroids should be avoided and may be considered only in certain scenarios such as recent-onset incomplete heart block and in case of myocardial involvement/hydrops. Hydroxychloroquine protects against occurrence of cardiac and cutaneous lupus and hence is a potential preventive therapy.
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References
Brito-Zerón P, Theander E, Baldini C, Seror R, Retamozo S, Quartuccio L et al (2016) Early diagnosis of primary Sjögren’s syndrome: EULAR-SS task force clinical recommendations. Expert Rev Clin Immunol 12(2):137–156
Sandhya P, Jeyaseelan L, Scofield RH, Danda D (2015) Clinical characteristics and outcome of primary Sjogren’s syndrome: a large Asian Indian cohort. Open Rheumatol J 9:36–45
Mohanasundaram K, Mani M, Chinnadurai S, Mahendran B, Balaji C, Bhoorasamy A et al (2016) Study on demography and outcome of extraglandular manifestations of primary Sjögren’s syndrome. Indian J Rheumatol 11:202–206
Seror R, Ravaud P, Bowman SJ, Baron G, Tzioufas A, Theander E et al (2010) EULAR Sjogren’s syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjogren’s syndrome. Ann Rheum Dis 69(6):1103–1109
Flint J, Panchal S, Hurrell A, van de Venne M, Gayed M, Schreiber K et al (2016) BSR and BHPR guideline on prescribing drugs in pregnancy and breastfeeding—part I: standard and biologic disease modifying anti-rheumatic drugs and corticosteroids. Rheumatology (Oxford) 55(9):1693–1697
Vassiliou VA, Moyssakis I, Boki KA, Moutsopoulos HM (2008) Is the heart affected in primary Sjögren’s syndrome? An echocardiographic study. Clin Exp Rheumatol 26(1):109–112
Mariette X, Criswell LA (2018) Primary Sjögren’s syndrome. N Engl J Med 378(10):931–939
Izmirly PM, Costedoat-Chalumeau N, Pisoni CN, Khamashta MA, Kim MY, Saxena A, Friedman D, Llanos C, Piette J-C, Buyon JP (2012) Maternal use of hydroxychloroquine is associated with a reduced risk of recurrent anti-ssa/ro-antibody–associated cardiac manifestations of neonatal lupus. Circulation 126(1):76–82
Barsalou J, Costedoat-Chalumeau N, Berhanu A, Fors-Nieves C, Shah U, Brown P et al (2018) Effect of in utero hydroxychloroquine exposure on the development of cutaneous neonatal lupus erythematosus. Ann Rheum Dis 77(12):1742–1749
Fauchais AL, Lambert M, Launay D, Michon-Pasturel U, Queyrel V, Nguyen N et al (2004) Antiphospholipid antibodies in primary Sjögren’s syndrome: prevalence and clinical significance in a series of 74 patients. Lupus 13(4):245–248
Seror R, Bootsma H, Saraux A, Bowman SJ, Theander E, Brun JG et al (2016) Defining disease activity states and clinically meaningful improvement in primary Sjögren’s syndrome with EULAR primary Sjögren’s syndrome disease activity (ESSDAI) and patient-reported indexes (ESSPRI). Ann Rheum Dis 75(2):382–389
Abbassi-Ghanavati M, Greer LG, Cunningham FG (2009) Pregnancy and laboratory studies: a reference table for clinicians. Obstet Gynecol 114(6):1326–1331
Razeghinejad MR, Nowroozzadeh MH (2010) Anti-glaucoma medication exposure in pregnancy: an observational study and literature review. Clin Exp Optom 93(6):458–465
Kumar S (2016) Neonatal lupus: an update. Indian J Rheumatol 11(Suppl S2):139–144
Pruetz JD, Miller JC, Loeb GE, Silka MJ, Bar-Cohen Y, Chmait RH (2019) Prenatal diagnosis and management of congenital complete heart block. Birth Defects Res 111(8):380–388
Brucato A (2008) Prevention of congenital heart block in children of SSA-positive mothers. Rheumatology 47(Suppl 3):iii35–iii37
Brito-Zerón P, Izmirly PM, Ramos-Casals M, Buyon JP, Khamashta MA (2015) The clinical spectrum of autoimmune congenital heart block. Nat Rev Rheumatol 11(5):301–312
Friedman DM, Kim MY, Copel JA, Davis C, Phoon CKL, Glickstein JS et al (2008) Utility of cardiac monitoring in fetuses at risk for congenital heart block: the PR Interval and Dexamethasone Evaluation (PRIDE) prospective study. Circulation 117(4):485–493
Cuneo BF, Sonesson S-E, Levasseur S, Moon-Grady AJ, Krishnan A, Donofrio MT et al (2018) Home monitoring for fetal heart rhythm during anti-Ro pregnancies. J Am Coll Cardiol 72(16):1940–1951
Rosenthal D, Druzin M, Chin C, Dubin A (1998) A new therapeutic approach to the fetus with congenital complete heart block: preemptive, targeted therapy with dexamethasone. Obstet Gynecol 92(4 Pt 2):689–691
Saleeb S, Copel J, Friedman D, Buyon JP (1999) Comparison of treatment with fluorinated glucocorticoids to the natural history of autoantibody-associated congenital heart block: retrospective review of the research registry for neonatal lupus. Arthritis Rheum 42(11):2335–2345
Eliasson H, Sonesson S-E, Sharland G, Granath F, Simpson JM, Carvalho JS et al (2011) Isolated atrioventricular block in the fetus: a retrospective, multinational, multicenter study of 175 patients. Circulation 124(18):1919–1926
Jaeggi ET, Fouron J-C, Silverman ED, Ryan G, Smallhorn J, Hornberger LK (2004) Transplacental fetal treatment improves the outcome of prenatally diagnosed complete atrioventricular block without structural heart disease. Circulation 110(12):1542–1548
Izmirly PM, Saxena A, Kim MY, Wang D, Sahl SK, Llanos C et al (2011) Maternal and fetal factors associated with mortality and morbidity in a multi-racial/ethnic registry of anti-SSA/Ro-associated cardiac neonatal lupus. Circulation 124(18):1927–1935
Fredi M, Andreoli L, Bacco B, Bertero T, Bortoluzzi A, Breda S et al (2019) First report of the Italian Registry on immune-mediated congenital heart block (Lu.Ne Registry). Front Cardiovasc Med 6:11
Levesque K, Morel N, Maltret A, Baron G, Masseau A, Orquevaux P et al (2015) Description of 214 cases of autoimmune congenital heart block: results of the French neonatal lupus syndrome. Autoimmun Rev 14(12):1154–1160
Van den Berg NWE, Slieker MG, van Beynum IM, Bilardo CM, de Bruijn D, Clur SA et al (2016) Fluorinated steroids do not improve outcome of isolated atrioventricular block. Int J Cardiol 225:167–171
Izmirly PM, Saxena A, Sahl SK, Shah U, Friedman DM, Kim MY et al (2016) 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 75(6):1161–1165
Ciardulli A, D’Antonio F, Magro-Malosso ER, Saccone G, Manzoli L, Radolec M et al (2019) Maternal steroid therapy for fetuses with immune-mediated complete atrioventricular block: a systematic review and meta-analysis. J Matern Fetal Neonatal Med 32(11):1884–1892
Friedman DM, Kim MY, Copel JA, Llanos C, Davis C, Buyon JP (2009) Prospective evaluation of fetuses with autoimmune-associated congenital heart block followed in the PR Interval and Dexamethasone Evaluation (PRIDE) Study. Am J Cardiol 103(8):1102–1106
Brucato A, Tincani A, Fredi M, Breda S, Ramoni V, Morel N et al (2017) Should we treat congenital heart block with fluorinated corticosteroids? Autoimmun Rev 16(11):1115–1118
Ruffatti A, Cerutti A, Favaro M, Del Ross T, Calligaro A, Hoxha A et al (2016) Plasmapheresis, intravenous immunoglobulins and betamethasone—a combined protocol to treat autoimmune congenital heart block: a prospective cohort study. Clin Exp Rheumatol 34(4):706–713
Hussein SZ, Jacobsson LTH, Lindquist PG, Theander E (2011) Pregnancy and fetal outcome in women with primary Sjogren’s syndrome compared with women in the general population: a nested case-control study. Rheumatology 50(9):1612–1617
De Carolis S, Salvi S, Botta A, Garofalo S, Garufi C, Ferrazzani S et al (2014) The impact of primary Sjogren’s syndrome on pregnancy outcome: our series and review of the literature. Autoimmun Rev 13(2):103–107
Skopouli FN, Papanikolaou S, Malamou-Mitsi V, Papanikolaou N, Moutsopoulos HM (1994) Obstetric and gynaecological profile in patients with primary Sjögren’s syndrome. Ann Rheum Dis 53(9):569–573
Ishihara O, Saitoh M, Hayashi N, Kinoshita K, Takeuchi T (2001) Successful treatment of embryo implantation failure in patients with the Sjögren syndrome with low-dose prednisolone. Fertil Steril 75(3):640–641
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Sandhya, P. (2020). Management of Sjögren’s Syndrome During Pregnancy. In: Sharma, S. (eds) Women's Health in Autoimmune Diseases. Springer, Singapore. https://doi.org/10.1007/978-981-15-0114-2_18
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