Sjögren’s syndromes mother’s baby with neonatal lupus syndrome

Yıl 2012, Cilt: 22 Sayı: 2, 67 - 70, 01.01.2012

Adem Küçük Sinan Bağçacı İlknur Albayrak Ali Baykan Sami Küçükşen Küçükşen

Öz

Objective: We aimed to present a baby developing neonatal lupus syndrome whose mother is Sjögren's syndrome. Case report: Neonatal lupus syndrome NLS develops due to transmission of maternal antibodies from a mother with autoimmun disorders like Sjögren’s syndrome and other disorders. Cardiac involvement occurs due to the transmission of anti-Ro/SSa and anti-La/SSb antibodies in patients. In this article, we presented a baby developing congenital heart block and treated successfully by constant pacemaker. Conclusion: The risk of NLS should be kept in mind during pregnancy in the women with anti-Ro/SSA and anti-La/SSB antibodypositive SS. When fetal bradycardia is detected heart block should be considered. Appropriate treatment in intrauterine and neonatal period should be planned

Anahtar Kelimeler

Sjögren’s syndrome, neonatal lupus syndrome, cardiac involvement

Sjögren sendromlu annenin neonatal lupus sendromlu bebeği

Öz

Amaç: Sjögren sendromlu annenin neonatal lupus sendromu gelişen bebeğini olgu olarak sunmayı amaçladık. Olgu sunumu: Neonatal lupus sendromu NLS , Sjögren Sendromu SS ve diğer otoimmün hastalığı olan annelerin bebeklerinde anti-Ro/SSA ve anti-La/SSB antikorlarının geçişine bağlı olarak başta kardiyak tutulum oluşur. Bu yazıda SS’li annenin konjenital kalp bloğu tanısı alan ve kalıcı kalp pili uygulaması ile başarılı bir şekilde tedavi edilen NLS’li bebeği vaka olarak sunulmuştur. Sonuç: Anti-Ro/SSA ve anti-La/SSB antikoru pozitif SS gibi hastalığı olan kadınlarda gebelik boyunca NLS riski mutlaka akılda tutulmalı, fetal bradikardi saptandığında kalp bloğu akla gelmeli, intrauterin ve neonatal dönemde uygun tedavi planlanmalıdır

Anahtar Kelimeler

Sjögren sendromu, neonatal lupus sendromu, kalp tutulumu

Kaynakça

• Kassan SS, Moutsopoulos HM. Clinical manifestations and early diagnosis of Sjögren syndrome. Arch Intern Med. 2004;164:1275-84.
• Tincani A, Rebaioli CB, Taglietti M, Shoenfeld Y. Heart involvement in systemic lupus erythematosus, anti- phospholipid syndrome and neonatal lupus. Rheumatology. 2006; 45: 8-13.
• Jenkins RE, Kurwa AR, Atherton DJ, Black MM. Neonatal lupus erythematosus. Clin Exp Dermatol. 1994;19:409-11.
• Buyon JP. Neonatal lupus syndrome. www.utdol.com. UpToDate online version 17.3. Last Updated October 16, 2008. Accessed 1 December 2009.
• Kurosaki K, Miyazaki A, Watanabe K, Echigo S. Long-term outcome of isolated congenital complete atrioventricular block pacing since neonatal period: experience at a single Japanese institution. Circ J. 2008;72:81-7.
• Eftekhari P, Sallé L, Lezoualc'h F, Mialet J, Gastineau M, Briand JP, et al. Anti-SSA/Ro52 autoantibodies blocking the cardiac 5-HT4 serotoninergic receptor could explain neonatal lupus congenital heart block. Eur J Immunol. 2000;30:2782- 90.
• Jayaprasad N, Johnson F, Venugopal K. Congenital complete heart block and maternal connective tissue disease. Int J Cardiol. 2006;112:153-8.
• Buyon JP, Hiebert R, Copel J, Craft J, Friedman D, Katholi M et al. Autoimmune-associated congenital heart block: demographics, mortality, morbidity and recurrence rates obtained from a national neonatal lupus registry. J Am Coll Cardiol. 1998;31:1658–66
• Lee LA, Neonatal lupus erythromatosus. J Invest Dermatol. 1993;100: 9S-13S.
• Frankovich J, Sandborg C, Barnes P, Hintz S, Chakravarty E. Neonatal lupus and related autoimmune disorders of infants. Neo Rev. 2008;9:206-17.
• Blanford AT, Murphy BE. In vitro metabolism of prednisolone, dexamethasone, betamethasone, and cortisol by the human placenta. Am J Obstet Gynecol. 1977;127:264–7.
• Friedman DM. Fetal echocardiography in the assessment of lupus pregnancies. Am J Reprod Immunol. 1992;28:164-7.