Yadav K*, Samant PY**, Thakur HS***
(* Junior Resident, ** Additional Professor, *** Assistant Professor, Department of Gynecology and Obstetrics, Seth G S Medical College and K E M Hospital, Mumbai, India.)
We present a case of a 30 year old woman in her second pregnancy with secondary Sjogren’s syndrome associated with systemic lupus erythematosus (SLE), rheumatoid arthritis and right internal jugular vein thrombosis. This overlap is a rare phenomenon seen in pregnancy as the syndrome is mostly seen in the late reproductive age.
Sjogren’s syndrome is a chronic inflammatory disorder. It is characterised by lymphocytic infiltration of exocrine glands. Patients with the syndrome present with xerostomia, xerophthalmia, parotid gland enlargement affecting quality of life. Prevalence rate of the disease is between 0.1 % to 4.8 %. Primary Sjogren’s syndrome occurs in absence of another underlying rheumatic disorder. Secondary sjogren syndrome is associated with underlying rheumatic disorder such as SLE, rheumatoid arthritis or scleroderma. Sjogren’s syndrome predominantly occurs in women.
Sjogren’s syndrome may have associated antiphospholipid syndrome (APS) which is a hypercoagulable state that may cause arteriovenous thrombosis and/ or thrombocytopenia resulting in a series of thrombotic clinical symptoms. In pregnancy APS can cause miscarriage, premature birth, and still birth. A multidisciplinary approach can achieve successful maternal and fetal outcome.
A 30 year old second gravida, was transferred to our institute at 22 weeks of gestation with severe multiple small joint pains and swelling of right lower limb for 2 months. She was diagnosed with rheumatoid arthritis two years prior. Around one year prior to presentation, she had right internal jugular vein thrombosis extending to superior vena cava and was on treatment for same with warfarin, prednisolone and immunosuppressive agents; hydroxy chloroquine and azathioprine. In the first pregnancy, she had a termination of pregnancy at 16 weeks for single umbilical artery in the fetus and a positive screen for aneuploidy.
In a private hospital, during the first trimester of present pregnancy, warfarin was changed to low molecular weight heparin 0.4 mg subcutaneously. At her presentation here, she was admitted by rheumatologists. Investigations revealed positive anticardiolipin antibody immunoglobulin G (aCLA Ig G), anti- Sjogren’s syndrome-related antigen A (SSA) positivity. Diagnosis of secondary Sjogren’s syndrome was made according to American College of Rheumatology criteria as she was SSA positive and ANA positive with a titre of 1:320. There was no xerostomia, xerophthalmia or parotid swelling. Prednisolone was stopped and tab dexamethasone 4 mg was started to prevent fetal adrenal insufficiency and 75 mg aspirin. LMWH was continued. Right lower limb venous Doppler was indicative of varicose veins and conservative treatment with elastic stockings and limb elevation were advised. Her obstetric evaluation was normal. Obstetric sonography was normal and corresponded to 23 weeks. Fetal echocardiogram was normal and congenital heart block which can occur due to positive SSA antibody. After the pain and swelling subsided, she was discharged and advised antenatal check-up.
She was monitored for preeclampsia, anemia or antepartum haemorrhage and intrauterine growth restriction (IUGR). She was admitted at 36 weeks gestation for fetoplacental insufficiency and asymmetrical IUGR. Non-stress test (NST) was reactive. Her haemoglobin was 12.9 gm %, WBC-16,900/ cu mm, platelet count 2.2 lakh/ cu mm, INR-1.02, aPTT-27.3 (control 29) , fasting blood sugar 101mg/ dl, postprandial blood sugar 114 mg/ dl and TSH was 3.93 IU/ ml. She went into spontaneous labor and heparin was stopped. She delivered uneventfully vaginally with baby weight of 2.2 kg with Apgar score of 9/ 10. Tablet warfarin was restarted postpartum and she was discharged on day 9 once INR reached desired level. Contraception counselling was done and she chose barrier method of contraception. She was asked to follow up in cardiology and rheumatology clinics.
Sjogren’s syndrome overlapping SLE and rheumatoid arthritis in pregnancy is associated with maternal and neonatal complications. There is high incidence of poor fetal outcome hence prenatal counselling and antenatal care play a major role. Such pregnancies require multidisciplinary approach with rheumatologist, obstetrician and paediatrician.
Several studies have demonstrated increased rate of spontaneous abortion, intrauterine growth restriction, preterm labour and fetal loss. Most dreaded complication of pregnancy in patient with Sjogren’s syndrome is increased risk of delivering baby with complete heart block (CHB) especially if she is anti SSA positive. Hence frequent surveillance by serial echocardiograms has been advised. In our case, fetal 2D-Echo at 24 weeks was normal. The reported prevalence of CHB with anti SSA positive woman is 1-2 % with recurrence rate approximately 10 times higher.
Maternal treatment with dexamethasone or betamethasone reduces the antibody mediated inflammatory damage hence improves fetal outcomes. But this treatment may increase the risk of infection, osteoporosis and glucose intolerance while in fetus it may cause IUGR, oligohydramnios and possibly adrenal suppression. Alternative therapies include plasmapheresis, intravenous immunoglobulins and beta sympathomimetics. Our patient was given dexamethasone from 22 weeks of gestation and delivered at near term with IUGR.
Young women with Sjogren’s syndrome often have systemic and severe disease compared with patient with primary Sjogren’s syndrome which occur in later stage of life. Our patient was 30 years old with Sjogren’s syndrome with SLE and rheumatoid arthritis. Relative risk of fetal loss is 2.7 in primary Sjogren’s syndrome and 2.2 in SLE which may be attributed to secondary Sjogren’s syndrome. Brucato et al found that anti-Ro positive SLE women had higher rate of spontaneous abortion (ie) 18% as compared to 5.6% with anti Ro negative SLE women. There is significantly higher rate of recurrent pregnancy loss in patients with anti-Ro non SLE positive women which was 23.7%. Long acting progesterone intrauterine device (IUD) or subdermal implants are preferred and depot medroxyprogesterone acetate (DMPA) should be avoided in patient on steroids or patients at risk of osteoporosis.
Various studies have proven that general outcome of the pregnancies in these pregnant women with autoimmune disorders can be excellent with multidisciplinary team approach is adopted.
Women with Sjogren’s syndrome are likely to have more complications during pregnancy with increased risk of poor fetal outcome. Women with these disorders should undergo preconceptional counselling and these high risk pregnancies should be optimally managed by multidisciplinary approach involving obstetrician, rheumatologist and neonatologist.
- Gupta S, Gupta N. Sjogren syndrome and pregnancy. A literature review. Perm J 2017; 21: 16047. doi: 10.7812/TPP/16-047.
- Bowman SJ, Fox RI. Classification criteria for Sjogren's syndrome: nothing ever stands still! Ann Rheum Dis. 2014; 73(1): 1–2.
- Tegethoff M, Pryce C, Meinlschmidt G. Effects of intrauterine exposure to synthetic glucocorticoids on fetal,newborn, and infant hypothalamic-pituitary- adrenal axis function in humans: A systematic review. Endocr Rev.2009; 30:753-89.
- Hussein SZ, Jacobsson LHT, Lindquist PG, Theander E. Pregnancy and fetal outcome in women with primary Sjogren’s syndrome compared with women in the general population: a nested case control study. Rheumatology 2011; 50:1612-7
- Brucato A, Cimaz R, Caproli R, Ramoni V, Buyon J. Pregnancy outcomes in patients with autoimmune diseases and anti RO/SSA antibodies. Clin Rev Allergy Immunol. 2011; 40(1):27-41.
- Sammaritano LR. Contraception in patients with systemic lupus erythematosus and antiphospholipid syndrome. Lupus. 2014; 23(12):1242-5.
Yadav K, Samant PY, Thakur HS. Secondary Sjogren’s Syndrome in Pregnancy. JPGO 2018. Volume 5 No.4. Available from: http://www.jpgo.org/2018/04/secondary-sjogrens-syndrome-in-pregnancy.html