Akalyaa K*, Sreelatha S**, Kusuma M V*** ,Yashaswini G *, Ramaiah R**.
(* Junior Resident, ** Professor, *** Assistant Professor. Department of Obstetrics and Gynecology, ESIMC PGIMSR, Bangalore, India.)
Sjogren's syndrome is an autoimmune disease with a high prevalence of anti SSA (anti Ro) and anti SSB (anti la) antibodies. Pregnancy associated with such disorders have a very high clinical impact on both the mother as well as the fetus. Sjogren's syndrome is one of the most common autoimmune disease with a prevalence between 0.1 and 4.8 % affecting women in the fourth decade of life.
Sjogren’s syndrome (SS) is an autoimmune disease and is characterized by lymphocytic infiltration of lacrimal and salivary glands resulting in sicca symptoms. It can present either alone, that is primary Sjogren's syndrome (pSS), or can be associated with an underlying connective tissue disease, most commonly rheumatoid arthritis or systemic lupus erythematosus which is called as secondary Sjogren's syndrome. Sjogren's syndrome manifests from mild glandular manifestations to severe extra glandular disease like vasculitis, neuropathy and glomerulonephritis. The effect of autoimmune disease on pregnancy differs according to the maternal disease activity, severity of organ damage, antibody profile, and drug treatment. Pregnancy complications due to the autoantibodies like anti-Ro/SSA and anti-La/SSB in the maternal serum causes neonatal lupus and congenital heart block (CHB), intrauterine growth restriction and low birth weight babies. The incidence of neonatal lupus in an offspring born to a mother with anti-Ro/SSA antibodies is 12 % , but it can be as high as more than 20 % if the mother has given birth to a child with neonatal lupus or CHB before. The diagnosis of Sjogren's syndrome is challenging due to varied presentation of symptoms and thus large number of patients remain undiagnosed. Therefore preconceptional counseling is very important in such patients.
An unbooked primigravida with term gestation and with IUGR was admitted to our hospital with labor pains. On examination she was normotensive, pulse rate was normal, with no pallor or pedal edema. Uterus was 32 weeks in size with IUGR. Uterus was contracting with frequency of 1 to 2 contractions in 10 minutes, each lasting for 20 seconds. Presentation was cephalic and fetal heart rate was 140 beats per minute. On per vaginal exam, cervix was soft, 1 cm dilated, bag of membranes were present, and vertex was at -3 station. She underwent an emergency cesarean section in view of non reassuring NST. She delivered a single, live, male baby with birth weight of 1.8 kg. On postoperative day 2, she developed purpuric rashes over the lower limbs and deviation of angle of mouth to right side with left sided facial palsy for which she was evaluated and was found to have Primary Sjogren's syndrome as ANA profile with anti Ro and anti la antibodies were positive. Her ECG and 2D Echo were normal. Her rashes subsided by post operative day 7 and the facial palsy improved with physiotherapy. She was stable and was discharged on day 10.
Sjogren's syndrome is the second most common autoimmune disease after rheumatoid arthritis, with the prevalence between 0.1 % and 4.8 % in various group of populations. It occurs at any age but mainly affects women in the fourth decade of life. The prevailing recent social trend toward late marriages and advanced maternal age increases the disease impact on pregnancy. Patient may present with generalized symptoms such as fatigue, dry mouth and eyes. Sjogren's syndrome may present with extra glandular features such as isolated leucopenia, urticaria, irritable bowel syndrome, vasculitis, arthralgia, chronic non-deforming non-erosive polyarthritis, psychiatric symptoms, bladder symptoms, auditory, vestibular and dental problems. Vascular manifestations are palpable purpura, erythematous rash, urticaria, photosensitivity, erythema nodosum, Raynaud’s phenomenon and gangrene. Neurological manifestations are mononeuritis multiplex, symmetrical polyneuropathy, carpal tunnel syndrome, trigeminal neuropathy, recurrent seizures, aseptic meningitis, and chronic immune demyelinating polyneuropathy. Marker’s used to diagnose Sjogren's syndrome are antinuclear antibodies (most frequent), anti SSA (anti-Ro; most specific), anti SSB (anti-La), and cryoglobulins and hypocomplementemia (prognostic marker). These antibodies cross the placenta by the beginning of 12 weeks of gestation and cause fetal cellular damage by macrophage activity, myocarditis and arrhythmia. Fetal surveillance is done by serial echocardiograms and obstetric sonograms between 16 to 20 weeks of gestation. The goals of management includes early diagnosis and treatment of congenital heart block to improve the fetal outcome. The pregnancy outcome is excellent with the use of antenatal corticosteriods such as dexamethasone or betamethasone, which can reduce the antibody-mediated inflammatory damage of nodal tissue. Dexamethasone reverses carditis and incomplete heart block and also improves fetal hemodynamics and hence it is recommended in recent or incomplete heart block, or in cardiac failure.
Women with Sjogren's syndrome must undergo prenatal counseling explaining all the risks and complications, medications that are contraindicated in pregnancy, whether the patient is in the best condition to get pregnant according to underlying disease activity and the need to control the disease well before conception. These high-risk pregnancies can be optimally managed by a multidisciplinary approach involving a high-risk obstetrician, a rheumatologist, and a pediatrician. High degree of suspicion and proactive approach can pick up the disease at the earliest.
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Akalyaa K, Sreelatha S, Kusuma M V, Yashaswini G, Ramaiah R. A Rare Case Of Sjogren's Syndrome In Pregnancy And Perinatal Outcome. JPGO 2019. Vol 6 No. 5. Available from: https://www.jpgo.org/2019/05/a-rare-case-of-sjogrens-syndrome-in.html