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 Table of Contents  
Year : 2023  |  Volume : 13  |  Issue : 1  |  Page : 99-102

Anaesthesia for caesarean section in a parturient with sjogren syndrome and scoping review

1 Department of Anaesthesia and Intensive Care, PGIMER, Chandigarh, India
2 Department of Anaesthesia, Sawai Mann Singh Medical College, Jaipur, Rajasthan, India

Date of Submission29-Dec-2021
Date of Acceptance09-Apr-2022
Date of Web Publication09-Mar-2023

Correspondence Address:
Dr. Shalvi Mahajan
Department of Anaesthesia and Intensive Care, PGIMER, Chandigarh - 160 012
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JOACC.JOACC_115_21

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Sjogren syndrome is a slowly progressive autoimmune disease with lymphocytic infiltration of exocrine glands, peri-epithelial and extra-epithelial tissues. In light of better diagnostic modalities and improved treatment options, patients with Sjogren syndrome are now commonly encountered by anaesthetist for various surgeries. Here, we would like to describe an anaesthetic management of a parturient with bad obstetric history who was diagnosed with Sjogren syndrome and was planned for caesarean delivery. This article also reviewed anaesthetic problems faced due to inherent disease pathology and concurrent alterations in treatment modalities.

Keywords: American Standard of Anaesthesiology, caesarean delivery, Modified Mallampati Grade, Sjogren syndrome, subarachnoid block

How to cite this article:
Mahajan S, Sharma A, Kumar S, Gandhi K. Anaesthesia for caesarean section in a parturient with sjogren syndrome and scoping review. J Obstet Anaesth Crit Care 2023;13:99-102

How to cite this URL:
Mahajan S, Sharma A, Kumar S, Gandhi K. Anaesthesia for caesarean section in a parturient with sjogren syndrome and scoping review. J Obstet Anaesth Crit Care [serial online] 2023 [cited 2023 Apr 1];13:99-102. Available from: https://www.joacc.com/text.asp?2023/13/1/99/371301

  Introduction Top

Sjogren syndrome is an autosomal recessive chronic, autoimmune disorder with a reported prevalence of 0.1–4.8% in the general population.[1] It can present either alone (primary disease) or in combination with other autoimmune diseases such as rheumatoid arthritis and systemic lupus (secondary). During pregnancy, it predominantly affects women in the fifth to sixth decades but can manifest in other age groups as well.[1] It can lead to adverse foetal outcomes such as congenital heart blocks; henceforth, its timely detection and prophylaxis during pregnancy are of supreme importance.[2]

  Case Description Top

A 31-year-old booked parturient (sixth gravida, four previous missed abortions and one previous live birth) was brought for caesarean delivery at 36 + 5 weeks of gestation. Due to her history of recurrent abortions, an autoimmune disease was suspected, and various laboratory tests including autoantibody workup were conducted in her current gestation. The anti–Sjögren's-syndrome-related antigen A autoantibodies (anti-Ro/SSA) (>240 Miu/ml) and anti-nuclear antibodies (ANAs). were reported positive along with the persistent non-specific features of arthralgia and dry mouth. The patient was, hence, diagnosed with Sjogren syndrome. Fundus examination and salivary gland scintigraphy were found to be normal. Because of the risk of congenital heart block due to Sjogren disease, foetal echocardiogram as well as foetal heart rate (FHR) were evaluated routinely from 16 weeks of gestation. To prevent any foetal cardiac complications, the patient was started on a prophylaxis of tab ecospirin 75 mg HS and tab hydroxychloroquine (HCQ) 300 mg once a day (OD).

The parturient was planned for caesarean delivery at 36 + 5 weeks of gestation. Preoperative anaesthesia examination revealed Modified Mallampati Grade-3 with normal temporomandibular joint movement. Grossly spine appeared to be normal and no signs and symptoms of neuropathy were present. Preoperative laboratory tests revealed Hemoglobin (Hb) – 10.4, Total Leukocyte Count (TLC) – 15,500/mm3 and a platelet count – 1.89 lakh/mm3. The coagulogram, serum electrolytes and renal function tests were normal. Liver enzymes, that is Aspartate aminotransferase (AST) and Alanine transaminase (ALT) levels, were slightly raised – 110.4 and 89.1, respectively. The preoperative electrocardiogram (ECG) revealed normal sinus rhythm.

Anti-aspiration prophylaxis of intravenous ranitidine 50 mg and metoclopramide 10 mg were given prior to shifting of patient to operation theatre. Standard American Society of Anesthesiologists (ASA) monitoring, which includes ECG, oxygen saturation (SPO2) and Non-invasive Blood Pressure (NIBP), were initiated after arrival in the operating room. Two 18-G iv cannulas were secured and a 500-ml ringer lactate infusion was initiated. Her opening vitals were heart rate – 80 beats/min, non-invasive blood pressure – 128/78 mmHg, respiratory rate – 18 breaths/min and room air oxygen saturation – 99%. Oxygen at 4 l/min was supplied via a venturi facemask.

Regional anaesthesia with subarachnoid block (SAB) was planned after obtaining proper consent and explaining risks/benefits. First, the patient was positioned in the lateral decubitus position; subsequently, under aseptic precautions, SAB was administered at the L3–4 intervertebral space using a 26-G quinke's needle. Once free flow of cerebrospinal fluid was confirmed, 1.5 ml of 0.5% hyperbaric bupivacaine with 25 μg of fentanyl was administered. Then, she was positioned supine with a left lateral tilt of 15 deg, and maximum sensory level of T4 was achieved within 4 min. Eight minutes following the skin incision, a healthy male neonate (weight, 2.8 kg) was delivered. Intravenous infusion of 20 international units (IU) of oxytocin mixed with 200 ml normal saline was started once placenta was removed. The patient's haemodynamics was stable throughout, and the estimated blood loss were around 600 ml. In neonatal intensive care unit, neonate's ECG displayed a heart rate of 150–170 beats/min with normal sinus rhythm. Later, mother and baby were successfully discharged from hospital.

  Discussion Top

Sjogren syndrome belongs to organ-specific auto-immune disorder of pregnancy and same auto-antibody can affect multiple organs. It is commonly seen in females due to oestrogen which might affect immune system.[3] It is characterized by lymphocytic infiltration and destruction of exocrine glands (salivary and lacrimal) leading to decreased glandular secretions and consequently dry mouth and dry eyes [Dry syndrome]. Extra glandular involvement seen in 71% of patients further adds to concern during anaesthesia exposure in pregnancy.[4]

This disease can be seen in any sex with high female propensity, especially in the fourth or fifth decade of life. Young onset disease (less than 35 years of age) has high association with severe clinical manifestation. There are limited studies on the effect of Sjogren's syndrome in pregnancy. Data collected from these studies indicate worsening of symptoms during pregnancy. Parturient with autoimmune diseases has higher rates of preeclampsia, premature delivery, increased rate of spontaneous abortions and foetal losses, and caesarean sections.[5],[6] Both young age and bad obstetric history were evident in our patient.

Congenital heart blocks in the neonate requiring to subsequent pacemaker implantation is a known complication. The positive antibodies such as antinuclear antibodies (most frequently detected), anti–Sjögren's-syndrome-related antigen A antibodies (anti-SS-A) (also called anti-Ro; most specific) and Anti-Sjögren's syndrome type B antibodies (anti-SS-B) (anti-La) cross the placenta after 12 weeks of gestation and bind with apoptotic cells leading to myocarditis and arrhythmias.[7] Therefore, periodic monitoring of heart rate by foetal echocardiogram and foetal growth by serial maternal ultrasound (starting from 16 to 20 weeks of gestation) is a must in improving foetal outcomes, as was done in our patient also.

Maternal use of corticosteroids and HCQ has been considered in such patients. The maternal use of fluorinated steroids has been stated to reverse incomplete, recent-onset heart block and cardiac failure by dropping immune response secondary to maternal antibody.[8] While the number of studies with HCQ is limited, however systematic meta-analysis demonstrated its relative safety and advocated its use in expectant mothers with lupus.[9] HCQ has been shown to have decreased risk of foetal myocarditis in anti-SS-A-positive mothers by inhibiting Toll-like receptor signalling.

Owing to the increased risk of foeto-maternal complications, delivery by caesarean section is preferred. For deciding the mode of anaesthesia, various factors such as an informed consent, obstetric emergency and anaesthesia concerns related to Sjogren syndrome should be considered. Regional anaesthesia is usually preferred unless there are emergency conditions like foetal distress or high-grade obstetric haemorrhage or immune thrombocytopenia (5–15% cases).[10] The Society for Obstetric Anesthesia and Perinatology states that it is reasonable to proceed with neuraxial procedure with platelet count of ≥70k 106/l; competing risk/benefits if platelet count is between 50k and 70k 106/l may justify proceeding with neuraxial procedure and avoid neuraxial procedure if platelet count <50k 106/l.[11] Howsoever, various authors have successfully administered general Anaesthesia (GA) in non-obstetric surgeries with Sjogren syndrome. A review of successful anaesthesia administration in patients suffering from Sjogren syndrome planned for various surgeries has been depicted in [Table 1].
Table 1: Review of literature

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In our case, we planned for regional anaesthesia. Preloading with a 500-ml crystalloid solution and using fentanyl as a regional adjuvant helped us to preserve haemodynamics intra-operatively. We hereby provide summary of perioperative anaesthesia concerns due to glandular and extraglandular manifestations of disease in these patients [Table 2].
Table 2: Summary of anaesthetic concerns in Sjogren syndrome

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In conclusion, meticulous preoperative evaluation, prophylaxis and foetal surveillance as well as individualized intra-operative management can improve foetal outcomes in autoimmune disorders.

Contribution of authors

Dr. Shalvi Mahajan – Initial observation, literature search and manuscript preparation.

Dr. Aakriti Sharma – Initial observation, acquisition and interpretation of data.

Dr. Sanjay Kumar – Manuscript editing.

Dr. Komal Gandhi – Manuscript editing and proof reading.


Taken from patient.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, et al. European study group on classification criteria for Sjögren's syndrome. Ann Rheum Dis 2002;61:554-8.  Back to cited text no. 1
Miranda-Carús ME, Askanase AD, Clancy RM, Di Donato F, Chou TM, Libra MR, et al. Anti-SSA/Ro and anti-SSB/La autoantibodies bind the surface of apoptotic fetal cardiocytes and promote secretion of TNF-alpha by macrophages. J Immunol 2000;165:5345-51.  Back to cited text no. 2
Voulgarelis M, Tzioufas AG. Pathogenetic mechanisms in the initiation and perpetuation of Sjögren's syndrome. Nat Rev Rheumatol 2010;6:529-37.  Back to cited text no. 3
Ramos-Casals M, Solans R, Rosas J, Camps MT, Gil A, Del Pino-Montes J, et al. Primary Sjogren syndrome in Spain: Clinical and immunologic expression in 1010 patients. Medicine 2008;87:210-9.  Back to cited text no. 4
De Carolis S, Salvi S, Botta A, Garofalo S, Garufi C, Ferrazzani S, et al. The impact of primary Sjogren's syndrome on pregnancy outcome: Our series and review of the literature. Autoimmun Rev 2014;13:103-7.  Back to cited text no. 5
Skomsvoll JF, Ostensen M, Irgens LM, Baste V. Obstetrical and neonatal outcome in pregnant patients with rheumatic disease. Scand J Rheumatol 1998;107:109-12.  Back to cited text no. 6
Brucato A, Cimaz R, Caporali R, Ramoni V, Buyon J. Pregnancy outcomes in patients with autoimmune diseases and anti-Ro/SSA antibodies. Clin Rev Allergy Immunol 2011;40:27-41.  Back to cited text no. 7
Costedoat-Chalmeau N, Amoura Z, Le Thi Hong D, Wechsler B, Vauthier D, Ghillani P, et al. Questions about dexamethasone use for the prevention of anti-SSA related congenital heart block. Ann Rheum Dis 2003; 62:1010-2.  Back to cited text no. 8
Sperber K, Hom C, Chao CP, Shapiro D, Ash J. Systematic review of hydroxychloroquine use in pregnant patients with autoimmune diseases. Pediatric Rheumatol 2009;7:1-9.  Back to cited text no. 9
Dai F, Yang G, Rao P, Wu P, Chen R, Sun Y, et al. Clinical characteristics of secondary immune thrombocytopenia associated with primary Sjögren's syndrome. Front Med (Lausanne) 2020;7:138.  Back to cited text no. 10
Bauer ME, Arendt K, Beilin Y, Gernsheimer T, Perez Botero J, James AH, et al. The Society for obstetric anesthesia and perinatology interdisciplinary consensus statement on neuraxial procedures in obstetric patients with thrombocytopenia. Anesth Analg 2021;132:1531-44.  Back to cited text no. 11
Kim NE, Lee JH, Chung IS, Lee JY. Anesthetic management of patient with Sjogren's syndrome who underwent cesarean section-a case report. Korean J Anesthesiol 2016;69:283-6.  Back to cited text no. 12
Hong JM, Kim E, Kim HK, Lee DW, Baik JS, Lee JY. Combined spinal–epidural anaesthesia for radical hysterectomy in a patient with Sȍgren syndrome with progressive interstitial lung disease. Springerplus 2016;5:1737.  Back to cited text no. 13
Demirel I, Ozer AB, Bayar MK, Erhan OL. Anaesthesia management for acute appendicitis in cases with Sjögren's syndrome accompanying autoimmune hepatitis. BMJ Case Rep 2013;1-4.  Back to cited text no. 14
Sen I, Gombar S, Deva C. Anaesthetic management of a pregnant patient with Sjogren syndrome-A case report. Indian J Anaesth 2002;46:210-11.  Back to cited text no. 15
  [Full text]  
Takahashi S, Ogasawara H, Tsubo T, Ishihara H, Matsuki A. Anesthetic management of a patient with Sjögren's syndrome and pulmonary fibrosis. Masui 1990;39:1393-6.  Back to cited text no. 16


  [Table 1], [Table 2]


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