2021 recommendations of the Brazilian Society of Rheumatology for the gynecological and obstetric care of patients with Sjogren’s syndrome

Abstract

Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs. Women with SS often experience gynecological symptoms due to the disease and need extra care regarding their sexual activity, reproductive health and during pregnancy, conditions that are not properly conducted in the clinical practice. To cover this gap, a panel of experts from the Brazilian Society of Rheumatology conducted a systematic review and meta-analysis on the identification of symptoms, diagnosis, monitoring, prognosis, and treatment of these manifestations. A Focus Group meeting was held and included experts in the field and methodologists, based on a previously developed script, with themes related to the objective of the study. The most important topics were summarized and 11 recommendations were provided.

Obstetric management of the most common autoimmune diseases: A narrative review

“…These antibodies can be present in different AD such as SLE (30%–50%), Sjögren syndrome (60%–90%), Rheumatoid arthritis (11%), etc ( 52 ). The presence of anti-Ro/SSA and/or anti-LA/SSB associates a high risk of several fetal and infant manifestations.…”

“…But it is controversial if this should be done in those women with no previous pregnancy affected, as the global risk is low. Nevertheless, as intensive surveillance is well accepted and carries no risk, it is also recommended for these women ( 3 , 9 , 52 , 54 ).…”

“…Recent data suggest the potential benefit of hydroxychloroquine in preventing the recurrence of complete fetal heart if there was a previous infant affected ( 62 , 63 ) or if anti-Ro/SSA and/or anti-LA/SSB antibodies were present ( 62 , 64 ). That is why most guidelines recommend using hydroxychloroquine in all pregnancies with anti-Ro/SSA and/or anti-La/SSB ( 3 , 9 , 52 ).…”

“…There has not been a proven beneficial effect in preventing fetal heart block ( 65 ). In some studies, it appears that dexamethasone can reverse carditis and incomplete fetal heart blocks ( 55 , 57 , 66 – 68 ), which is why some guidelines recommend the use of dexamethasone in first and second-degree fetal heart block after assessing the risk and benefits ( 3 , 52 ). Nevertheless, a systematic review in 2019 did not find any improvement in the use of antenatal corticosteroids alone or in combination in fetal or neonatal morbidity or mortality if fetal second-degree heart block had occurred ( 69 ).…”

The Future of Targeted Treatment of Primary Sjögren's Syndrome: A Focus on Extra-Glandular Pathology

“…Recent studies on novel drugs have shown that promising results on the improvement of disease prognosis have been met with unintended effects on the immune system, a variety of complications and rising costs for patients [4]. In addition, ESSDAI (EULAR Sjögren's syndrome disease activity index), which is a a primary outcome measure in most trials, does not include certain common symptoms such as vaginal dryness (occurring in up to 64% of female patients) [5,6], fatigue (70% of patients) [7] and mental effects (over 30% of patients) [8].…”