Systemic Lupus Erythematosus: Perinatal and Neonatal Implications

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder that can affect almost all organ systems in the body. It is most common in women of childbearing age and may cause multiple peripartum complications. This article reviews the pathophysiology of SLE and the effects of SLE on fertility and pregnancy. The complexities of managing a pregnant patient with SLE are reviewed, and the importance of interdisciplinary collaboration discussed, as well as the effects of SLE on the fetus and a review of neonatal lupus erythematosus. Finally, a case report of a pregnant patient with SLE with challenging clinical management issues is presented.     

Myasthenia gravis and pregnancy.

Myasthenia gravis (MG) is a chronic autoimmune disorder of neuromuscular transmission characterized by varying degrees of weakness and easy fatigability of the skeletal muscles. Precipitants of myasthenic symptoms or crises include physical and emotional stress, systemic illness, infections, hypo or hyperthyroidism, pregnancy, any type of surgery with general anaesthesia as well as corticosteroids. The authors report two cases of MG in pregnancy and discuss briefly the various aspects of the disease course and management in pregnancy. As MG occurs predominantly in women of reproductive years, it is important that obstetricians are aware of this condition and its management in pregnancy.     

Autoimmune thrombocytopenic purpura: current concepts and recommended practices

Autoimmune thrombocytopenic purpura is the most common autoimmune disorder encountered in the pregnant patient. It is potentially fatal for the mother and fetus yet treatable and potentially curable. Analysis of current perinatal literature reveals not only a great deal of interest and activity in the study of this syndrome and its special problems during pregnancy but also significant controversy. The disease can be acute or chronic and vary in time of onset and severity of manifestations. If not forewarned with an awareness of this disorder's pathogenesis and potential fetal effects particularly in the pregnant woman who has undergone splenectomy, the obstetrician cannot respond appropriately. The usefulness of platelet antibody determinations to facilitate obstetric management decisions is discussed. The importance of cooperative care among the obstetrician, hematologist, and neonatologist is emphasized. Recommendations for management of autoimmune thrombocytopenic purpura in pregnancy are derived from a review of current concepts of the disorder's pathogenesis, pathophysiology, criteria for diagnosis, and modes of therapy as well as special maternal/fetal considerations of antepartum, intrapartum, and postpartum care.     

Connective tissue and dermatological conditions in pregnancy

Connective tissue disorders are common in women of reproductive age, and hence are seen frequently in maternal medicine clinics. The disorders, and their treatments, may have significant adverse effects on fertility, the developing fetus, and on pregnancy outcomes. In turn pregnancy may affect the natural course of the illness. Pre-pregnancy planning and multidisciplinary management are vital to optimise maternal and fetal outcomes. This review will cover a general approach and specific management points of common autoimmune and genetic conditions.Alterations in the maternal immune system affect the disease course of pre-existing skin conditions during pregnancy, and pharmacotherapy may be limited due to effects on the fetus. Some dermatological conditions arise de novo during pregnancy. Women may present to maternity services directly; therefore obstetricians must be able to diagnose, investigate and initiate management of pregnancy dermatoses, with input from dermatologists where available.     

Autoimmune hemolytic anemia of the cold antibody type associated with pregnancy

A case is reported of autoimmune hemolytic anemia of the cold antibody type occurring during pregnancy in a 28-year-old Negro patient. The disorder appears to have been stimulated by a septic abortion which preceded the onset of symptoms. The disease did not appear to have any adverse effects on the pregnancy. The pregnancy affected the disease by increasing the rate of hemolysis and necessitating higher doses of prednisone than had been used previously.     

Myasthenia gravis and pregnancy

Myasthenia gravis (MG) is an autoimmune disorder of neuromuscular junction that has higher incidence in younger women than men, which could be related to differences in sex hormones physiology and immune system functioning between males and females. MG can first present during pregnancy and variably affect pregnancy, labor, and postpartum period. In this paper, we had an updated overview on our understanding about MG presentation and its effect on pregnancy and vice versa, therapeutic options for MG pregnant women, management of pregnancy or labor complications in MG patients, and finally fetal and neonatal considerations in MG pregnant women. A multidisciplinary approach, involving obstetricians/gynecologists, neurologists, and anes-thesiologists, plays a pivotal role in improving the clinical outcomes in both MG mothers and their infants during pregnancy, delivery and postpartum.     

Pregnancy in myositis and scleroderma

Myositis and scleroderma are both rare autoimmune diseases with female predominance and often occur before and during reproductive years. The rarity of diseases explains the low frequency of concurrent disease and pregnancy. Like other autoimmune diseases, myositis and scleroderma may be more active during pregnancy as well. To date, many patients with myositis and scleroderma can have favorable pregnancy outcomes with careful management. This chapter provides a current overview of pregnancy outcomes in myositis and scleroderma. A major theme that appears to have emerged across these inflammatory diseases is that active maternal disease during pregnancy is associated with adverse pregnancy outcomes, and thus, personalized management is necessary depending on the disease state and comorbidities.     

Management and monitoring of severe preeclampsia

Preeclampsia is associated with increased maternal and perinatal morbidity and mortality. Preeclampsia is more than pregnancy-induced hypertension. The hypertension is only one manifestation of an underlying multifactorial, multisystem disorder, initiated early in pregnancy. In established severe disease there is volume contraction, reduced cardiac output, enhanced vascular reactivity, increased vascular permeability and platelet consumption. Medical treatment of severe hypertension in pregnancy is required. The more controversial issues are the role of pharmacological treatment in conservative management of severe preeclampsia aiming at prolongation of pregnancy, the ability of such therapy to modify the course of the underlying systemic disorder and the effects on fetal and maternal outcome. This paper presents an overview concerning the current developments in management and monitoring of severe preeclampsia. Controversial topics such as the role of plasma volume expansion in preeclampsia, expectant versus aggressive management of severe preeclampsia remote from term, and pharmacological interventions in the management of eclampsia and the HELLP syndrome are addressed.     

Herpes gestationis. Apropos of 2 cases

Herpes gestationis is an autoimmune disorder specific of pregnancy. The authors report two cases of this condition with distinctive clinical and immunological features. Both the pathogenesis and the nosologic connections between Herpes gestationis and Bullous pemphigoid are reviewed and discussed.     

Abrupt reversal of gestational autoimmune thrombocytopenia after delivery. A case report

Autoimmune thrombocytopenia purpura frequently is first noted during pregnancy. The severity of the disorder varies, but when the patient presents with active bleeding from any site, therapy is mandatory. Treatment generally consists of corticosteroids and/or splenectomy. A woman developed severe autoimmune thrombocytopenia purpura during pregnancy that proved refractory to corticosteroids and splenectomy. Six days after delivery her platelet count returned to normal. The etiology of autoimmune thrombocytopenia purpura remains unknown, but this experience suggests that in this patient the thrombocytopenia was related to her pregnancy.     

Pregnancy complicated with autoimmune diseases

Autoimmune disorders such as SLE and ITP occur more commonly in young women and are the most common complications in pregnancy. There is considerable controversy concerning the risk to the mother and fetus, and the optimal prepartum management for minimizing that risk. 1. SLE is an autoimmune disorder in which IgG antibodies such as anti dsDNA-IgG, anticardiolipin IgG, and anti SS-A/Ro IgG are produced. Lupus nephropathy accompanied by diminished serum complement (CH50) and a rise in antibodies against dsDNA is a frequent clinical problem during pregnancy, which represents the adverse effect of hypertension or superimposed toxemia and causes fetal death or intrauterine fetal growth retardation. Habitual abortion or fetal death is common in a case with high anticardiolipin IgG titre. Anti SS-A antibodies are often found in the infants of antibody-positive mothers, and the deposition of antibodies in the perinodal region cause congenital heart block. IgG or immune complexes crossing the placenta directly injures the cardiac conduction system. In these cases which have high titre crossing the placenta directly injuries the cardiac conduction system. In these cases which have high titre of autoimmune antibodies, corticosteroid therapy should be started. 2. Management of ITP in pregnancy involves the consideration of three issues: 1) treatment of maternal thrombocytopenia, 2) prediction of fetal thrombocytopenia, 3) obstetrical management. ITP increases the risk for postpartum bleeding of sufficient severity to require blood transfusion. In most of these cases, maternal platelet counts are found to be less than 30,000/mm3. Women who have symptomatic severe steroid-unresponsive ITP may benefit from intravenous IgG(IvIgG) given as elective treatment.(ABSTRACT TRUNCATED AT 250 WORDS)     

Intrahepatic cholestasis of pregnancy

Intrahepatic cholestasis of pregnancy is the most common liver disorder unique to pregnancy in women without hypertension. The cause of intrahepatic cholestasis of pregnancy is still under discussion but genetic and hormonal factors are predominant. The main symptom is skin pruritus, associated with increase in serum transaminase activities and bile acid concentrations. Intrahepatic cholestasis of pregnancy carries a risk for the pregnancy because of preterm delivery and sudden intrauterine fetal death. Ursodeoxycholic acid (usually 1000mg per day or 15mg/kg per day) is currently the most effective pharmacologic treatment. Ursodeoxycholic acid reduces pruritus, transaminases and bile acid levels and probably prematurity without adverse effects. Obstetric management is still under debate. The majority of authors recommend active management with elective delivery usually before or at 38 weeks of gestation according the severity of cholestasis. Prospective controlled studies are required to confirm the benefit of ursodeoxycholic acid treatment on fetal outcome and to clarify the obstetrical management near term.     

自身免疫性甲状腺疾病对妊娠结局和女性生育功能的影响

甲状腺自身免疫性疾病是育龄妇女的常见病,包括妊娠期甲状腺功能亢进症、甲状腺功能减退症及产后甲状腺炎。抗甲状腺过氧化物酶抗体(TPOAb)、甲状腺球蛋白抗体(TGAb)及促甲状腺激素受体抗体(TRAb)是机体自身免疫功能紊乱的重要标志。甲状腺功能紊乱对妊娠妇女及胎儿可产生诸多不良影响。甲状腺功能正常但甲状腺自身抗体阳性的妊娠妇女在妊娠期发生甲状腺功能减退、分娩后发生产后甲状腺炎的危险性增高,应注意监测甲状腺功能。甲状腺自身免疫的存在可能与不孕不育以及自然流产等相关。对高危人群进行筛查、早期诊断并及时给予治疗,可有效降低妊娠不良结局的发生。     

Autoimmunity and pregnancy loss

Clinicians have recognized for several decades that certain autoimmune conditions, such as systemic lupus erythematosus (SLE), are associated with pregnancy loss. During the 1980s, investigations focused attention on fetal wastage in women with antiphospholipid antibodies and the antiphospholipid antibody syndrome (APS) was characterized. Its defining features include fetal wastage in the presence of significant levels of anticardiolipin antibodies. Since that time, interest in other autoimmune diatheses and various specific autoantibodies as possible causes of pregnancy loss has increased. Investigators have attempted to establish an association between recurrent pregnancy loss and the presence of a specific autoantibody or patterns of autoantibodies. Thus far, only modest evidence supports the concept that other autoantibodies are linked to, much less cause, pregnancy loss. In this review, we will define pregnancy loss in its various forms and discuss pregnancy loss in well-characterized autoimmune diseases such as SLE and APS. We will focus on the diagnosis and management of these conditions in women attempting to achieve successful pregnancies. Later we discuss the evidence concerning the less well defined association of antiphospholipid antibodies other than the lupus anticoagulant and anticardiolipin antibodies to recurrent pregnancy loss. We then outline the significance of antinuclear antibodies and antithyroid antibodies pertaining to adverse pregnancy outcome and conclude by summarizing and making some suggestions for further study.     

Thyroid disorders and other endocrinological disorders in pregnancy

Endocrine disorders are commonly encountered in pregnancy. To optimize pregnancy outcome, it is essential to understand the physiology underlying these conditions. Clinicians should be aware of which investigations are required for diagnosis and monitoring during pregnancy as well as current guidance on which treatments are safe to use. The most common endocrine disorder encountered in pregnancy is diabetes, followed by hypo and hyperthyroidism. Rarer endocrine disorders including pituitary dysfunction, adrenal and parathyroid disease are as important to be aware of due to the potential for significant maternal and fetal morbidity or mortality if not diagnosed or managed appropriately. Over recent years awareness of the potential adverse effects of vitamin D deficiency has driven the guidance for vitamin D supplementation for pregnant and lactating women. This review focuses on the physiology and current management of thyroid dysfunction and the rarer endocrine disorders in pregnancy, and includes current guidance on vitamin D supplementation.     

Manejo obstetrico y perinatal en miastenia gravis

Myasthenia gravis is an autoimmune disorder characterized by the presence of IgG antibodies against human acetylcholine receptors on striated muscle. Presentation during pregnancy is very rare. This disease is characterized clinically by fatigability of voluntary muscles, particularly those of the eyes, face, oropharynx and extremities.     

Myasthenia gravis in mothers and their newborns

Myasthenia gravis is a complex autoimmune disorder. Anti-AChR antibodies destroy elements of the postsynaptic membrane of the myoneural junction in affected muscle groups. This results in decreased nerve-impulse transmission. Myasthenic patients have diminished skeletal muscle strength and tire rapidly with exercise. Pregnancy threatens maternal myasthenic exacerbation and crisis, particularly early in the puerperium. The medication requires frequent adjustment during pregnancy due to changing requirements and physiologic changes in absorption and excretion. Myasthenia results in an increase in maternal mortality, morbidity, pregnancy wastage, and premature labor. Anticholinesterase medications and corticosteroids are the mainstays of medical therapy of maternal MG. Thymectomy, plasmapheresis, immunosuppressant drugs, gamma globulin, and ACTH are adjuvants of varying usefulness. Enforced rest periods, a tranquil environment, and prompt treatment of intercurrent infections are important for myasthenic mothers. The management of myasthenic crisis requires hospital intensive care with mechanical respiratory support and careful monitoring of blood gases. Plasmapheresis is an effective means of controlling crises. It is usually combined with intensive steroid and or immunosuppressant therapy. The myasthenic mother undertakes pregnancy with increased risk to herself and her infant. There is a 40% chance of exacerbation of her MG during pregnancy and an additional 30% risk in the puerperium. Maternal mortality risk is approximately 40 per 1,000 live births. Perinatal mortality approximates 68 per 1,000 births, five times that of uncomplicated pregnancies. Modern management minimizes these risks to the extent that pregnancy is not precluded in myasthenic women. A good outcome depends on meticulous maternal and fetal prenatal surveillance and early detection and management of exacerbations. Facilities and trained personnel must be available to support labor and manage vaginal or operative delivery. Intensive care of myasthenic crises is critical to the prevention of maternal complications and death.     

Cogan's syndrome complicating pregnancy

Cogan's syndrome is a rare autoimmune disorder characterized by systemic vasculitis as well as eye and ear inflammation. A 33-year-old G1 P0 with Cogan's syndrome experienced an uneventful pregnancy while being treated with hydroxychloroquine and prednisone. In the absence of systemic vasculitis, Cogan's syndrome may be associated with favorable pregnancy outcome.     

妊娠合并类风湿性关节炎对母胎的影响及诊治

类风湿性关节炎是一种自身免疫性疾病,在妊娠期,疾病症状有所缓解,而在产后加重,这种现象可能是多种机制作用的结果。妊娠期抗风湿药物的使用受到限制,其用药应依据病情活动性和避免对母儿的毒副反应进行,非甾体抗炎药、柳氮磺胺吡啶、羟氯喹和肿瘤坏死因子（TNF）抑制剂由于被证明在妊娠期使用较安全,可以用来治疗妊娠合并类风湿性关节炎。     

Recommendations of the AGG (Section Maternal Disease) for Myasthenia Gravis in Pregnancy

Myasthenia gravis is an autoimmune disease with a range of clinical presentations which manifest as combinations of weakness of the ocular, bulbar, and respiratory muscle groups and muscles of the extremities. Young women of reproductive age are most commonly affected. Preconception planning, the impact of pregnancy, prepartum management, drug therapy in pregnancy, myasthenic and cholinergic crises, fetal monitoring, peripartum management including analgesia and anesthesia during labor and cesarean section as well as neonatal management and neonatal myasthenia gravis are described here and the appropriate recommendations are given.Key words: myasthenia gravis, pregnancy, labor, neonatal features