Obstetric Complications in Women With Connective Tissue Disorders - An Overview

Introduction

Connective tissue is the structure that holds the body together and is primarily composed of collagen and elastin. There are several types of connective tissue disorders. Pregnancy can be extremely unpredictable for women suffering from autoimmune disorders. Some autoimmune illnesses improve during pregnancy and are unlikely to cause major obstetric difficulties. Others may worsen during pregnancy, causing maternal deterioration as well as obstetric and perinatal problems. In systemic lupus erythematosus, placental transmission of particular autoantibodies can result in fetal or neonatal illness. Hereditary connective tissue disorders (HCTDs) include conditions including Ehlers-Danlos syndrome, Marfan's syndrome, and pseudoxanthoma elasticum.

What Are Mixed Connective Tissue Disorders?

This was formerly thought to be an overlap or mixture of systemic lupus erythematosus (SLE), scleroderma, and polymyositis. Patients exhibit features of all three diseases. They typically exhibit very high levels of antinuclear antibodies (ANAs) and antibodies to ribonucleoprotein (anti-RNP). Frequently, the disease evolves to become dominated by symptoms of one of the three component disorders, most notably scleroderma.

Unclassified connective tissue disorders (UCTDs) are those that have some features of connective tissue disorders, such as anti-nuclear antibodies, or features of three separate diseases but cannot be classified as one. Follow-up on unclassified connective tissue disorders suggests that approximately 20 percent of patients develop a particular condition. The inherited forms typically manifest early in childhood. They are rarely visible at birth but are typically diagnosed before the onset of reproduction. The autoimmune variants usually appear after the reproductive years, but they can also appear before middle age. Reproductive difficulties could be the presenting aspect.

How Frequent Are Obstetric Complications in Women With Connective Tissue Disorders?

Hereditary connective tissue disorder are often inherited in a Mendelian fashion as autosomal dominant. Males and females are affected equally. Autoimmune disease are acquired rather than inherited, however they often run in families. The specific cause is unknown, although if there is an extrinsic trigger, it appears that a genetic predisposition is involved.

Pregnancy in Inherited Connective Tissue Diseases

Genetic counseling is recommended for inherited types, preferably before the woman gets pregnant.

Ehlers-Danlos Syndrome

The risk varies significantly depending on the type of condition.

• Obstetric problems include the danger of uterine rupture during labor, vaginal and perineal injury, and bleeding and rupture of blood vessels and the intestinal tract during the puerperium.

• Type IV patients are at risk of serious bleeding and artery rupture due to pregnancy.

• A Shirodkar's suture may be necessary to address cervical incompetence.

• Ehlers-Danlos syndrome is generally well tolerated during pregnancy, except for type IV.

Marfan's Syndrome

• The most dangerous for women with Marfan's syndrome is aortic dissection during pregnancy. Family history may indicate the level of risk, but echocardiography to check the aortic root during pregnancy is recommended.

• Aortic dissection is not the primary problem hence a multidisciplinary approach is necessary.

• Surgery may be required before beginning a pregnancy. The aortic repair can be performed during pregnancy.

• Beta-blockers may be protective, although they have been linked to an increased risk of low birth weight kids.

Pseudoxanthoma Elasticum, or PXE

• PXE is distinguished by the degradation of elastic tissues. Clinically, its effects are mainly noticeable in the skin, retina, blood vessels, and myocardium.

• It is not related to an increase in fetal loss or bad outcomes. Some pregnancies are associated with worsening skin symptoms.

• Gastric hemorrhage is likely more common in afflicted people than in the general population.

• Retinal problems are infrequent.

Pregnancy and Autoimmune Connective Tissue Diseases

• In addition to any direct symptoms of the connective tissue problem, the woman may be taking medicines for the condition that are teratogenic, such as Methotrexate, which induces abortion. The relative hazards and benefits of the various medicines in pregnancy must be carefully considered.

Fetal Loss

• The risk of fetal loss is significantly higher than in the general population, and this is especially true for persons with SLE or a component of the disease in their connective tissue disorder.

• The largest risk is for persons with antiphospholipid antibodies, and the issue of pregnancy is explored in a separate page on Antiphospholipid Syndrome. Miscarriage is not only common in the first trimester, but there is also a large risk of intrauterine death in the second and third trimesters.

For women with SLE, the abbreviation 'PATH' represents:

• Proteinuria.

• Antiphospholipid syndrome.

• Thrombocytopenia.

• Hypertension occurs early in pregnancy.

All of them are significant risk factors for poor pregnancy outcomes.

Pregnancy and Rheumatoid Arthritis

• Rheumatoid arthritis (RA) is rather frequent among women of reproductive age. Women with rheumatoid arthritis have reduced fertility due to the use of specific drugs and active disease. RA often improves during pregnancy, although nearly half of patients still have active illness in the third trimester. Pregnancy outcomes are slightly less favorable, particularly in women with high disease activity. Managing rheumatoid arthritis while pregnant is difficult because therapy options are restricted.

Effects on Fertility and Pregnancy Problems

• Women with rheumatoid arthritis (RA) have fewer children than the general population, but this appears to be due to a mix of decreased fertility and a purposeful decision to cope with the difficulties of childrearing and the restrictions of the condition.

• Pregnancy hypertension affected 23.2 percent of individuals with systemic lupus erythematosus and 11.1 percent of those with rheumatoid arthritis, compared to 7.8 percent of the general population.

• They had more cesarean sections and spent longer in the hospital.

• They were also older than the normal population when having babies, but this did not explain the difference.

What Medications Are Recommended During Pregnancy and Breastfeeding?

The safety of the medications most commonly used for connective tissue disorder during pregnancy and breastfeeding. Although nonsteroidal anti-inflammatory medicines (NSAIDs) have traditionally been avoided during pregnancy, they are generally safe to use if taken in short, limited doses. Although they are not teratogenic, prolonged use can lead to renal and cardiac failure, hypertension, and fluid overload in the mother, as well as oligohydramnios and renal impairment in the fetus. They should be avoided near the end of pregnancy (>30-32 weeks) since they raise the danger of the baby's ductus arteriosus closing prematurely, as well as the risk of maternal hemorrhage and asthma in the child. Taking colchicine throughout pregnancy looks to be safe.

Conclusion

Connective tissue diseases are linked to numerous intentional and involuntary abortions, as well as intrauterine fetal deaths. Even among women who have live births, many undergo cesarean procedures for a variety of reasons, including fetal distress, a poor biophysical profile, a non-reassuring non-stress test, and so on. Systemic sclerosis is linked to an increased risk of preterm birth (14 to 29 %), intrauterine growth restriction, prolonged hospital stays, and miscarriage in women with long-standing diffuse scleroderma. Women with minimal scleroderma tend to have better pregnancy outcomes than those with extensive disease.