Autoimmune disease
• -a disruption in the function of the immune system of the body, resulting in the production of antibodies against the body's own cells.
• -The cause of these conditions is unknown but it is thought to be multifactorial with:
• - genetic • -environmental• -hormonal• - viral influences. • -Many autoimmune diseases are more
prevalent in women, particularly between puberty and the menopause
• - suggests that female hormonal factors may play a role
• 1 Multisystem disease such as systemic lupus erythematosus (SLE).
• 2 Tissue- or organ-specific disorders such as autoimmune thyroid disease.
• -these disorders are characterized by periods of remission interrupted by periods of crisis, which may require hospitalization
• Treatment is aimed at lessening the severity of the symptoms rather than effecting a cure.
• -Mild cases usually respond to anti-inflammatory drugs; more severe illnesses may require steroids or immunosuppressant therapy.
Systemic lupus erythematosus
• (SLE), or lupus, is an autoimmune, connective tissue disorder
• SLE produces multisystem disorders affecting muscles, bone, skin, blood, eyes, nervous system, heart, lungs and kidneys.
• Infection is the major cause of mortality at all stages of SLE; early deaths are usually due to active SLE and late deaths are attributed to thromboembolic disorders
Diagnosis
• a collection of signs and symptoms particularly when joint pain, skin conditions and fatigue.
• The initial manifestation of SLE is often arthritis accompanied by fever, fatigue, malaise, weight loss, photosensitivity and anemia.
• skin lesions are seen and an erythematous facial ‘butterfly’ rash is characteristic of the disorder.
• pruritus, pericarditis, glomerulonephritis, neuritis and gastritis may arise.
• Renal disease and neurological abnormalities are the most serious manifestations of the disease.
• Blood tests are used to confirm the diagnosis andCBC, (ESR) and testing for antinuclear antibody (ANA).
• There is often norm chromic normocytic anemia
Antiphospholipid syndrome (Hughes syndrome)
• -Antiphospholipid syndrome (APS) is a prothrombotic disorder .
• -characterized by :• -arterial and/or venous thrombosis• - recurrent spontaneous miscarriage• - neurological disease including stroke). • -Approximately 30–40% of women with SLE
have aPL antibodies and some will develop APS.
• A blood test will detect aPL and lupus anticoagulant.
• -APS in conjunction with SLE increases the risk of :
• 1-thromboembolic disorders in pregnancy • 2- a higher risk of pregnancy loss• 3- intrauterine growth restriction• 4- placental insufficiency• 5- pre-eclampsia• 6- pre-term birth
• Reducing the risk of thrombosis through the use of antithrombolytic therapy during pregnancy improves pregnancy outcome
Effects of SLE on pregnancy
• lupus flares (worsening of SLE symptoms) • -it will become active during the course of the
pregnancy. • -Exacerbation of SLE with major organ
involvement (such as the kidneys and central nervous system) may occur in approximately 20% of cases .
• - fetal risk include : spontaneous abortion, therapeutic abortion, intrauterine death or stillbirth
-maternal effect include
• 1- Maternal renal disease • 2-fetal loss• 3- development of pre-eclampsia • 4- intrauterine growth restriction.• -Neonatal lupus syndrome is rare but may
occur as a result of the transplacental passage of maternal IgG autoantibodies
• -The neonate presents with a mild form of lupus that is transient and resolves when the antibodies are cleared in a few months following birth.
• - A more severe form of the disease results in fetal anemia, leucopenia and thrombocytopenia.
• -When anti-Ro and/or anti-La antibodies have passed to the fetus, then there is a risk of developing congenital heart block (CHB), which is permanent and carries significant morbidity and mortality- Over 60% of affected children require lifelong pacemakers
Preconception care
• management of SLE should start before conception so that baseline assessments and alterations to drug therapy can be undertaken.
• - It is recommended that the disease has been in remission for at least 6 months prior to conception.
• - SLE in conjunction with pulmonary hypertension, renal nephritis or APS confers a high risk of maternal morbidity and mortality
Antenatal care
• -Antenatal care should be provided by a multidisciplinary team.
• -The frequency of antenatal visits is dependent on the severity of the disease
• - women with SLE may have additional social and psychological needs
Baseline investigations include:
• - full blood count• - urea, creatinine and electrolytes• - liver function tests• - immunological blood tests to detect antibodies• - blood pressure• - urinalysis and 24 hrs urine collection for
creatinine clearance and total protein to assess renal function
• -u\s is undertaken to confirm fetal viability
• -Women with SLE and APS are offered a fetal cardiac anomaly scan at 24 weeks' gestation and echocardiography to detect CHB
• -careful monitoring of fetal growth and well-being by:• 1- ultrasound examinations for fetal growth• 2- placental Doppler studies • 3-amniotic fluid volume• 4- CTG. • 5-Doppler assessment of uterine artery blood flow
studies at 20–24 weeks to predict pre-eclampsia and intrauterine growth restriction
• - Avoidance of emotional stress and the promotion of a healthy lifestyle may play a part in reducing exacerbations of SLE arising during pregnancy.
• - exercise may be utilized by women to reduce the effects of pain, joint stiffness and fatigue.
• - Simple analgesics such as paracetamol and codeine derivatives may be used.
• - Women who have a mild form of the disease or are in remission require minimal to no medication
• - prednisolone (up to 10 mg/day) For mild cases• -Anti malarial drugs are effective (hydroxychloroquine)
is considered safe to use in pregnancy.• -immunosuppressant drug . • -Women with SLE and APS have associated recurrent
miscarriage, thrombosis and thrombocytopenia• - it is recommended that treatment with anticoagulants
such as low dose aspirin and/or heparin • -Thromboprophylaxis promotes successful embryonic
implantation and protects against thrombosis.
Intrapartum care
• normal labor and vaginal birth should be the aim.
• healthcare professionals involved: the midwife, obstetrician, rheumatologist, anaesthetist, paediatrician and haematologist.
• The woman and her family should continue to be involved in the development of the care
• -Women with SLE are particularly prone to :• infection, hypertension, thrombocytopenia and
thromboembolic disorders
-midwifery care to reduce infection
• 1-Careful hand-washing• 2-strict aseptic techniques with invasive
procedures • 3-limiting the number of vaginal examinations
will reduce the risk of infection.• -Close monitoring of the maternal condition is
required by the midwife, obstetrician and anaesthetist to evaluate cardiac, pulmonary and renal function
• Blood tests should be undertaken to screen for hematological conditions, which may lead to clotting disorders.
• - Comfort measures, the use of TED stockings can reduce the risk of pressure sores and the development of deep vein thrombosis.
• - parenteral steroid should be given during labor.
• - continuous fetal monitoring in conjunction with fetal blood gas estimation is recommended
Postpartum care
• observe closely for: • signs of SLE flares that may occur as a result of
the stress of labour• signs and symptoms of infection• pre-eclampsia• renal disease• thrombosis and neurological changes. • -most of the drugs used to treat SLE are
excreted in breast milk: paracetamol is the drug of choice for postpartum analgesia;
• -low dose steroids and hydroxychloroquine are considered safe
• - immunosuppressive therapy is contraindicated;
• -large doses of aspirin should be avoided and non-steroidal anti-inflammatory drugs (NSAIDs) are contraindicated when breastfeeding jaundiced neonates.
• advising women with regard to her contraceptive options
• -Combined oral contraception increases the risk of hypertension, thrombosis and SLE flares.
• -Low dose oestrogen combined pills may be considered in women with well-controlled SLE without a history of thromboembolic disease or APS.
• - Intrauterine contraceptive devices are associated with an increased risk of infection in SLE women.
• - Progestogens and barrier methods represent the safest options and may be suitable for those women