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