Autoimmune disease

1. 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:

2. - 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

3. 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

4. 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.

5. 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

6. 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.

7. 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

8. 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.

9. 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

10. Reducing the risk of thrombosis through the use of antithrombolytic therapy during pregnancy improves pregnancy outcome

11. 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

12. -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

13. -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

15. 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

16. 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

17. 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

18. -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

19. - 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

20. - 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.

21. 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

22. -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

23. 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

24. 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;

25. -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.

26. 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