Transcript Autoimmune disease

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:
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- 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 antiinflammatory 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.
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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
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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:
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- 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