Sunday 30 June 2013

Systemic lupus erythematosus in Pregnancy

Systemic lupus erythematosus in Pregnancy

  • SLE is known to increase the risk of spontaneous miscarriage; it can also cause fetal growth restriction and increased rates of sudden intrauterine death, pre-eclampsia and preterm delivery
Pre-pregnancy counselling :

  • The presence of anti-Ro/La and 
    antiphospholipid antibodies should  be determined. 
    These antibodies are associated with congenital heart block 
    and neonatal cutaneous lupus syndrome. 
    Antiphospholipid 
    antibodies are present in about 30% of women with SLE and 
    are associated with arterial and venous thrombosis, recurrent 
    miscarriage, fetal growth restriction, fetal loss and preterm 
    delivery due to uteroplacental insufficiency.
  • Quiescent disease, without associated antiphospholipid syndrome (APS), hypertension or renal involvement the risks of miscarriage/stillbirth and fetal growth restriction are not significantly increased
  • The risk of miscarriage and stillbirth in pregnancies complicated by lupus varies in the literature from 6–35% and from 0–22%
  • The great concern are women with SLE and associated pulmonary arterial hypertension who wish to conceive: maternal mortality rates have been quoted as being as high as 33%
  • Women should  be advised not to conceive during a period of active disease, particularly with lupus nephritis, because of worse maternal and fetal outcomes
  • Counsel women with SLE for increased risk of pre-eclampsia, preterm delivery and fetal growth restriction
  • In active lupus nephritis with worsening renal function, increasing proteinuria and hypertension,it may be necessary to use such treatments as cyclophosphamide and mycophenolate mofetil, which are associated with congenital malformations if used in the first trimester,
  • Pregnant women with active SLE/lupus nephritis or anti-Ro/La/antiphospholipidantibodies should be considered as a higher risk group and managed in tertiary centre
  • If severe disease, patients need hospitalisation
  • For patients with stable disease, 4-weekly reviews of disease activity and regular assessment of fetal growth, blood pressure and proteinuria are acceptable.
  • For those women who are anti-Ro/La positive the fetal heart rate should be monitored and recorded at each visit and fetal echocardiography assessments made at 18–20 and ~28 weeks of gestation
  • Women who have had a previous venous thromboembolism  should receive thromboprophylaxis with low molecular weight heparin throughout pregnancy and for 6 weeks postpartum 
  • The risk of an SLE flare in pregnancy is increased with active disease in the 3–6 months prior to conception, with the majority of flares occurring after the 20th weeks of pregnancy
  • Most flares are  managed expectantly with medical management and adjustments to drug therapy
  • At every antenatal visit, measure the BP, test the urine for proteinuria,  and quantify the proteinuria by PCR or 24 hour urine proetien estimation.
  • The features of lupus nephritis include hypertension and proteinuria with or without haematuria and renal impairment
  • The presence of hematuria or red cell casts as well as a rise in anti-dsDNA titres or a fall in complement levels help to distinguish this from pre-eclampsia
  • In cases of lupus nephritis that fail to respond to increasing dosages of steroids and azathioprine, and where there is a deterioration of renal function and/or hypertension, other immunosuppressive drugs may be considered, such as mycophenolate mofetil or tacrolimus. Such management decisions should be undertaken in consultation with nephrologists and rheumatologists.
  • Premature rupture of membranes is also regarded as being more frequent in pregnancies complicated by SLE; rates vary and are generally quoted as ~20% and is common in women who are on steroids
SLE and fetal risks
  • Increased rates of miscarriage,
  • Increased risks of congenital heart block, fetal growth restriction and increased rates of preterm delivery 
  • Scanning at least every 4 weeks to screen for fetal growth restriction in those women at risk is generally accepted and fetal echocardiography referral should be arranged for those with anti-Ro/La antibodies
  • Doppler studies can be used to estimate placental function
  • A uterine artery Doppler should be first carried out at 20 weeks and repeated 4 weeks later if any abnormality is found
  • A raised pulsatility index or diastolic notching are associated with increased risk for developing  pre-eclampsia, as they can indicate underlying placental dysfunction
  • Congenital heart block is associated with maternal anti-Ro/La autoantibodies.
  • usual presentation is a fixed fetal bradycardia of 60–80 beats per minute on ultrasound scan.
  •  It occurs in 2–3% of fetuses of women with the anti-Ro/La antibody 
  • CHB is associated with significant perinatal morbidity and mortality, with about half of infants requiring pacing by the first year of life. 
  • Congenital heart block develops between 18– 28 weeks of gestation and fetal echocardiography should be performed around this period to detect it. Hydrops fetalis can occur in utero and is thought to be due to the degree of endomyocardial fibrosis and associated myocarditis.
Neonatal lupus rash

  • lesions are  commonly seen on the face and scalp 
    and appear typically after ultraviolet exposure in the first 
    2 weeks of life, but can appear  up to 3–6 months postpartum
Drug treatment in SLE

  • Glucocorticoids, mainly in the form of prednisolone, are frequently but not exclusively used as one of the first-line treatments in pregnancy
  • Women on moderate to high dosages of steroids should should be screened regularly for gestational diabetes.
  • Immunosuppressants  that are used and are generally considered safe during pregnancy include azathioprine and hydroxychloroquine. There is no  need to discontinue them during pregnancy
Postpartum management

  • To consider thromboprophylaxis
  • Manage hypertension







            Tuesday 25 June 2013

            How to differentiate Amniotic fluid embolism and Pulmonary embolism?




            Amniotic Fluid Embolism
            Pulmonary Embolism
            Onset of symptoms
            Usually during labour
             Any time in pregnancy
            Early symptoms
            Dyspnoea, restlessness, panic, feeling, cold, paraesthesiae. Usually no pain
            Breathlessness, , cough, haemoptysis,
            pleuritic pain
            Coagulopathy
            Present
            Absent
            ECG
            Not helpful
            S1Q3T3
            Chest x-ray:
            Pulmonary oedema, ARDS, right atrial
            enlargement, prominent pulmonary artery.

            Segmental collapse, raised
            hemidiaphragm, unilateral effusion
            CTPA
            Negative
            Positive
            Complement levels
            Raised
            Normal
            Serum Fetal Sialyl Tn antigen
            Present
            Absent

            Nine maternal risks of Obesity and Three fetal risks of maternal obesity

             





            Mother

            1.   Severe morbidity – surgical or aneshtetic
            2.   Cardiac disease
            3.   Spontaneous miscarriage
            4.   Pre-eclampsia
            5.   Gestational diabetes
            6.   Thromboembolism
            7.   Post caesarean wound infection
            8.   Infection from other causes
            9.   Post partum haemorrhage
            Baby

            1.   Stillbirth and neonatal death

            2.   Congenital abnormalities (can be missed by scan)

            3.   Prematurity

            Reasons why tachycardia is important in a pregnant patient?








             Presence of tachycardia is the most significant clinical feature of an unwell patient and is regularly ignored or missed   or misinterpreted .



            Measurements of respiratory rate and heart rate are seriously more important than measurements of blood pressure.



            A normotensive patient may all too often be unwell and compensating.



             A tachycardic patient is hypovolemic until proved otherwise.



            A patient with tachypnea has a cardiorespiratory cause until proved otherwise.



            Do not attribute presence of tachycardia and tachypnea to anxiety

            Three most important causes of abdominal pain in later stages of pregnancy


            1. PET, eclampsia and HELLP syndrome, especially if the pain is epigastric or accompanied by jaundice 
            2. Placental abruption   
            3. Sepsis

            Ten Red Flag Signs of Maternal sepsis




            1.   Pyrexia > 38_C

            2.   Sustained tachycardia > 100 bpm

            3.   Breathlessness (RR > 20)

            4.   Abdominal or chest pain

            5.   Diarrhoea and/or vomiting

            6.   Reduced or absent fetal movements, or absent fetal heart

            7.   Spontaneous rupture of membranes or significant vaginal discharge

            8.   Uterine/renal angle pain and tenderness

            9.   appears or feels generally unwell 

            10.        Unduly anxious, distressed 

            The combination of any of the above two makes a seriously valid case for correct thought process to rule out maternal sepsis. Of all the vital signs respiratory rate is the most important variable for assessing the clinical situation of the patient. Respiratory rate is the most sensitive indicator of patient's physiological well being.