SLE and pregnancy: Aboubakr Elnashar

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Systemic lupus erythematosus During pregnancy Aboubakr Elnashar Benha university, Egypt

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  • 1.Systemic lupus erythematosus During pregnancy Aboubakr Elnashar Benha university, Egypt

2. Incidence Women > men (ratio 9:1) During the child-bearing years (ratio 15:1). Incidence: 1 in 1000 women Aboubakr Elnashar 3. Clinical features Systemic connective tissue disease Heterogeneous: variety of clinical& antibody patterns. Other autoimmune disorders: 6%. Periods of disease activity (flares)& remissions. Average age at diagnosis: 30 y Aboubakr Elnashar 4. Commonest clinical feature (90%): Arthritis: nonerosive, peripheral, tenderness& swelling. Other features: Skin: (80%): malar rash Photosensitivity vasculitic lesions on the "fingertips& nail folds, Raynaud's phenomenon Discoid lupus. Aboubakr Elnashar 5. Serositis: pleuritis, pericarditis Renal: glomerulonephritis with proteinuria& cellular casts Neurological: psychosis, seizures or chorea. Haematological: haemolytic anaemia, thrombocytopenia & lymphopenia or leukopenia. Aboubakr Elnashar 6. Pathogenesis Cause: Un known Genetic predisposition Environmental triggers e.g. ultraviolet light or viral infection. Immunological: polyclonal B-cell activation impaired T-cell regulation of the immune response failure to remove immune complexes. Circulating non-organ-specific autoantibodies. Deposition of immune complexes: vasculitis. Aboubakr Elnashar 7. Diagnosis American Rheumatic Association criteria Many patients have a lupus-like illness without fulfilling these. 1. CBC: Normochromic normocytic anaemia Neutropenia Thrombocytopenia. 2. ESR: raised {high immunoglobulin levels} 3. CRP: Normal 4. 3rd or 4th components of complement: low or falling: active disease. Aboubakr Elnashar 8. 5. Auto-antibody a. The most common: Antinuclear antibody (ANA): (96%) Titres do not change with disease activity. b. The most specific antibodies to double-stranded DNA (78%) and Smith (Sm). Glomerulonephritis occurs more frequently in women with these antibodies. c. Other anti-Ro and anti-La: (30%) anticardiolipin antibodies (aPLs): (40%). Aboubakr Elnashar 9. Effect of pregnancy on SLE Flares: Increases: from 40% to 60%. When: at any stage of pregnancy or the puerperium more likely immediately postpartum: little evidence Prediction: not possible more likely if disease has been active within 6 ms of conception. Aboubakr Elnashar 10. Diagnosis during pregnancy: difficult {many features such as hair loss, oedema, palmar & facial erythema, fatigue, anaemia, raised ESR & musculoskeletal pain also occur in normal pregnancy}. most commonly involving the skin& joints Prevention: prophylactic steroids or routine increases of dose: no effect & not recommended Aboubakr Elnashar 11. Women with lupus nephritis SLE nephropathy may manifest for the first time in pregnancy. Risk of deterioration Moderate renal impairment (serum creatinine 125- 200 umol/I): uncomplicated pregnancies. High baseline serum creatinine: Inc risk of deterioration Aboubakr Elnashar 12. Preconception counseling. 1. Delay pregnancy until at least 6 ms after a lupus nephritis flare. 2. Prediction of the risks to the woman& fetus: anti-Ro/La aPLs Renal function Blood pressure Aboubakr Elnashar 13. Effect of SLE on pregnancy 1. The increased risks spontaneous miscarriage fetal death pre-eclampsia preterm delivery IUGR Related to 1. Age, parity 2. anticardiolipin antibodies 3. lupus anticoagulant 4. lupus nephritis 5. hypertension 6. active disease at the time of conception or 7. first presentation of SLE during pregnancy.Aboubakr Elnashar 14. During pregnancy, lupus improves in a third of women remains unchanged in a third worsens in the remaining third. Clinical condition can worsen or flare without warning (Khamashta and colleagues, 1997). Risk of major morbidity during pregnancy 7% (Petri, 1998) Lupus can be life threatening to both the mother and her fetus-infant. Aboubakr Elnashar 15. Good pregnancy outcome if: 1. Lupus activity has been quiescent for at least 6 months before conception 2. No active renal involvement manifest by proteinuria or renal dysfunction 3. Superimposed preeclampsia does not develop 4. No evidence of antiphospholipid antibody activity. Aboubakr Elnashar 16. Renal lupus: increased risk of fetal loss, PET, IUGR particularly if there is hypertension or proteinuria. Women in remission, but without hypertension, renal involvement or aPLs: Risk of pregnancy loss& PET is not higher than in the general population. 2. Chorea very rare complication of pregnancy in women with SLE or aPLs. Aboubakr Elnashar 17. Management Preconception counseling 1. Prediction of the risks to the woman& fetus: anti-Ro/La aPLs Renal Blood pressure. 2. Outcome is improved if conception occurs during disease remission. Aboubakr Elnashar 18. Multidisciplinary team in combined clinics: Physicians& obstetricians Monitor 1. disease activity 2. Fetal growth 3. uterine artery Doppler blood flow at 20-24 w 4. umbilical artery blood flow from 24 w Aboubakr Elnashar 19. Baseline values in early pregnancy: 1. FBC 2. KFT: U, creatinine, uric acid, quantify any proteinuria. 3. LFT: 4. anti DNA 5. Complement titres 5. Electrolytes Serial measurements at intervals dependent on disease severity Aboubakr Elnashar 20. Disease flare Symptoms: Arthralgia, pleuritic pain, skin rash Lab: A. Urine: Red blood cells or cellular casts B. Rising anti-DNA antibody titre C. Fall in complement levels >25% fall in C3 or C4 suggests active SLE. N.B. elevation of complement split products, particularly Ba & Bb, often accompanies flares, so high ratios of CH50: Ba may differentiate PET from active lupus. Aboubakr Elnashar 21. Treatment Corticosteroids are the drugs of choice. Aboubakr Elnashar 22. Hydroxychloroquine (Plaquenil) should be continued {stopping may precipitate flare}. For control of hypertension: Drug of choice: methyldopa 2nd -line agents: nifedipine or hydralazine Although long-term hydralazine& methyldopa use may rarely induce a SLE-like syndrome, they are not contraindicated in SLE. Aboubakr Elnashar 23. Differentiation of active renal lupus from PET Difficult 1. The two conditions may be superimposed. 2. Hypertension, proteinuria, thrombocytopenia& even renal impairment are all features of PET 3. A doubling of baseline proteinuria may be expected in pregnancy but more than this would be indicative of either worsening lupus nephritis or PET} Aboubakr Elnashar 24. How: 1. Hyperuricaemia & abnormal LFT point more towards PET. 2. Renal biopsy The only definitive investigation Rarely undertaken in pregnancy. More likely to be appropriate prior to fetal viability Aboubakr Elnashar 25. TT of active lupus nephritis Increase oral prednisolone Pulsed IV methyl predisolone Azathioprine (Imuran). Cyclophosphamide: Rarely used Aboubakr Elnashar 26. If lupus flare & PET cannot be dd beyond 24-28 w, when the fetus is viable: Delivery {cure PET allow administration of cyclophosphamide} Aboubakr Elnashar 27. Conclusions There is an increased rate of flare during pregnancy. Disease flares must be actively managed with corticosteroids. Adverse pregnancy outcome is related to the presence of renal involvement, hypertension, antiphospholipid antibodies& disease activity at the time of conception. These factors increase the risks of spontaneous miscarriage, fetal death, PET, PTL& IUGR. Aboubakr Elnashar 28. Pregnancy care is best undertaken in combined clinics allowing close monitoring of disease activity, fetal growth& well-being. In Ro-positive mothers risk of transient neonatal cutaneous lupus: 5% risk of CHB: 2%. Aboubakr Elnashar 29. Aboubakr Elnashar Thanks