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Systemic Lupus Erythematosus (SLE) and Pregnancy

Systemic Lupus Erythematosus (SLE) and Pregnancy. Mishelle M. Hernandez, M.D. Objectives. To discuss how pregnancy affects SLE in increasing lupus flare rates To discuss the effects of SLE on maternal and fetal outcome in pregnancy To discuss management of Lupus flare in pregnancy

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Systemic Lupus Erythematosus (SLE) and Pregnancy

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  1. Systemic Lupus Erythematosus(SLE) and Pregnancy . Mishelle M. Hernandez, M.D.

  2. Objectives • To discuss how pregnancy affects SLE in increasing lupus flare rates • To discuss the effects of SLE on maternal and fetal outcome in pregnancy • To discuss management of Lupus flare in pregnancy • To discuss ethical issues on the case

  3. General Data • K. G. • 18/F • Makati City • CC: bipedal edema • DOA: 3/18/08

  4. Diagnosed case of Systemic Lupus Erythematosus since Aug. 2007 1997 Revised Classification Criteria for Systemic Lupus Erythematosus [1] 1 Kliegman, Robert, M.D., et al. Nelson’s Textbook of Pediatrics. 18th ed. USA: Sanders, 2007, pp. 1015-191

  5. History of Present Illness 1 yr PTA (+) alopecia, (+) malar rash 9 mo PTA (+) fever, (+) discoid rash, (+) oral ulcers (+) R eyelid swelling (+) joint pain and swelling of hands RHEUMA CLINIC A> SLE Labs: ANA (+4) homogenous 1:80 leukopenia (3,800), anemia (10), lymphopenia (ALC 0.934) BUN 2.3 mol/L (N), Crea (N), Proteinuria(++), RBC 0-1

  6. History of Present Illness 2 mo PTA Pregnant discontinued Prednisone No consult done 1 wk PTA (+) persistence of cough (+) bipedal and periorbital edema 4 d PTA (+) persistence of edema (+) 2 pillow orthopnea (-) PND, palpitations, chest pain

  7. History of Present Illness 2 d PTA (+) easy fatigability (+) difficulty of breathing (+) vomiting (+) epigastric pain (+) diarrhea (+) tea-colored urine (+) oliguria Rheuma clinic consult PAY

  8. Review of Systems • General: (-) generalized weakness, (-) weight loss, (-) anorexia • Neurologic: (-) seizure, (-) headache, (-) change in sensorium, (-) change in behavior • HEENT: (-) eye pain, blurring of vision, (-) sore throat • Hematologic: (-)epistaxis, (-)hematemesis, (-) hematochezia, (-) hemoptysis, (-) easy bruisability, (-) increased bleeding, • Dermatologic: (-) active skin lesions

  9. Past Medical History Family History Birth/Maternal History Immunization History Nutritional History No intake of other Meds except Prednisone (+) similar illness – grandmother, paternal side noncontributory Completed at Local health center Unremarkable

  10. Developmental History Obstetrics/Menstrual History • At par with age • G1P0, (+) pregnancy test in February, • (+) spotting in February, (-) vaginal discharge • LMP: Dec 3, 2007, 30 days interval, 4 days duration, 3 pads/day, (+) dysmenorrhea • 2nd child from a brood of 9 • Mother is a 39 y/o,housewife. • Father is 45 y/o, nurse at PGH PICU. Personal/Social History

  11. HEADSS • Home • living with parents and siblings • good relationship with them (closest to her older sister) • Education • incoming 1st year college student, taking up BS Psychology • She didn’t finished first year due to her illness • plans to finish her study and work to help her parents

  12. HEADSS • Activity • hangs out with friends in the mall or in their house, go out preferably at night • love to talk about gossips • Drugs • Denies illicit drug use • occasional beverage drinker • doesn’t smoke

  13. HEADSS • Sex • one relationship and sexually active, with a 15 y/o guy, who is also the father of her present pregnancy • Her boyfriend impregnated another woman prior to her • no plans of getting married now • Suicidal ideations • when scolded by parents • felt very sad when she was diagnosed with SLE

  14. Physical Examination on Admission • General exam: conscious, coherent, not in cardiorespiratory distress • Vital signs: BP 140/80, PR 110, RR 24, T 38C, wt 47 kg, ht 151 cm • HEENT: slightly pale conjunctivae, anicteric sclera, (+) periorbital edema, bilateral • (-) cervical lymphadenopathy, (-) anterior neck mass, (-)neck vein engorgement, (-) tonsillopharyngeal congestion

  15. Physical Examination on Admission • Chest and Lungs: Equal chest expansion, no retractions, (+) clear breath sounds, (-) crackles/wheeze • Cardiovascular: adynamicprecordium, distinct HS, tachycardic, normal regular rhythm, AB at 5th LICS MCL, (-) murmur • Abdomen: globular abdomen, (+) NABS, soft, (+) epigastric tenderness, (-) organomegaly, abdominal girth = 76 cm, fundic height = 20 cm, fetal heart tone not appreciated by stethoscope

  16. Physical Examination on Admission • Internal examination: (+) vulvar edema, nulliparous vagina, corpus enlarged to AOG, cervix soft closed, (-) abnormal discharge or masses • Extremities: Pink nailbeds, FEP, (-) cyanosis, (+) bipedal edema, pitting, grade 1 • External genitalia: grossly female, SMR 4 • Skin: (-) active dermatoses • Neurologic exam: essentially normal

  17. Initial Impression SLE in activity Pregnancy Uterine 17 2/7 weeks by early UTZ, NIL UTI

  18. Problem List Pregnancy SLE Nephritis, Hypertension Pericarditis Anemia Pulmonary edema, noncardiogenic Pleural Effusion, B Infection

  19. Pregnancy

  20. Lupus Nephritis and Hypertension

  21. Lupus Pericarditis

  22. Anemia of Chronic Disease

  23. Pulmonary Edema and Pleural Effusion

  24. Infectious

  25. Ward stay – 17 days • PICU stay – 10 days • Discharged – on April 15, 2008 • Home Meds • Prednisone • Aspirin • Azathioprine • Nifedipine • Methyldopa • Hydralazine • Multivitamins • Folic acid • MgSO4 • Fe

  26. Whether pregnancy exacerbates lupus? • Among retrospective and prospective studies [2] • Lupus flare rates ranges from approximately 20% – 60% • Lupus that is active at the onset of pregnancy is activated further during pregnancy 2 Singh, Ajay K. Lupus nephritis and anti-phospholipid activity syndrome in pregnancy. Kidney International. Vol 58. (2000), pp 2240-2254.

  27. Table 1. Distribution of SLE flares occurring during pregnancy a [3] 3 Cortez-Hernandez, J., et al. Clinical Predictors of Fetal and Maternal Outcome in Systemic Lupus Erythematosus, a Prospective Study. Rheumatology. 2002; 41: 643-50.

  28. How to treat SLE flare during pregnancy? • Prednisone (1-2 mg/kg/day) – drug of choice for most SLE manifestation • Methylprednisone pulse 1g/day fowllowed by oral Prednisone at 0.5-1.0 mg/kg/day – severe systemic disease • Azathioprine (2 mg/kg/day) – for initial mild flare • Stress doses of Hydrocortisone – for emergency surgery, cesarean section, prolonged labor and delivery 5 Obstetric Emergencies: Management of Lupus Flare. www.obgmanagement.com. May 2006.

  29. 6 Mackillop, Lucy H., et al. Pregnancy plus Systemic Lupus Erythematosus. BMJ.2007; 335: 93336.

  30. Table 2. Evidence for adverse effects of immunosuppressant used in pregnancy and breastfeeding[6] 6 Mackillop, Lucy H., et al. Pregnancy plus Systemic Lupus Erythematosus. BMJ.2007; 335: 93336.

  31. Refractory cases • Rule: To treat the lupus flare before irreparable maternal harm occurs • Use of other new line immunosuppressive drugs • Benefits must be outweighed by potential risks • No conclusive data suggest pregnancy termination will ameliorate lupus flare. 5 Obstetric Emergencies: Management of Lupus Flare. www.obgmanagement.com. May 2006.

  32. Management Preconception Visit • counseled on appropriate timing of planned pregnancy • remission of at least 6 months and preferably more than 12 months and minimal or no need of immunosuppressives • Risks to patient and fetus are discussed in detail • The following baseline investigations are obtained at the start • CBC • Urea, creatinine, electrolytes • Liver function tests • ANA, anti dsDNA, aPL, anti-Ro/anti-La Mackillop, Lucy H., et al. Pregnancy plus Systemic Lupus Erythematosus. BMJ.2007; 335: 93336.

  33. Management after conception • follow-up frequency is dependent on disease activity • hydroxychloroquine is given to prevent flares • Low dose aspirin is administered to prevent preeclampsia • If APLS positive or history of thrombosis or fetal loss, treatment with heparin or LMWH and low dose aspirin Mackillop, Lucy H., et al. Pregnancy plus Systemic Lupus Erythematosus. BMJ.2007; 335: 93336.

  34. Management after conception • fetus is regularly monitored by obstetrician using Doppler UTZ • 20 weeks, a detailed morphology scan is done • Regular growth scans at 28, 32 and 36 weeks is done • If with anti-Ro and anti-La, fetal heart pulsed Doppler echocardiography at 18 weeks and 3rd trimester • Delivery method and timing depends on obstetric indications Mackillop, Lucy H., et al. Pregnancy plus Systemic Lupus Erythematosus. BMJ.2007; 335: 93336.

  35. Management after conception • Nutrition management • Megavitamin therapy • adequate dietary intake • Breastfeeding is contraindicated when taking the following drugs: mycophenolate, cyclophosphamide, methotrexate and leflunomide • Breastfeeding is appropriate if the maternal dose of prednisone is <30 mg/d, to take her medications just after breast-feeding Ferris, Ann M., et al. Nutritional consequences of chronic maternal conditions during pregnancy and lactation: lupus and diabetes. American Journal of Clinical Nutrition. 1994; 59 (suppl): 465S-73S.

  36. How SLE affects pregnancy? • Spontaneous abortion • Preeclampsia • IUGR • Fetal death rate • Preterm delivery • Thromboembolism • Lupus nephritis • Renal failure • Antiphospholipid syndrome • Active disease at conception • First presentation of SLE at pregnancy 7 Molad, Yair. Sytemic Lupus in Pregnancy. Current Opinion in Obstetrics and Gynecology.2006; 18: 613-617.

  37. Table 4. Fetal Outcome [8] 8 Valdez, Corazon, et al. Systemic Lupus Erythematosus in Pregnancy: a 23-year review. Acta Medica Philippina

  38. Updates on the Patient • On regular follow up to Rheuma, Renal, Perinatology • Maintained on Prednisone, Azathioprine, Aspirin, megavitamin • Controlled hypertension • Normal fetus on serial scans • EDC: Aug. 26, 2008the • Awaiting APAS • Father is alienating the patient.

  39. Conclusion • Whether pregnancy does exacerbate SLE is a controversial issue. • Women with SLE can have successful pregnancies. • In the care of lupus pregnant patient, the most diffiucult dilemma is saving both the mother and the unborn child.

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