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d fetal outcome in pregnancy To discuss management of Lupus flare in pregnancy To discuss ethical issues on the case
Kliegman, Robert, M.D., et al. Nelsons Textbook of Pediatrics. 18th ed. USA: Sanders, 2007, pp. 1015-191
1 yr PTA 9 mo PTA
(+) alopecia, (+) malar rash (+) 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
9 mo PTA
(+) fever (+) discoid rash (+) oral ulcers (+) R eyelid swelling (+) joint pain and swelling of hands Consult at PGH OPD Rheuma
2 mo PTA
1 wk PTA
4 d PTA
Pregnant discontinued Prednisone No consult done (+) persistence of cough (+) bipedal and periorbital edema (+) persistence of edema (+) 2 pillow orthopnea (-) PND, palpitations, chest pain
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
7 mo PTA
5 mo PTA
Prednisone (1 mkd) (-) alopecia, oral ulcers, eyelid swelling, malar rash arthritis (-) proteinuria Prednisone tapered Lost to follow-up but asymptomatic
2 mo PTA
2 wks PTA
Pregnant discontinued Prednisone No consult done (+) fever (+) cough (-) dyspnea Meds: Paracetamol Bromhexine syrup
1 wk PTA
(+) persistence of cough (+) bipedal and periorbital edema (+) persistence of edema (+) 2 pillow orthopnea (-) PND, palpitations, chest pain
4 d PTA
2 d PTA
(+) easy fatigability (+) difficulty of breathing (+) vomiting (+) epigastric pain (+) diarrhea (+) tea-colored urine (+) oliguria Rheuma clinic consult PAY
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
No intake of other Meds except Prednisone (+) similar illness grandmother, paternal side noncontributory Completed at Local health center Unremarkable
Family History
Birth/Maternal History
Immunization History
Nutritional History
(-) other illness (-) hospitalization (-) allergy (-) accidents Medications: no intake of Sulfonamide, Minocycline, anti-TNF biologics, Thiazides, Ca channel blockers, ACEI, MV, FeSO4 and Folic acid
(+) HPN, CA grandmother, paternal side (+) kidney disease maternal side (+) similar illness grandmother, paternal side (-) DM, BA, PTB, CA, liver disease
Full term via spontaneous vaginal delivery to a then 25 y/o G2P1 (1001) mother at a lying in clinic c/o midwife. Regular prenatal check-up, (-) fetomaternal illness, (-) birth complications good cry and good activity
(+) BCG (+) OPV 3 doses (+) DPT 3 doses (+) Hepa B 3 doses (+) measles
Developmental History
Obstetrics/Menstrual History
G1P0, (+) pregnancy test in February, (+) spotting in February, (-) vaginal discharge LMP: Dec 3, 2007, 30 days interval, 4 days duration, 3 pads/day, (+) dysmenorrhea
Personal/Social History
2nd child from a brood of 9 Mother is a 39 y/o,housewife. Father is 45 y/o, nurse at PGH PICU.
breastfed for 6 months shifted to formula milk eats regular table food
2nd child from a brood of 9 Mother is a 39 y/o,housewife. Father is 45 y/o, nurse at PGH PICU.
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 didnt finished first year due to her illness plans to finish her study and work to help her parents
Education
incoming 1st year college student, taking up
BS Psychology She didnt finished first year due to her illness plans to finish her study and work to help her parents.
Activity
hangs out with friends in the mall or in their
Drugs
Denies illicit drug use occasional beverage drinker doesnt smoke
Drugs
Denies illicit drug use occasional beverage drinker doesnt smoke
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
Suicidal ideations
when scolded by parents felt very sad when she was diagnosed with
SLE
G1P0, (+) pregnancy test in February, (+) spotting in February, (-) vaginal discharge LMP: Dec 3, 2007, 30 days interval, 4 days duration, 3 pads/day, (+) dysmenorrhea
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
Chest and Lungs: Equal chest expansion, no retractions, (+) clear breath sounds, (-) crackles/wheeze Cardiovascular: adynamic precordium, 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
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
Conscious, coherent Oriented to time, place and person (-) anosmia, visual acuity 20/20 OU, pupils 2-3 mm EBRTL, EOMS full and equal, (+) corneal, (-) facial asymmetry, (-) gross hearing loss, (+) gag reflex, (+) good shoulder shrug, (+) tongue midline Motor: 5/5 on all extremities Sensory: (-) sensory deficit DTR: (++) on all extremities (-) Babinski Cerebellar: (-) nystagmus (-) nuchal rigidity
RHEUMA CLINIC
periorbital and bipedal edema easy fatigability difficulty of breathing vomiting epigastric pain diarrhea tea-colored urine oliguria
PAYWARD
Serositis (pericarditis)
Renal involvement (lupus nephritis, hypertension, renal failure, nephrotic syndrome) Anemia (normochromic, normocytic)
SLE in activity Pregnancy Uterine 17 2/7 weeks by early UTZ, NIL UTI
1. 2.
3. 4.
Pregnancy SLE Nephritis, Hypertension Pericarditis Anemia Pulmonary edema, noncardiogenic Pleural Effusion, B Infection
O Pregnancy Test (+) UTZ: Pregnancy Uterine 17 2/7 weeks, good cardiiac and somatic acrtivities
P For APAS Serial Fetal biometry Aspirin FeSO4, CaCO3, MgSO4, Folic acid, MV
S Edema Hematuia
O BP 140/80 on admission, BP spikes of 160/100) Proteinuria on urinalysis and 24 hr urine collection (+) fine, coarse, waxy casts Raised creatinine
P For Biopsy Albumin transfusion Prednisone and Azathioprine MPPT Multidrug antiHPN
(-) signs of Lupus cardiac Pericarditis tamponade CXR: cardiomegaly 2D echo : mod pericardial effusion, RA and RV wall collapse, fair LV systolic function
O On admission, Hgb = 82 mg/dl At PICU, Hgb = 54 mg/dl Retic index 0.05 Direct and Indirect Coombs (-)
P BT of PRBC
O Moderate cardiorespiratory distress ABG metabolic acidosis CXR: Bilateral pleural effusion Inhomogenous opacities BLF Pulmonary infiltrates hypoalbumine mia
O UTI
P Cefuroxime Ceftazidime
1. On admission U/A: pyuria 2. At PICU Blood CS: NG5d Urine CS: Micrococcus luteus U/A: pyuria
Nosocomial sepsis
Ward stay 17 days PICU stay 10 days Discharged on April 15, 2008
Home Meds x Prednisone x Aspirin x Azathioprine x Nifedipine x Methyldopa x Hydralazine x Multivitamins x Folic acid x MgSO4 x Fe
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.
Manifestations
No. of Flares (% Total) 27 (69%) 13 (33%) 4 (10%) 4 (10%) 1 (3%) 3 (8%) 1 (3%) 1 (3%)
1st Trimester 3 3 0 0 0 0 0 0
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.
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
Table 2. Evidence for adverse effects of immunosuppressant used in pregnancy and breastfeeding[6]
Whether drug can be used Drug
Hydroxychloroquine/ Chloroquine
Evidence
No increased risk of miscarriage, congenital malformation, stillbirth at doses 200-400 mg/day Cessation increase risk of flare Long half life means stopping does not prevent fetal exposure Metabolized by placenta In high doses have caused cleft palate in experimental animal models and low birth weight in humans Fetus lacks enzyme to convert to active form Fetal and neonatal immunosuppression minimal if dose is <2 mg/kg and maternal white cell count is normal No increase in congenital malformation Prematurity and IUGR trends not significant Small amounts in breastmilk but no adverse effects noted No increase in congenital malformation Increased rates of prematurity related to maternal disease In one case report, a baby received 0.02% of maternal dose via breastmilk Cross the placenta after 32 weeks but with no adverse effects to fetus Increased risk of congenital abnormalities Enterohepatic recirculation Long half life Alkylating agent Teratogenic, fetotoxic Risk of suppression of neonatal hematopoiesis Folate antagonist Teratogenic and Fetotoxic Congenital abnormality in animal studies Human studies limited Long half life of active metabolites Limited experience in human pregnancies but no adverse fetal or neonatal outcomes to date
In pregnancy
Y
In breastfeeding
Y
Ciclosporin
Tacrolimus
Y N (stop 6 weeks before conception) N (stop 3 months before conception) N (stop 3 months before conception and give Folic acid 5 mg daily) N (use cholestyramine to increase clearance preconception) Limit to severe disease
Cyclophosphamide
Methotrexate
Leflunomide
Probably avoid
6 Mackillop, Lucy H., et al. Pregnancy plus Systemic Lupus Erythematosus. BMJ.2007; 335: 93336.
In pregnancy Y
In breastfeeding Y
Azathioprine
6 Mackillop, Lucy H., et al. Pregnancy plus Systemic Lupus Erythematosus. BMJ.2007; 335: 93336.
Rule: To treat the lupus flare before irreparable maternal harm occurs Use of other new line immunosuppressive drugs
risks
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.
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.
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
Mackillop, Lucy H., et al. Pregnancy plus Systemic Lupus Erythematosus. BMJ.2007; 335: 93336.
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.
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.
Mortality Full term delivery Preterm delivery Abortion Total 2 (5%) 12 (28%) 8 (19%) 22 (52%)
8 Valdez, Corazon, et al. Systemic Lupus Erythematosus in Pregnancy: a 23-year review. Acta Medica Philippina
Table 5. Diagnostic criteria for the antiphospholipid syndrome Antiphospholipid antibodies plus at least one of the following: y Arterial or venous thrombosis y Three or more miscarriages (at <10 weeks gestation) y Fetal death (at >10 weeks gestation with normal fetal morphology)
In pregnant women
repeated abortions , risk of unexpected intrauterine
deaths, venous/arterial thrombosis, thrombocytopenia, pregnancy-induced hypertension, chorea, multi-system organ failure and post-natal depression
Singh, Ajay K. Lupus nephritis and anti-phospholipid activity syndrome in pregnancy. Kidney International. Vol 58. (2000), pp 2240-2254.
generalized photosensitive rash thrombocytopenia and anemia giant cell hepatitis with severe cholestasis isolated complete heart block or cardiomyopathy If the fetus has an abnormal echocardiogram (dexamethasone and plasmapharesis have been suggested
Singh, Ajay K. Lupus nephritis and anti-phospholipid activity syndrome in pregnancy. Kidney International. Vol 58. (2000), pp 2240-2254.
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.
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.