lupus nephritis in pregnancy

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    Lupus Nephritis in Pregnancy

    Journal case recordYovita Devi K

    030.08.261

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    CASE

    A 29-year-old pregnant woman withsystemic lupus erythematosus was admitted

    to the hospital because of renal failure at 20

    weeks 6 days of gestation.

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    HISTORY OF THE ILLNESS

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    21 years of age

    Pain and swelling developed in the distal andproximal interphalangeal joints of both hands and

    feet.

    Diagnosis : systemic lupus erythematosus.

    Therapy : Low-dose prednisone

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    Three years before admission

    the nephrotic syndrome developed. Renal biopsy : class IV lupus nephritis.

    Therapy : Prednisone (10 mg), azathioprine (25

    mg), furosemide (20 mg), and enalapril (15 mg)were administered daily.

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    Eighteen months before admission

    Renal biopsy : class IV lupus nephritis, diffuseproliferative type.

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    One year before admission

    The blood pressure was 85/50 mm Hg and theweight 50.3 kg.

    Therapy : patient declined the option of

    cyclophosphamide therapy because of a desire to

    preserve fertility. Azathioprine was discontinued,

    and mycophenolate mofetil was added to

    prednisone therapy.

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    Five months before admission

    The patient reported that during the precedingmonth she had had unprotected intercourse.

    The blood pressure was 92/48 mm Hg and the

    pulse more than the 80 beats per minute.

    Therapy : mycophenolate mofetil was discontinued

    and enalapril was withheld temporarily.

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    Three months before admission

    The patient still had not menstruated, testing forquantitative serum levels of the beta subunit of

    human chorionic gonadotropin was positive at

    162,000 U per liter.

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    Two months before admission

    The patient was seen in the high-risk obstetricalclinic for prenatal care, at 11 weeks 6 days of

    gestation.

    The vital signs and physical examination were

    normal.

    Therapy : supplementary prenatal vitamins, folic

    acid, and calcium were begun, and prednisone was

    continued at 10 mg a day.

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    Six weeks before admission

    The patients weight was 53.5 kg. Two weeks later, the weight was 60.3 kg, and 3+

    pedal edema was noted.

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    Three weeks before admission

    The blood pressure was 132/74 mm Hg, the pulse 88 beatsper minute, and the weight 62.1 kg.

    There was mild pallor, no icterus, and no jugulovenous

    distention. The results of an examination of the heart and

    lungs were normal The uterine fundus was compatible with a gestational age of

    five months. There was 2+ pitting edema of the ankles and

    mild sacral edema.

    Treatment non-pharmacology : patient was advised to wearantiembolic stockings and to restrict her intake of fluids to

    1500 ml per day.

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    The day of admission Patient had fatigue and weakness.

    She had no allergies. She did not smoke cigarettes or drink alcohol. There was

    no family history of systemic lupus erythematosus or kidney disease.

    Physical examination : patients skin was pale.

    The temperature was normal, the blood pressure 110/60 mm Hg, the pulse 96 beats

    per minute, and the weight 62.1 kg.

    the lungs revealed no abnormalities, and a 2/6 systolic ejection murmur was heard at

    the upper left sternal border.

    The abdomen was soft, nontender, and gravid, with a fundal height consistent with the

    gestational age of 20 weeks. There was 3+ edema extending to the thighs. An

    ultrasonogram of the uterus obtained on the second hospital day revealed a normal

    fetus and normal volume of amniotic fluid.

    Treatment non-pharmacology :

    bed rest, Fluids were restricted to 1500 ml per day, and her vital signs were

    monitored.

    The option of pregnancy termination was discussed and declined by the patient.

    Pharmacology : Methylprednisolone IV (250 mg per day) for three days, followed

    by prednisone (60 mg per day). Epoetin alfa was begun, 3000 IU three times per

    week.

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    On the third hospital day, the blood pressure rangedfrom 107/59 to 138/78 mm Hg.

    On the seventh hospital day, the weight was 64.1 kgand the blood pressure 140/80 mm Hg. Anultrasonogram ofthe patients kidneys revealed noevidence of renal-vein thrombosis.

    On the eighth hospital day, the blood pressure ranged

    from 120/60 to 150/70 mm Hg. Therapy : subcutaneous heparin, 5000 IU twice per day, was

    begun.

    On the ninth day, the blood pressure was 140/80 to

    140/90 mm Hg, and the weight 65.0 kg. Treatment : azathioprine was again added. Two units of

    packed red cells were transfused.

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    On the 14th hospital day, the patients weight was

    67.3 kg.

    Ultrasonography of the uterus again showed a normalfetus and normal volume of the amniotic fluid.

    The blood pressure rose transiently to 170/98 mm Hg,

    and the patient was dizzy, 30 minutes later the blood

    pressure was 140/80 and remained stable.

    On the 24th day, the blood pressure had risen to

    174/100 mm Hg.

    Therapy : betamethasone, 12 mg intramuscularly, was

    given daily for two days.

    On the 25th hospital day (24 weeks 2 days of

    gestation), a diagnostic procedure was performed.

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    Hematologic Laboratory Data

    Variable Before Admission Hospital Day

    33 mo 18 mo 1 yr 3 mo 2 mo 2 days 1 3 10 24 25

    Hb 13,5 11,2 9,8 8,8 8,1 7,1 7,7 7,3 6,9 8,0 7,9

    Ht 38,8 31,3 26,8 25,3 22,9 20,1 22,4 20,4 19,5 23,3 22,8

    Leuko 13.800 4.800 8.300 8.800 10.200 14.300 11.000 16.700 21.200 18.600 15.800

    Trombo 347jt 277jt 209jt 244jt 282jt 295jt 326jt 271jt 106jt 84jt

    Antiphos-

    pholipid ab

    Negative Negative

    Lupus

    anticoagulantNegative Negative

    Urinalysis

    -Occult

    blood

    - albumin

    - red cells

    - white

    cells

    -Bacteria

    -Hyaline

    casts

    -Granular

    casts

    -Waxy cast

    Negative

    2+

    3-5

    -

    +2

    +3

    0-2

    10-20

    Few

    +3

    +3

    10-20

    50-100

    moderate

    10-20

    0-2

    +2

    +3

    20-50

    20-50

    Few

    3-5

    3-5

    +3

    +3

    >100

    >100

    Many

    >29

    3-5

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    Urine Chemical Value

    Variable Before admission Hospital

    day

    38 mo 33 mo 18 mo 1 yr 4 mo 2 mo 6 wk 1 wk 10

    Urine

    creatinine

    0,59 0,84 1,61 1,13 0,21 0,82 1,24

    Urine

    protein

    1660 1590 2740 2530 2390 4340 18.200

    Protein tocreatinine

    ratio

    1,70 2,24 11,38 14,68

    Albumin 712,8

    Albumin to

    creatinine

    ratio

    8692,7

    24-h urinespecimen-Protein

    -Creatinine

    -volume

    6700 2656

    944

    1600

    2465

    1302

    1550

    8289

    1910

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    Immunologic Laboratory Data

    Variable Normal

    range

    Before admission Hospital day

    33 mo 1 yr 5 mo 2 mo 10

    Antinuclear

    antibody titer

    Negative 1:640

    Speckled

    >1:5120

    Speckled

    1:640

    Speckled

    >1:5120

    Speckled

    >1:2560

    Speckled

    Anto-double-

    stranded DNA

    antibody titer

    Negative 1:640 1:160 1:80 1:40

    Anti Ro SS-A

    antibody

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    Blood Chemical Value

    Variable Before Admission Hospital Day

    33 mo 18 mo 1 yr 2 mo 2 days 1 3 10 23 24

    Urea

    nitrogen

    23 18 24 18 18 25 36 34 39 44

    Creatinine 0.8 0.8 0.8 0.7 1.5 1.4 1.8 1.8 2.3 2.0

    Protein-Total

    -Albumin

    -Globulin

    3.1 2.9

    7.0

    3.0 1.8 1.1

    4.7

    1.2

    4.1 3.7

    1.1

    2.6

    3.7

    1.1

    2.6

    Lactate

    dehydrogen-

    ase

    154 399

    Cholesterol

    -HDL-LDL

    -TG

    283

    45179

    295 562

    339

    60

    490

    325

    51

    845

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    Differential Diagnosis

    Pregnancy-induced flare of systemic lupuserythematosus.

    Preeclampsia, with the nephrotic syndrome,

    thrombocytopenia, and hemolytic anemia.

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    Clinical Diagnosis

    Pregnancy-induced flare of systemic lupuserythematosus.

    Preeclampsia.

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    Discussion of Management

    received azathioprine and corticosteroids but notcyclophosphamide.

    This patient underwent cesarean delivery because

    the fetus was in the breech presentation.

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    Pathological Discussion

    The renal biopsy performed 18 months beforeadmission, showed findings that were diagnostic of

    glomerulonephritis related to systemic lupus

    erythematosus

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    v

    the findings on renal biopsy satisfy the criteria for both membranous lupus

    nephritis (class V) and diffuse segmental proliferative and sclerosing (class

    IV-S [A/C]) disease.

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    v

    These findings a small placenta, decidual vasculopathy with or without

    acute atherosis, sheets of intermediate trophoblast cells, and hypermature

    villi as well as acute and chronic infarcts and a small-diameter umbilical

    cord.

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    Because the placental findings in pregnancies affected by

    preeclampsia and lupus are similar, it is difficult to

    distinguish between the two on the basis of placental

    pathology alone.

    A cardinal pathological feature of preeclampsia is that the

    spiral arteries of the mother do not undergo the normal

    spectrum of changes necessary for adequate oxygen and

    nutrient delivery to the placenta and fetus.

    To explain the systemic findings of proteinuria,

    hypertension, and coagulation abnormalities in the mother, it

    has been hypothesized that the ischemic placenta secretes

    one or more soluble factors into the maternal bloodstreamthat induce generalized endothelial dysfunction and the

    syndrome of preeclampsia.

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    Anatomical Diagnosis

    Combined membranous (class V) and diffusesegmental proliferative and sclerosing (class IV-S

    [A/C]) lupus nephritis.

    Small, hypermature placenta with severe decidual

    vasculopathy and acute atherosis, consistent with

    preeclampsia

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