Lupus in Pregnancy

1. Clinical
Conference
3/15/2023

2. Warm up: In these 11 U.S. states, abortion is now
illegal. Can you name some of them?
• Alabama
• Arkansas
• Idaho
• Kentucky
• Louisiana
• Mississippi
• Missouri
• Oklahoma
• South
Dakota
• Tennessee
• Texas

3. HPI
• G1P0 23 y.o. female with 9 weeks pregnant and history of HTN, recent dx of SLE with
prior manifestations of seizure 2/2 PRES, AIHA, acute PE, and lupus nephritis (class IV
+ V) who was transferred from Lahey to BMC for MFM and was consulted for concern
of lupus flare in the setting of not taking medications for a couple of months
• ROS: (+) lower abdominal pain, flank pain, nausea/vomiting, rashes (left earlobe x 2
weeks, hands x 1 month), peripheral edema; (-) seizure, alopecia, photosensitivity,
malar rash, oral ulcer, arthritis, serositis, easy bruising, blood clots, previous
miscarriage, hematuria or melena
• Other history:
• Social Hx: no alcohol, smoking, IVDU, office worker, married with her husband. Had copper IUD
but removed it last year, not on any contraception
• Surgical hx: none relevant
• Family Hx: none relevant
• Medical Hx: HTN, PRES
• Medications: None (has stopped taking every about 1-2 months ago, part of it was cost. Stated
she ran out of refills

4. Rheum Hx
8/3 –
8/10/2022
• South Shore Hospital with SOB and leg swelling. Renal biopsy with lupus nephritis class IV + V
and AIHA (+Coombs test for IgG). Started on pulse steroid x 3 days for AIHA. She was tapered to
prednisone 20 mg daily, followed by hematologist.
12/2022
• Lahey with DOE and pleuritic chest pain, found to have RLL acute PE so she was discharged with
warfarin. Also had headache and witnessed seizure 2/2 PRES (supported by brain MRI) during
that hospitalization. She was started on Plaquenil 400 mg daily and CellCept 500 mg BID
12/2022
– 3/2023
• Lost to follow up, no appointment with Rheumatology, only with neurology for PRES and PCP in
1/2023 (check HTN and INR for warfarin – no blood work done)
3/8/2023
• Presented to Lahey ED with n/v/abdominal pain. Had positive pregnancy test 1 week ago. LMP
was in January. AKI with Cr 6.6, and thrombocytopenia with Plts 40. US with 9 week IUP and
subchorionic hematoma => transfer to BMC for MFM service

5. Vital signs:
BP 158/97 (BP Cuff Location: Left Upper Arm, Patient Position: Sitting) | Pulse 87 | Temp 98.4 °F (36.9 °C) (Oral) |
Resp 18 | Ht 1.651 m (5' 5") | Wt 72.2 kg (159 lb 2.8 oz) | SpO2 98% | BMI 26.49 kg/m²
Discoid rash
Small ulcer on
upper palate

6. Prior labs
• ANA titer 1:320, nuclear homogeneous pattern
• Cryoglobulin normal
• APLs: Beta-2 glycoprotein antibodies IgM and IgG,
Anticardiolipin, Lupus anticoagulant normal
• dsDNA 659
• Smith > 8
• Smith/RNP > 8
• RNP 2.7
• SSA > 8, SSB negative
• Complement C4 18 (normal), Complement C3 77 (low)
• ANCA negative
• Haptoglobin 305 (elevated)
• LDH 297 (elevated)
• QuantiFERON gold indeterminate
UPCR: 777/61.5 =
12.6 g/g
cellular casts (not
RBC) and broad
hyaline and granular
casts

7. Current Labs
• C4 < 4, C3 28
• CRP 34.5, ESR 60
• LDH 354, normal haptoglobin
• D-dimer 9298
• Fibrinogen 424
• APLA: pending
• DsDNA pending
UPCR: 6759/1630
= 4.1 g/g

8. What’s your
management?
• In summary, G1P0 23 yo female 9 week
pregnant with PMHx of recent dx of SLE
with prior manifestations of seizure 2/2
PRES, AIHA, acute PE, and lupus
nephritis (class IV + V) who presented
with acute renal failure, anemia and
thrombocytopenia, discoid and
cutaneous vasculitis,
hypocomplementemia consistent with
severe multisystem SLE flare

9. Management of SLE during pregnancy
• Medications
• Complications
associated with
pregnancy
• Pre-conception
evaluation
• Contraception
counseling
• Lactation

10. Pregnancy planning
Dao KH, Bermas BL. Systemic Lupus Erythematosus Management in Pregnancy. Int J Womens Health. 2022;14:199-211.
Published 2022 Feb 15.

11. Who should not get pregnant in SLE?
Lateef et. al. Best Pract Res Clin Rheumatol. 2013;27(3):435-447

12. Contraception Counseling
• What percentage of SLE patient receives contraception
counseling?
• Yazdany J et al 2008 study at UCSF
• Among 206 women, 86 were at risk for unplanned pregnancy.
• Only 41% received contraceptive counseling in the last year
• 53% depended solely on barrier methods
• Intrauterine device contraceptives (IUDs) were used by 13%
• Women using potentially teratogenic medications were no more
likely to have received contraceptive counseling

13. ACR 2020 Guidelines – Contraception
Stable, Low Disease Activity
• Strongly recommend
• IUD
• Progestin implant
• Combined estrogen-progesterone pill
• Progestin-only pill
• Vaginal ring
• DMPA (uncles at increased risk for osteoporosis)
• Conditionally recommend because of lowest failure rates

14. ACR 2020 Guidelines – Contraception
Moderate, Severe Disease Activity
• Strongly recommend
• Progestin-only or IUD over combined estrogen-progestin contraception in
SLE patients, including nephritis
• Avoid estrogen-containing contraceptives (have not been studied in
this population of patients with SLE)
• Conditionally recommend against transdermal estrogen-progestin patch
• Use an IUD or 2 other methods of contraception together if taking
CellCept or Myfortic

15. ACR 2020 Guidelines – Contraception
APL positive
• Strongly recommend IUDs (levonorgestrel or copper) or the
progestin-only pill in women with positive aPL
• Avoid the following
• Strong recommendation against: combined estrogen-progestin
contraceptives => estrogen increases risk of clotting
• Do not recommend: DMPA due to concern regarding clotting risk
• No comment due to lack of data: progestin implant

16. ACR 2020 Guidelines – Emergency
Contraception
• Levonorgestrel
• OTC
• Readily available
• Safe to use in SLE patients including positive aPL

19. Management of SLE during pregnancy
• Medications
• Complications
during pregnancy
• Pre-conception
evaluation
• Contraception
counseling
• Lactation

20. Lupus Mimicker in Pregnancy
• 23 yo female 25 week pregnant with SLE presented with HTN,
thrombocytopenia, low complements, proteinuria, and acute
renal failure. Patient was started on pulse steroids x 3 days, but
did not improve?

21. Proteinuria, HTN, thrombocytopenia, renal failure = Pre-eclampsia vs. Lupus Nephritis
Preeclampsia =
proteinuria only
LN = Red/white/cellular
casts
Elevated liver enzymes
(HELLP)
Lateef et al 2013;27(3):435-447

22. Treatment of LN Class III, IV, V in pregnant patients
Hahn, Bevra H et al. Arthritis care & research vol. 64,6 (2012)

23. Neonatal Lupus
• Higher risk in patients with SSA (anti-Ro) and
SSB (anti-La) antibodies which can cross the
placenta
• Most features disappear with clearance of
maternal abs by 6-8 months of life
• Rash
• Elevated liver enzymes
• Blood count abnormalities
• Most serious are cardiac involvement
• 1st degree, 2nd degree, CHB*
• HCQ has been shown to reduce risk of cardiac
neonatal involvement

24. HCQ use in a mother with anti-SSA/Ro antibodies and
a previous child with cardiac-NL may reduce the risk
of cardiac-NL recurrence in a subsequent offspring

25. Differentiation of SLE flare from physiological pregnancy
changes
Lateef et al 2013;27(3):435-447

26. Back to our patient
• She was given pulse solumedrol 1g IV x 3 days on admission
• She did not want to abort the pregnancy
• Per MFM: pregnancy is contraindicated and recommended for
pregnancy termination.
• The pregnancy has passed the period of implantation (0-2 weeks after
fertilization) organogenesis (2-8 weeks) where teratogenesis, or fetal risk of
anomalies is of concern.
• No withholding any lifesaving interventions include DMARDs, biologics, or HD.
• Per renal: want a kidney bx, start HD
• Per heme: hold AC d/t thrombocytopenia and instability

27. Continue
• On day #3, she agreed to undergo
pregnancy termination with D&C

28. Future plan?
• Currently she does not want any contraception, wants to get
pregnant again
• Start on Benlysta infusion (difficulty obtain medication, non-
adherence, etc)
• Renal biopsy?

29. References
• Dao KH, Bermas BL. Systemic Lupus Erythematosus Management in
Pregnancy. Int J Womens Health. 2022;14:199-211. Published 2022 Feb 15.
doi:10.2147/IJWH.S282604
• Lateef A, Petri M. Managing lupus patients during pregnancy. Best Pract Res Clin
Rheumatol. 2013;27(3):435-447. doi:10.1016/j.berh.2013.07.005
• Sammaritano, Lisa R et al. “2020 American College of Rheumatology Guideline for
the Management of Reproductive Health in Rheumatic and Musculoskeletal
Diseases.” Arthritis care & research vol. 72,4 (2020): 461-488.
doi:10.1002/acr.24130
• Lateef A, Petri M. Managing lupus patients during pregnancy. Best Pract Res Clin
Rheumatol. 2013;27(3):435-447. doi:10.1016/j.berh.2013.07.005

31. HPI
• 39 yo female with no past medical hx who presented with headache,
diffuse rash, myalgia/arthralgia x 1 week. Rashes are itchy and tender to
the touch. Headache is worsening which prompted her to present to the
ED
• ROS: (+) weakness (cannot get out bed without assistance),
photosensitivity (blurry vision) and phonophobia, left ear and drainage
(since 9, occurred 2x since she moved here), 1 prior miscarriage in 1st
trimester (-) alopecia, oral ulcers, malar rash, sicca symptoms, serositis,
easy bruising or clot, seizure, Raynaud's, sclerodactyly
• SHx: no alcohol, smoking, IVDU. Married. Came to US from Nigeria in
2015, no recent travel except to Maryland about 2 months ago. Had a 2 yrs
old baby, was sick with RSV 2 months ago

32. Physical exam
• BP 142/97 | Pulse 83 | Temp 98.2 °F (36.8 °C) (Oral) | Resp 18 | SpO2 99% | BMI 25.26 kg/m²
Small effusion (L>R). TTP
Diffuse TTP with all fingers, hands,
wrists, elbows, shoulders, ankles,
and toes are without synovitis
General:
Fatigue-looking, cover her eyes with
eye masks, generalized weakness in
both upper and lower extremities

33. Labs
• CBC: WBC 15.2, Hgb 9.7, Plt 223
• CMP: Cr 0.65, LFTs wnl
• CK: 113
• ESR > 130, CRP 270
• INR 1.42
• LP: nucs 14, 62% poly, RBC 5,
glucose 79, protein 22, ME panel
negative
• Imaging:
• CXR: cardiomegaly, no PNA
• CT brain: no ICH, opacification of the
left middle ear, ?otomastoiditis

34. • In summary, patient is a 39 yo female with no significant past
medical history who presented with acute onset of headache,
diffuse rash, with myalgia/arthralgias and found to have
elevated inflammatory markers, proteinuria, ?middle ear
infection
• What is your DDx? Any other work up?

35. Hospital course
• Headache improving
• WBC trending down
• ESR/CRP trending down
• ANA negative
• ANCA-pending