This is a 39-year-old woman who presented with headache, diffuse rash, myalgia, arthralgia, and weakness. Her exam found diffuse tender rashes, mild effusions, and elevated inflammatory markers. The working differential diagnosis includes systemic lupus erythematosus or another autoimmune connective tissue disease. Further workup of autoantibodies and imaging may help determine a diagnosis to guide treatment.

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