A case report on newly diagnosed systemic lupus erythematosus in

pregnancy and intrauterine fetal demise

*
HarshineeN 1,Nivedhitha P2 , Vidhya R3 ,PavithraM 4

1,2,3,4
Department of Obstetrics and Gynaecology
SreeBalaji Medical College and Hospital , Chennai, India

EMAIL ID: harshinee.nk@gmail.com1

*
Harshinee N; Email id: harshinee.nk@gmail.com

ABSTRACT

Systemic lupus erythematosus (SLE)is an autoimmune disease known for its multisystem involvement
can pose significant challenges during pregnancy and significantly increase the risk of adverse
outcomes such as intrauterine fetal demise (IUFD), spontaneous abortions, preeclampsia and
eclampsia, intrauterine growth retardation (IUGR), preterm delivery, especially in primigravida. SLE
can complicate the pregnancy especially when it presents with manifestations such as lupus nephritis
and thrombocytopenia. Here we are about to discuss a case report of a 27 year old primigravidawith
SLE complicated with lupus nephritis, thrombocytopenia and preeclampsia who was found to have
intrauterine fetal demise at 32 weeks. The patient underwent induction of labour following which she
spontaneously expelled a dead fetus.This case underscores that early recognition and proactive
management of SLE in pregnancy is crucial to optimize maternal and fetal outcomes.
Multidisciplinary care is essential to mitigate adverse outcomes including IUFD.

Keywords : Intauterine fetal demise (IUFD), Lupus nephritis, Preeclampsia, Systemic lupus
erythematosus (SLE), Thrombocytopenia.

Introduction kidneys become inflamed, leading to potential

damage to the kidneys. It occurs in a
Systemic lupus erythematosus (SLE) is a
significant number of patients ranging from
chronic autoimmune disease characterized by
mild to severe forms that can result in renal
immune dysregulation and the production of
failure if not treated properly. Pregnant women
autoantibodies that can target various tissues
with SLE are more prone to complications
and organs throughout the body. SLE affects
such as fetal growth restriction, preeclampsia,
0.1% of the population worldwidewith striking
preterm birth, miscarriages and hematological
predilection for women in their reproductive
abnormalities.
years, often posing challenges during
pregnancy. SLE is diagnosed by the presence SLE is often referred to as the great imitator,
of autoantibodies, particularly Antinuclear which was highlighted in this challenging
antibodies(ANA) and antibodies to double case. The patient's initial presentation shared
stranded DNA (anti-dsDNA). Lupus nephritis signs and symptoms [1] of sepsis and other
is a specific manifestation of SLE where the diagnoses and pregnancy complications, which
caused a delay in the identification of SLE. challenging [9]. Nonetheless,
Delayed diagnosis increases the risk of SLE,preeclampsia, and HELLP syndrome can
maternal and fetal complications [2], but this be distinguished by laboratoryfindings of
case had successful maternal and fetal abnormal complement levels and inactive
outcomes. Currently, distinguishing SLE in urine sediment[10]. Clinical findings such as
pregnant women from other diseases is new-onset lymphadenopathy, arthritis,fever, or
difficult because of its shared presentation and rash may help in distinguishing the two
the limited literature reporting new-onset SLE processes [11]. In casesin which it still is
cases in pregnancy. The majority of the current unclear, renal biopsy may be obtained.
literature focuses on flare management and Obtaining adiagnosis is critical because
maternal and fetal outcomes. In non-pregnant management varies between the two
patients, the diagnosis of SLE is challenging diseasestates: while SLE may be treated
and often delayed for multiple reasons [3]. medically, preeclampsia often requiresdelivery
First, the onset of the disease often is not [12].SLE in pregnancy predisposes many
evident until many years due to its gradual adverse maternal and fetal
progression. Due to its limited and non-
outcomes. In 2008, maternal mortality in
specific features in its initial presentation, SLE
women with SLE and pregnancy was reported
often resembles other pathologic processes
to be 20-fold higher than in non-pregnant
such as infectious or hematological
women[13]. Fortunately, maternal mortality
abnormalities, which can lead patients to
has significantly improved. A studyby Mehta
specialists not trained in diagnosing or
et al. found that mortality rates decreased five-
managing SLE. In addition, SLE can mimic
fold in pregnantwomen with SLE in the United
many of the symptoms and pathological
States [14]. Although improvement isnoted,
processes of pregnancy [4], and SLE
adverse pregnancy complications occur at a
symptoms can overlap with physiological
higher rate in pregnant women with SLE,
changes of pregnancy such as fatigue, myalgia,
specifically preterm delivery, low-birth-weight
skin changes, and hair loss [5]. However, there
are some distinguishing clinical characteristics infants, and fetal loss [15]. The risk of
reported in the literature. New-onset SLE pregnancy loss increases morefor women with
appears to occur in the first and second new-onset SLE (62.4%) compared to those
trimesters [6]. When compared to non- with pre-existing disease (27.1%), with the
pregnant women with SLE, women with new- majority of cases occurring during thefirst and
onset SLE in pregnancy are less likely to have second trimesters (95.3%) [16].
clinical features such as fever, arthralgia, hair
loss, Raynaud's phenomenon, and oral Thus, a multidisciplinaryapproach to monitor
ulcerations [7]. Three retrospective analyses obstetric and neonatal complications is
from China showed that when compared to necessaryto manage maternal and fetal
women with pre-existing SLE, those with outcomes.
new-onset SLE had higher disease activity, The mainstay for treatment in pregnancy
haematological abnormalities (primarily consists of hydroxychloroquine and
thrombocytopenia and anaemia), renal disease azathioprine [17]. Hydroxychloroquine, an
activity, and pregnancy loss [8].However, antimalarial drug, is to be initiated or
hematological and renal abnormalities are also continued during pregnancybecause it
seen in severe hypertensive disorders of decreases the number of flares, risk of
pregnancy such as preeclampsia and congenital heart block,and neonatal lupus
haemolysis, elevated liver enzymes, and low without adverse fatal effects [18]. Azathioprine
platelets (HELLP) syndrome, which makes the isconsidered safe in pregnancy based on renal
diagnosis of newonset SLE in pregnancy very transplant studies. The doseshould be limited
to a maximum of two mg/kg/day, to avoid the Environmental factors can trigger or
risk offetalcytopenia and immune suppression exacerbate the disease. The pathophysiology
[19]. Other immunosuppressiveagents like of systemic lupus erythematosus (SLE) during
cyclophosphamide, methotrexate, and pregnancy involves interactions between
mycophenolatearecontraindicated during maternal immune response and placenta with
pregnancy and should be discontinued its effect on maternal and fetal health. In SLE
threemonths before conception [20]. immune mediated inflammation and vascular
Corticosteroid exposure should belimited to abnormalities can impair placental function
the least effective dose during pregnancy due leading to complications such as placental
to the risk ofmaternal diabetes, preeclampsia, insufficiency and IUGR . Growth restriction,
and hypertension. preterm birth, IUFD in severe cases are due to
placental insufficiency. During pregnancy,
Pathophysiology immunological and hormonal changes
In SLE, there is a dysregulated immune influence the course of SLE. For example,
response including the production of estrogen levels rise affecting B cell function
autoantibodies against self antigens like DNA, and potentially exacerbating autoimmune
phospholipids and histones (antinuclear responses. Progesterone levels also increase
antibodies, anti-dsDNA antibodies, anti-Sm and play a role in affecting the course of SLE.
antibodies and antiphospholipid antibodies), Pregnancy induces a shift towards a Th2
activation of complement pathways which can immune response (anti-inflammatory) to
lead to systemic inflammation and damage to tolerate the fetus, but in SLE, this is disrupted
the tissues. Genetic factors play an important but Th1 and Th17 responses (pro-
role in predisposing an individual to SLE. inflammatory) will be active, contributing to
Genes encoding for complement proteins, the disease. Women with SLEhave.
cytokines and components of apoptotic
pathway are implicated in immune regulation.

an increased risk of adverse pregnancy and absence of fetal heart sound on doppler.
outcomes, including pre-eclampsia, IUGR, USG confirmed intrauterine fetal demise with
preterm birth and fetal loss.These outcomes no detectable fetal cardiac activity. Patient was
are often related to underlying vascular and counselled about the diagnosis of IUFD.
immunological abnormalities in SLE that can Induction of labour was done following which
lead to placental insufficiency and impaired she spontaneously expelled a dead boy fetus of
fetal development. weight ~540 grams. Complete blood count
showed thrombocytopenia (platelets:87000).
Case report Peripheral smear with manual platelet count
A 27 year old primigravida who was on showed 94000 platelets with platelet
irregular antenatal followups with LMP on morphology report as seen in singles,
24/10/23 and gestational age of 32 occasional large platelets present. Urine
weeks+3days, a known case of gestational albumin report showed 3+ . Urine spot PCR
hypertension on treatment and severe IUGR was 0.4 . Serum albumin level was 2.9 g/dl .
came to casualty with complaints of decreased Renal function test report showed Urea level
fetal movements and lower abdomen pain as 54 mg/dl and creatinine level as 1.3 mg/dl.
since morning. On general examination, BP USG KUB showed features favouring the
was 130/80mmHg and there was bilateral possibility of Acute kidney injury (AKI). USG
pitting pedal edema+ (grade 1) and the patient abdomen and pelvis showed minimal bilateral
had oral ulcers. On obstetric examination, pleural effusion and minimal free fluid in
fundal height corresponded to 24-28 weeks pelvis. Anti Nuclear Antibodies (ANA)
assessed by indirect immunofluorescence was occurring after 20 weeks of gestation is one of
positive (+++ ) in 1:100 dilution. Complement the serious complications in pregnant women
levels were decreased (C3 = 24.8 mg/dl and with SLE. Major causes of IUFD in SLE are :
C4 = 4.0 mg/dl). The patient was diagnosed to 1)Lupus Nephritis resulting in impaired renal
be SLE Positive.Serial platelet monitoring was function which affects the placental perfusion
done daily which showed decreasing pattern. and fetal growth 2) Systemic inflammation in
Serial RFT monitoring showed deranged urea SLE flare ups can affect the placental function
and creatinine levels.Postpartum the patient 3) Antiphospholipid syndrome (APS)
was referred to a rheumatologist and associated with SLE increases the risk of
nephrologist for initiation of corticosteroid thrombosis including placental thrombosis
therapy, immunosuppressive therapy and resulting in fetal growth restriction and IUFD
further management. Patient’s lupus condition 4) Pre-eclampsia 5)Thrombocytopenia
was closely monitored. The patient was
Impact of SLE in pregnancy:1) Maternal –
observed for a week postpartum in which the
Women with SLE are at high risks for
renal parameters and other deranged
thrombocytopenia, pre-eclampsia, eclampsia.
parameters came under normal limits. Patient
There is an increased incidence of
was counselled about the increased risk of
antiphospholipid syndrome (APS) which can
adverse pregnancy outcomes in future and the
result in thrombotic events. 2) Fetal –
significance of preconception planning and
Miscarriage, preterm birth, intrauterine growth
strict disease control.
restriction (IUGR) and intrauterine fetal
Discussion demise (IUFD) are common in pregnant
women with SLE. Neonatal lupus which
SLE in pregnancy is considered a high risk
occurs due to the passage of maternal
case because of its potential to cause maternal
autoantibodies can cause congenital heart
and fetal complications. Intrauterine fetal
block and other complications.
death (IUFD) defined as the fetal death
Impact of pregnancy in SLE:Pregnancy can SLE which complicates pregnancy
cause SLE flare ups mainly during the second management and delivery.
and third trimesters and postpartum period. Patients with SLE should be counselled about
The hormonal changes in pregnancy, that is stable disease control and disease remission
increased levels of estrogen and progesterone before they plan for conception to minimize
in particular can exacerbate the disease and the adverse maternal and fetal outcomes.
worsen the symptoms. 1)Lupus Nephritis – Regular prenatal and antenatal visits,
Pregnant women with SLE are at increased laboratory tests including renal function test
risk for the development or exacerbations of and disease activity markers and fetal
lupus nephritis. It can cause proteinuria, monitoring are essential for the management
hypertension and impaired renal function. of complications early. A multidisciplinary
2)Cardiovascular system – Cardiovascular team of rheumatologists, obstetricians,
complications during pregnancy including nephrologists and hematologists is essential
hypertension, pre eclampsia and thrombosis for the management of SLE in pregnant
are highly common in SLE. 3)Hematological women. Vaginal delivery is preferred but
abnormalities in SLE such as anemia, cesarean sections may be required if there are
leucopenia and thrombocytopenia are obstetric or medical indications. Adjustments
exacerbated during pregnancy making the in medications should be made when a patient
patients more prone for bleeding, thrombosis with SLE plans for conception.
and infections. Thrombocytopenia is
Conclusion
particularly more common in pregnant women
This case emphasize the significance of early pregnancy in central chine: a retrospective
diagnosis, appropriate treatment and regular study of 68 pregnancies, Clin. Rheumatol.
monitoring in pregnant women with SLE to 2121–2131.
mitigate the adverse maternal and fetal [6] C. Zhang, M.Y. Liang, X. Xu, X.W. Zhang,
outcomes. The occurrence of IUFD S. Chen,2018 Clinical features of new-onset
underscores the importance of vigilant fetal systemic lupus erythematous in pregnant
monitoring in lupus pregnancies. patients, J. Obstet. Gynaecol. Res234–240.
Multidisciplinary team of obstetricians, [7] M.E.B. Clowse, M. Jamison, E. Myers,
rheumatologist and nephrologists is needed to A.H. James,2008, A national study of the
optimize the outcome in such pregnancies. complications of lupus in pregnancy, Am. J.
Preconception counselling and disease Obstet. Gynecol. 127.
stabilization plays a significant role in the [8] B. Mehta, Y. Luo, J. Xu, L. Sammaritano,
management of pregnant women with SLE. J. Salmon, 2019., Trends in maternal and fetal
outcomes among pregnant women with
ACKNOWLEDGEMENTS systemic lupus erythematosus in the United
States: a cross-sectional analysis, Ann. Intern.
We are grateful to all those who volunteered to
Med. 164–171.
participate in this study. We would like to
thank our patient and colleague in treating this [9] M. Petri, 2020, Pregnancy and systemic
case. lupus erythematosus, Best Pract. Res. Clin.
Obstet. Gynaecol. 24–30.
FUNDING [10]. Andreoli L, Bertsias GK, Agmon-Levin
No funding sources. N, 2017, EULAR recommendations for
women’s health and the management of family
CONFLICT OF INTEREST planning, assisted reproduction, pregnancy and
No conflict of interest noted. menopause in patients with systemic lupus
erythematosus and/or antiphospholipid
REFERENCE syndrome. Ann Rheum 476- 485.
[1] K.B. Dao, B.L. Bermas, 2022, Systemic 11. Kim M, Rostas S, Gabardi
lupus erythematosus management in S.2013,Mycophenolatefetal toxicity and risk
pregnancy, Int. J. Women's Health 199–211. evaluation and mitigation strategies. Am J
[2] R. Aljohani, D.D. Gladman, J. Su, M.B. Transplant. 1383-1389.
Urowitz, 2013, Comparison of systemic lupus 12. Cromer BA, Bonny AE, Stager M, 2008,
erythematosus (SLE) patients managed early Bone mineral density in adolescent females
after diagnosis in specialty versus community using injectable or oral contraceptives: a 24-
care clinic, Clin. Rheumatol. 1773–1778. month prospective study. FertilSteril. 2060-
[3] N.N. Kapsala, D.S. Nikolopoulos, S.P. 2067.
Flouda, A.P. Chavatza, D.D. Tseronis, 2022,
From first symptoms to diagnosis of systemic 13. Beerthuizen RJ.1996, Pelvic inflammatory
lupus erythematosus: mapping the journey of disease in intrauterine device users. Eur J
patients in an observational study, Clin. Exp. ContraceptReprod Health Care.237-243.
Rheumatol. 1–8. 14. Keenan L, Kerr T, Duane M, Van Gundy
[4] S. Shakya, Z. Yang, 2016, New-onset K.2018,Systematic review of hormonal
systemic lupus erythematosus during contraception and risk of venous thrombosis.
pregnancy: a challenge in diagnosis, J. Reprod. Linacre Q.,470-477.
Health Med. 63–66. 15. Andrade RM, McGwin G Jr, Alarcon GS,
[5] X. He, D. Jiang, Z. Wang, Y. Li, J. Wang, et 2006, LUMINA study group. Predictors of
al.2021, Clinical features of new-onset post-partum damage accrual in systemic lupus
systemic lupus erythematosus during erythematosus: data from LUMINA, a
multiethnic US cohort (XXXVIII).
Rheumatology (Oxford).;1380- 1384.
16. Dao KH, Bermas BL.2022, Systemic lupus
erythematosus management in pregnancy. Int J
Womens Health.199-211.
17. Tedeschi SK, Massarotti E, Guan H, Fine
A, Bermas BL, Costenbader KH., 2015,
Specific systemic lupus erythematosus disease
manifestations in the six months prior to
conception are associated with similar disease
manifestations during pregnancy. Lupus. 1283-
1292.
18. ImbasciatiE, Tincani A, Gregorini G, 2009,
Pregnancy in women with pre-existing lupus
nephritis: predictors of fetal and maternal
outcome. Nephrol Dial Transplant. 519- 525.
19. Cabiddu G, Castellino S, Gernone G, 2016,
A best practice position statement on
pregnancy in chronic kidney disease: the
Italian Study Group on Kidney and Pregnancy.
J Nephrol. 277-303.
20. Clowse MEB, Eudy AM, Balevic S, 2022 .
Hydroxychloroquine in the pregnancies of
women with lupus: a meta-analysis of
individual participant data. Lupus Sci
Med.651-659.