Case Report

SYSTEMIC LUPUS ERYTHEMATOSUS

ANDALAS UNIVERSITY

By:

dr. Aswin Boy Pratama, SpOG

Trainee of Fetomaternal Subspeciality Education
Program

Mentor:
Prof. Dr. dr. Hj. Yusrawati, SpOG, Subs-KFM
(K)
Dr. Dr. dr. Joserizal Serudji SpOG, Subs-KFM
(K)

FETOMATERNAL SUBSPECIALITY EDUCATION PROGRAM

OBSTETRICS AND GYNECOLOGY
MEDICAL FACULTY OF ANDALAS UNIVERSITY
2024
 PROGRAM STUDI SUBSPESISALIS OBSTETRI DAN
GINEKOLOGI PEMINATAN KEDOKTERAN FETOMATERNAL
FAKULTAS KEDOKTERAN UNIVERSITAS ANDALAS
RSUP Dr. M. DJAMIL PADANG

LEMBAR PENGESAHAN

Nama : dr. Aswin Boy Pratama, SpOG

Semester : I (Satu)

Telah menyelesaikan kasus Kehamilan

dengan Penyulit SLE

Padang, Maret 2024

Pembimbing Peserta Pendidikan Subspesialis Obgyn

Peminatan Kedokteran Fetomaternal

Dr.Dr.dr.Joserizal Serudji SpOG(K) dr. Aswin Boy Pratama,SpOG

MENGETAHUI

KPS SUBSPESIALIS OBGYN

PEMINATAN KEDOKTERAN
FETOMATERNAL FK UNAND

Prof.Dr.dr.Hj.Yusrawati,SpOG(K)
 CHAPTER 1
INRODUCTION

Case: Mrs. Fitri Anita 38 years old 01169323, G 4P3A0H3 29-30 weeks gestational
age previous CS 1x with systemic lupus erythematosus (SLE) control to
fetomaternal. She was diagnosed SLE by internal medicine division of
rheumatologist in Tertiary Hospital..

Systemic lupus erythematosus (SLE) is a chronic, multisystem, inflammatory

autoimmune disease characterized by relapses (commonly called “flares”) and
remission. Many organs may be involved and although the manifestations are
highly variable, the kidneys, joints, and skin are commonly affected. Immunologic
abnormalities, including the production of antinuclear antibodies (ANA), are also
characteristic of the disease. The pathophysiology of SLE is complex and
incompletely understood. The condition involves breakdown in the tolerance of
both T and B cells to self-antigens, and abnormalities in immunologic processes
involving both innate and adaptive immunity.
SLE is a chronic, multisystem disease that carries significant risk of adverse
maternal, fetal, and obstetrical outcomes. Patients with SLE should be cared for
by clinicians with expertise in managing the condition, and multidisciplinary care
may be required. Medical therapy requires adjustment in pregnancy to avoid
medications associated with potential untoward fetal effects. Surveillance for
evidence of flare and for maternal or fetal complications is an important part of
prenatal care. Ideally, people should be counseled before pregnancy to optimize
timing of pregnancy and pregnancy outcomes. Appropriate and effective
contraception is an essential part of comprehensive care for patients with SLE.
 CHAPTER II
LITERATURE REVIEW

2.1 Introduction
Systemic lupus erythematosus (SLE) is a chronic, multisystem, inflammatory
autoimmune disease characterized by relapses (commonly called “flares”) and
remission. Many organs may be involved and although the manifestations are
highly variable, the kidneys, joints, and skin are commonly affected. Immunologic
abnormalities, including the production of antinuclear antibodies (ANA), are also
characteristic of the disease. The prevalence of SLE is estimated to be
approximately 28 to 150 per 100,000 individuals. 1 SLE is several times more
prevalent in females than males,2 and because it often affects young adults,
pregnancy is common among affected individuals. In the United States, there are
approximately 3300 deliveries per year in people with SLE. 3,4 Optimal care of a
pregnant patient with SLE involves consultation and co-management with a
rheumatologist.
The pathophysiology of SLE is complex and incompletely understood.
The condition involves breakdown in the tolerance of both T and B cells to self-
antigens, and abnormalities in immunologic processes involving both innate and
adaptive immunity.5 Anticardiolipin (aCL) antibodies are detected in 40% of
patients with SLE, although the development of antiphospholipid syndrome (APS)
is less common.6 SLE seems to be a disorder with some underlying genetic
component, an observation based in part on twin studies. In addition, 15% of
patients with SLE have a first-degree relative with the condition. 7,8 Although
numerous genes have been implicated, the genetics of SLE are
complex.9 Environmental and hormonal factors also play a role in the disease
process, and increased levels of estrogen have been implicated. 10

2.2 What are the diagnostic criteria for systemic lupus erythematosus?
SLE is a syndrome, and the diagnosis requires the presence of characteristic
clinical features and confirmatory laboratory studies. Major organs affected
include the kidneys, brain, lungs, heart, skin, and joints, and the most common
symptoms of SLE are fatigue, fever, arthralgias, myalgias, weight loss, and
rash.11 When a new diagnosis of SLE is suspected in pregnancy, many symptoms
of SLE are difficult to distinguish from normal pregnancy complaints.
The broad range of clinical manifestations and lack of pathognomonic
features or laboratory tests make the diagnosis of SLE challenging. Currently, the
diagnosis is often made on the basis of classification criteria developed by the
European Alliance of Associations for Rheumatology (EULAR)/American
College of Rheumatology (ACR)12 or the Systemic Lupus International
Collaborating Clinics (SLICC).13 These criteria were developed for research
purposes but are often used as diagnostic criteria for clinical management. The
sensitivity of the SLICC criteria for making the diagnosis of SLE is 97% vs 96%
12, 13
for the ACR criteria, and the specificity of SLICC is 84% vs 93% for ACR.
The mainstay of laboratory testing for diagnosis of SLE is the assessment of
ANA. Although useful in diagnosis, a positive ANA test result is not specific for
SLE. In contrast, antibodies against double-stranded DNA (anti-dsDNA) are
relatively specific for SLE.14, 15
In addition, complement levels, erythrocyte sedimentation rate and/or c-
reactive protein levels, and urine protein-to-creatinine ratio, although not
diagnostic, can be useful in supporting the diagnosis. Antibodies against
ribonuclear proteins, such as anti-Sjögren’s-syndrome-related antigen A (SSA)
(anti-Ro) and anti-Sjögren’s-syndrome-related antigen B (SSB) (anti-La), are
present in a minority of patients with SLE. These antibodies are associated with
neonatal lupus erythematosus (NLE) and are important in assessing fetal and
neonatal risks.16 Antiphospholipid (aPL) antibodies, including lupus
anticoagulants (LAC), aCL antibodies, and anti-beta-2-glycoprotein-I (anti-β2
GPI) antibodies can be used to establish the diagnosis of APS. 17 In addition to
diagnostic testing, some tests are useful to follow disease activity. Decreases in
complement activation (C3 and C4) and elevations in double-stranded DNA levels
are useful as markers and may indicate a flare.
2.3 What maternal complications are associated with systemic lupus
erythematosus during pregnancy?
Maternal morbidity and mortality are substantially increased in patients with SLE,
and an initial diagnosis of SLE during pregnancy is associated with increased
morbidity.18 Common complications of SLE include nephritis, hematologic
complications such as thrombocytopenia, and a variety of neurologic
abnormalities. Some patients with SLE also have APS, which is associated with
an increased risk of pregnancy loss and thrombosis. 19 In a large study in the
United States including >16 million pregnancies, patients with SLE had a several-
fold increased risk of thrombosis, thrombocytopenia, infection, multiorgan
disease, and need for blood transfusion when compared with those without SLE.
A 20-fold increase in maternal mortality was also reported.4
Pregnancy poses a theoretical risk for disease flares because of increased
levels of estrogen, which are linked to an increased risk of SLE. In addition, stress
and the effect of physical demands of pregnancy can increase the risk of flares.
However, it is not clear that the risk of flares is increased during pregnancy, and
several well-designed studies have yielded conflicting results. 20 Most flares during
pregnancy are mild, typically consisting of arthritis and cutaneous manifestations,
and easily treatable.21, 22 Fifteen percent to 30% of flares are severe, 23, 24 and some
can be life-threatening. Flares may occur during any trimester and in the
postpartum period.25 Risk factors for a flare occurring during pregnancy include
active disease within the 6 months before pregnancy, severe underlying disease,
active nephritis, and discontinuation of hydroxychloroquine (HCQ).23, 11

2.4 Severe systemic lupus erythematosus flares

In patients presenting with symptoms suggesting a more severe flare, the clinical
and laboratory evaluation described above is also recommended. Laboratory
testing for preeclampsia, including urine protein–creatinine ratio or 24-hour urine
assessment for protein and creatinine, uric acid, and liver function tests may also
be useful because the signs and symptoms of SLE flares and preeclampsia overlap
and are important to distinguish given that management differs. Glucocorticoid
dosage should be increased to 1.0 to 1.5 mg/kg, then tapered after clinical
improvement. If necessary, cyclosporine or azathioprine can be added to avoid
ongoing high doses of glucocorticoids. Hospitalization is generally appropriate in
this setting.
Rheumatology consultation is recommended for patients with a severe flare,
especially with renal or CNS involvement, for which intravenous glucocorticoids
are recommended and other immunosuppressive agents may be required.
 CHAPTER III
DISCUSSION

3.1 What is the appropriate obstetrical management for pregnant patients

with systemic lupus erythematosus?
Prepregnancy counseling
We recommend that patients with SLE undergo prepregnancy counseling with
both maternal–fetal medicine and rheumatology specialists that includes a
discussion regarding maternal and fetal risks. Patients should be informed that
even successful pregnancies are often complicated by preeclampsia, FGR, and
preterm birth. In addition, some patients will experience flares during pregnancy,
and medical options are more limited than in nonpregnant individuals. If delaying
pregnancy is advisable to optimize medical therapy or improve disease control,
appropriate contraception should be discussed and encouraged.
Prepregnancy counseling also allows for clinical and laboratory
assessment of disease status and maternal and fetal risks, and adjustment of
maternal therapeutic regimens. Factors that affect counseling include the patient’s
SLE history, specifically the presence or absence of lupus nephritis, CNS
involvement, thromboembolism, and APS; the patient’s obstetrical history,
particularly any history of neonatal lupus in previous children; and disease status.
Laboratory information can be useful in clarifying risks for a future pregnancy,
but laboratory results in an asymptomatic patient are not likely to be useful if
baseline values are already known. Testing for anti-SSA and anti-SSB antibodies
in patients without a previous infant with NLE is controversial. Results may
facilitate counseling, but the positive predictive value of testing for NLE in this
population is low. In addition, it is unclear whether CHB can be prevented with
antenatal management.
When possible, pregnancy should be deferred until the disease has been in
remission for at least 6 months. r ecommend that pregnancy be generally
discouraged in patients with severe maternal risk, including patients with active
nephritis; severe pulmonary, cardiac, renal, or neurologic disease; recent stroke; or
pulmonary hypertension. Patients who become pregnant with these conditions,
among others, may need abortion care because of life-threatening maternal risk.
The patient’s medical regimen may require modifications such as
discontinuing NSAIDs and full-dose aspirin and minimizing corticosteroid
dosages.19 HCQ should be continued because it is safe in pregnancy and abrupt
cessation may induce a flare. Immunosuppressive agents with the potential for
adverse or teratogenic fetal effects,
including cyclophosphamide, methotrexate, mycophenolate, and leflunomide,
should be discontinued before attempting pregnancy. 20 Azathioprine and
cyclosporine are acceptable immunosuppressive agents during
pregnancy.20 Patients requiring anticoagulant therapy should be switched from
warfarin to low-molecular-weight heparin before or as soon as pregnancy is
recognized.

Pregnancy management
Baseline laboratory testing early in pregnancy is similar to that advised for
preconception counseling. Although some experts recommend serial assessment
of autoantibodies, complement levels, complete blood count, and serum
chemistry,20 the utility of this testing remains unproven. Testing is recommended
if signs or symptoms suggest the possibility of a flare because clinical and
laboratory evidence of a flare can be used to adjust treatment.
Consultation with a rheumatologist and maternal–fetal medicine
subspecialist throughout pregnancy is recommended, especially for patients with
active and severe disease. Those with severe disease or a history of obstetrical
complications may benefit from more frequent evaluation. Use of a standardized,
validated tool, such as the Systemic Lupus Erythematosus Activity Index
(SLEDAI), has been reported to be helpful for assessing disease activity in the
general population and during pregnancy . Although there is no evidence to
support an optimal approach, weekly antenatal fetal surveillance may be
considered by 32 weeks of gestation, and ultrasonography to assess interval
growth is commonly performed monthly or at least at 28 and 32 to 34 weeks of
gestation. For pregnant patients with complicated SLE (eg, active lupus nephritis,
recent lupus flare, aPL antibodies with previous fetal loss, anti-SSA or anti-SSB
antibodies, or thrombosis), the gestational age at initiation of and frequency of
antenatal fetal surveillance should be individualized in consultation with
maternal–fetal medicine and may be considered on diagnosis or at a gestational
age when delivery would be undertaken for abnormal testing.
Timing, mode, and management of delivery in pregnant patients with complicated
SLE should be individualized. With uncomplicated SLE, early term delivery is
not indicated but delivery can be considered at term (39 weeks of gestation).
Complications such as preeclampsia or FGR, or comorbidities such as APS,
chronic hypertension, renal disease, or active SLE may modify delivery timing
and management, and may necessitate earlier delivery. For patients who required
prolonged use of corticosteroids during the pregnancy, stress dose steroids are
indicated for cesarean delivery but should be individualized for vaginal delivery.

Postpartum management
As with other autoimmune diseases, the incidence of relapse or flare of SLE
symptoms is increased in the postpartum period.
Prophylactic changes in medications are not recommended, but patients should be
informed of the potential for worsening symptoms and evaluated more frequently
as needed. NSAIDs can be used postpartum for mild joint pain. Patients who
require lifelong anticoagulation can be transitioned back to warfarin after delivery.
Those that do not require lifelong anticoagulation are generally continued on low-
molecular-weight heparin for 6 weeks after delivery.
Breastfeeding should be encouraged, with consideration of each person’s
medications. NSAIDS, HCQ, and corticosteroids are considered compatible with
breastfeeding by the American Academy of Pediatrics.130 There are limited data
regarding safety of lactation with many other medications used for SLE, and
decisions to breastfeed while taking these medications are often shared between
the patient and their clinician. Ongoing follow-up with a rheumatologist should be
encouraged. An appropriate, acceptable, and effective contraception method
should be recommended.
Contraception
Given the considerable maternal and fetal risks of pregnancy, the use of
appropriate contraception in patients with SLE is paramount. In particular, people
taking potentially teratogenic medications should avoid pregnancy. It has been
reported that many patients with SLE at risk for pregnancy do not use effective
contraception.
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