Challenges in Management of Systemic Lupus Erythematosus

on Pregnancy: A Single Case Report

Maya Oktari Yolanda

, Kristo A. Nababan

Department of Dermatology & Venereology,

Faculty of Medicine, Universitas Sumatera Utara Hospital,

Adam Malik Hospital, Medan

*Corresponding author

Keyword: Systemic lupus erythematosus; disease activity; pregnancy

Abstract: Introduction: Systemic Lupus Erythematosus (SLE) is an autoimmune disease with unknown etiology.

Pregnancy can trigger a recurrence of the disease or aggravate symptoms and threaten the lives of the

mother and/or the baby whom she carries. Case: A pregnant woman who is 18 years old, complained

thicked crust on the erythematous plaques distributed in the malar region, nasal, supraorbital and auricular

sinistra et dextra region, discontinuous hair marks on the lateral side of the scalp, mild arthritis. Laboratory

test results are leukocytosis (leukocyte 15,000 cells/µl; neutrophil 7.9 cells/µ; lymphocyte 6.29 cells/µ;

monocyte 0.74 cells/µ), CRP 0.7 mg/dl; C3 50mg/dl, ANA Test 2.4; Anti-dsDNA 28. The result of kidney

function tests is in the normal range. The USG results of a fetus: pregnancy in the uterus (8-9 weeks), child

alive (31/01/2019). This patient has been diagnosed as SLE for three years but is well controlled with

medication. However, the recurrence occurred when she is pregnant. This patient was diagnosed as mild

SLE in pregnancy, and the recurrence was assessed using the Lupus Activity Index in Pregnancy (LAI-P)

scale, and the score was 0.43. Then she was given the treatment with methylprednisolone 4mg once daily,

ranitidine 150mg two times daily, paracetamol 500mg three times daily, folic acid 400mg once daily, B-

complex vitamin two times daily, desoximetasone 0.25% cream on the face, sunscreen, and also she was

suggested to do a pregnancy control. In addition, she was also given education about her diseases, to avoid

direct sun exposure, drug side effects, psychological problems, how to deal with stress and keep a healthy

diet and lifestyle. Conclusion: Pregnancy can trigger the recurrence of SLE or aggravate the symptom so

that it can increase maternal and fetal mortality and morbidity. The treatment needs a multidisciplinary

approach together with Internal medicine (Rheumatology) and Obstetrics department, close monitoring to

check if there is a change in clinical manifestation, vital signs, liver function test, kidney function test,

hematology test, and also monitoring fetal development.

1 INTRODUCTION

Systemic Lupus Erythematosus (SLE) is a chronic

autoimmune inflammatory disease with unknown

etiology and various clinical manifestations, disease

pathways, and prognosis. Genetic and hormonal

factors play a role in its pathophysiology. The

annual incidence of SLE in the United States reaches

5.1 per 100,000 population. The clinical

manifestations of SLE are extensive, including the

involvement of the skin and mucosa, joints, blood,

heart, lungs, kidneys, central nervous system (CNS)

and immune system. With frequent symptoms of

arthritis at 48.1%, malar rash 31.1%, nephropathy

27.9%, photosensitivity 22.9%, neurologic

involvement 19.4%, and fever 16.6% while clinical

manifestations that are rarely found are myositis

4.3%, discoid rash 7.8%, 4.8% hemolytic anemia,

and acute subcutaneous lesions 6.7%.

SLE mainly occurs in women of childbearing

age, and there is an acceleration regarding the role of

pregnancy which can aggravate the symptoms of

SLE by 2-3 times, and also appear between 35%-

70% of all pregnancies. This is linked to hormonal

changes that induce physiological and

immunological changes (Th2 in an autoimmune

response). The risk of flares increases dramatically if

the patient has active lupus for six months before

Yolanda, M. and Nababan, K.

Challenges in Management of Systemic Lupus Erythematosus on Pregnancy: A Single Case Report.

DOI: 10.5220/0009990404110415

In Proceedings of the 2nd International Conference on Tropical Medicine and Infectious Disease (ICTROMI 2019), pages 411-415

ISBN: 978-989-758-469-5

411

pregnancy are 7.5 times. The most common SLE

activity is mild to moderate form. The symptoms are

often a rash on the skin, arthritis, and hematological

changes.

Symptoms of flares can appear at any time or

several months after birth. Monitoring is needed

during pregnancy and postpartum. The impact of

SLE on pregnancy is abortion or fetal death.

Essential factors that play a role are flares, the

timing of flares and antiphospholipid syndrome

(APS). In addition, preterm and low birth weight

(LBW) can occur in 9.4% of cases due to thrombosis

in the placenta. The increase in preeclampsia

incidence occurred in the range of 13-35%.

Nephritis also increased the incidence of abortion,

36-52%, and preterm labor 35-40%. However,

physiological changes related to pregnancy can also

resemble those of SLE, such as fatigue, arthralgia,

hair loss, headache, facial and palm erythema,

anemia, thrombocytopenia. An assessment of SLE

activity in pregnancy can use a more specific scale

of the SLE Disease Activity Index (SLEDAI), as

well as Lupus Activity Index in Pregnancy (LAI-P)

scale.

Treatment of SLE in pregnancy, mild SLE

activity can use low-dose prednisone (<7.5mg/day),

nonsteroidal anti-inflammatory (NSAID) can be

used at the end of the first trimester, and second

trimester. Hydrocloroquin (HCQ) has a protective

effect, antithrombosis, ability to have a longer life,

and minimal side effects. SLE moderate activity can

be treated with high-dose or pulse-doses

corticosteroid, intravenous immunoglobulin to

control the kidney and hematology organ function.

Immunosuppressant agents such as cyclosporine,

tacrolimus, azathioprine are contraindicated.

Morbidity and mortality in SLE are still quite high,

with a survival rate of 5-10 years is 84-95%, and 10-

15 years is 70-85%.

2 CASE

An 18-year-old pregnant woman from Deli Serdang

came to the Department of Dermatology and

Venereology of the Adam Malik Hospital in Medan

on 19

March 2019, with the chief complaint is

thickened and painful malar rash from the facial

area, extended to cheeks, nose, and eyebrow since

two months ago. She also complained that her face

would feel hot and red when exposed to sunlight.

This is accompanied by hair loss, oral thrush, fever,

and weakness. The patient was referred to the

rheumatology department because of the pain in her

joints.

Three years ago, the patient complained about

the malar rash on her face when exposed to the

sunlight, and she also felt the burning sensation in

her face. The patient also experienced oral thrush,

hair loss, and painful leg joint until she was unable

to walk. In addition, patients feel quickly tired,

decreased appetite, and high fever. Then she went to

Melati Hospital in Pakam and was referred to the

Pirngadi Hospital. Patients were diagnosed as lupus.

The patient was hospitalized, and complaints have

improved. All this time, she is routinely controlled

in Melati Hospital and was administered

methylprednisolone in maintenance dose, ranitidine,

and sunscreen. In December 2018, the patient was

found to be pregnant, and they complained slightly

reappeared.

In physical examination, we found that the

patient appeared to be mildly ill. Vital signs within

normal limits. In dermatology examination, we

found an erythematous plaque in the malar region

with thickened crust, extended to nasal bridge,

supraorbital and auricular sinistra et dextra regions.

We also found a discontinuous hair mark on the

temporal region and erythematous linear striae in the

abdominal and antebrachial regions. Laboratory test

results are leukocytosis (leukocyte 15.000 cells/µl;

neutrophil 7.9 cells/µ; lymphocyte 6.29 cells/µ;

monocyte 0.74 cells/µ), ferritin levels 372,50 ng/ml,

CRP 0.7mg/dl; C3 50mg/dl, ANA Test 2.4; Anti-

dsDNA 28. Kidney function tests are in the normal

range. USG of the fetus results in pregnancy in the

uterus (8-9 weeks), child alive (31/01/2019).

The patient was diagnosed as a mild degree of

SLE in pregnancy and was given therapy

methylprednisolone 4mg once daily, ranitidine

150mg twice daily, paracetamol 500mg three times

daily, folic acid 400mg once daily, B-complex

vitamin two times daily, desoximetasone 0.25%

cream on the face, sunscreen, and also she was

suggested to do a pregnancy control. In addition, she

was also given education about her diseases, to

avoid direct sun exposure, drug side effects,

psychological problems, how to deal with stress and

keep a healthy diet and lifestyle.

ICTROMI 2019 - The 2nd International Conference on Tropical Medicine and Infectious Disease

412

Picture 1. Dermatology examination found (A) erythematous plaque in the malar region with thickened crust, extended to

nasal bridge, supraorbital and (B, C) auricular sinistra et dextra regions. We also found (C) a discontinuous hair mark on the

temporal region, and erythematous linear striae (D) in the abdominal and (E) antebrachial region.

3 DISCUSSION

Systemic Lupus Erythematosus (SLE) is a chronic

autoimmune inflammatory disease with unknown

etiology and various clinical manifestations, and

often did not recognize by medical personnel and

treatment is only in accordance with dominant

manifestation.

In our case, this patient was diagnosed with SLE

3 years ago, by using the criteria of the American

College of Rheumatology (ACR) with the discovery

of 4 or more criteria having a sensitivity of 85% and

a specificity of 95% in Pirngadi Hospital. She was

hospitalized in Pirngadi and had an improvement.

All this time, she was routinely controlled in Melati

Hospital and was administered methylprednisolone

Challenges in Management of Systemic Lupus Erythematosus on Pregnancy: A Single Case Report

413

in maintenance dose, ranitidine, and sunscreen. She

admits that there was no history of recurrence.

In December 2018, the patient was found to be

pregnant, and the complained slightly reappeared but

in the mild form. The current diagnosis is mild grade

SLE with skin lesion manifestations (malar rash),

mild arthritis, no life-threatening symptoms, and

stable organ function according to average results of

laboratory tests. She was assessed by LAI-P scale to

measure the incidence of flares caused by

pregnancy, comparing the previous symptoms and

the new symptoms. The value of this score is 0.43,

which can be concluded that this flare is caused by

pregnancy (with a minimum positive score LAI-P is

0.25).

Pregnancy does carry a separate problem for

SLE patients. Pregnancy not only increase the

incidence of recurrence or aggravate the symptoms

of SLE but also associated with a higher risk of

complications of the mother and her baby. A large

national database study of 16.7 million deliveries

reported many folds increased risk of maternal

death, pre-eclampsia, preterm labor, thrombosis,

infection, and hematologic complications during

SLE pregnancy. The biggest issue is the 3-5 times

higher risk of pre-eclampsia, complicating 16-30%

of SLE pregnancies. The predisposing factors for

pre-eclampsia include advanced maternal age,

previous personal or family history of pre-eclampsia,

pre-existing hypertension or diabetes mellitus, and

obesity. It is recommended at least six months of

controlled disease activity or in a state of total

remission. In lupus nephritis, the period is more

extended to 12 months in total remission. This can

reduce SLE recurrences during pregnancy. Our

patient claimed that she has been in controlled

activity disease in the last two years, but the

symptoms reappeared gradually since she was found

to be pregnant.

Patients were given methylprednisolone 4mg

once daily according to the Indonesian Rheumatic

Study Guide. Steroid exposure should be limited to a

minimum during the pregnancy because the high

doses are associated with an increased risk of

diabetes, hypertension, pre-eclampsia, and

premature rupture of membranes. However, in the

case of disease flares, short courses of high doses

and/or intravenous pulse methylprednisolone can be

used. Corticosteroid doses do not exceed 7.5 mg/day

of prednisone or equivalent, because 88% of

prednisone is deactivated by placental enzymes, and

<10% reaches fetal circulation. It is said that

methylprednisolone is safer than

beta/dexamethasone, but the risk of miscarriage is

increased by 21%. Our patient was also given

ranitidine to minimize gastrointestinal side effects of

methylprednisolone, paracetamol to relieve the pain,

folic acid to prevent the neural tube defect and

vitamin B-complex from fulfilling the patient’s need

of vitamin. Topical desoximetasone, 0.25% cream,

applied twice daily for her cutaneous lesions and

sunscreen to avoid the sunlight were also

administered.

Ante-natal management of pregnant patients with

SLE requires close monitoring together with Internal

medicine (Rheumatology) and Obstetrics

Department. The monitoring should be more

frequent and detailed than the usual standard of care.

Each visite should include thorough physical

examination, routine laboratory tests, and specific

investigations. Our patient was given the education

to do routine laboratory tests include kidney function

test, liver function test, serological tests, disease

activity (CRP, anti-ds-DNA, antibodies,

complement), and urine protein. Monitoring of fetus

should be done every month from 16-28 weeks,

every two weeks from 28-34 weeks, and every week

for the next gestational age. In addition, blood sugar

control with HbA1c and oral glucose tolerance test

are also needed. Education about the nature of the

diseases and avoiding the precipitating factors (sun

exposure, stress), drug side effects, psychological

problems, and maintaining a healthy lifestyle and

diet are also essential things

It is vital to monitor fetal development. The main

obstetric issues in SLE pregnancy are higher rates of

fetal loss, preterm birth, intrauterine growth

restriction, and neonatal lupus syndromes. However,

the rate of fetal loss has declined, and live births

rates of 80-90% have recently been reported. About

10-30% of SLE pregnancies are complicated with

fetal growth restriction and small for gestational age

babies. Active disease and lupus nephritis increase

the risk of fetal loss and other adverse outcomes.

Proteinuria, hypertension, thrombocytopenia, and

the presence of anti-phospholipid antibodies are

other negative predictors for fetal survival.

4 CONCLUSION

Here we report a case about SLE on pregnancy in an

18 years old woman. Pregnancy can trigger the

recurrence of SLE or aggravate the symptom and

relate to maternal and fetal mortality and morbidity.

The treatment needs a multidisciplinary approach;

maternal and fetal close monitoring is essential for

optimal outcomes.

ICTROMI 2019 - The 2nd International Conference on Tropical Medicine and Infectious Disease

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