1

CHAPTER I
INTRODUCTION

Systemic lupus erythematosus (SLE) is the prototypic multisystem

autoimmune disorder with a broad spectrum of clinical presentations
encompassing almost all organs and tissues. The extreme heterogeneity of the
disease has led some investigators to propose that SLE represents a syndrome
rather than a single disease.1
SLE is more common in Native Americans, African Americans, and Asians. 2
Pediatric SLE (pSLE) represents approximately 15-20% of all SLE patients. 3 The
incidence of SLE in children is 10-20 cases per 100.000 children. This disease is
rarely occurs in children before 5 years old. 4 About 15-20% of lupus patients,
develop their first symptoms before 18 years of age. It is slightly more common in
girls, with the sex ratio being about 4:3 before puberty; however after puberty, the
sex difference increases to about 4:1.2 SLE in woman, generally occur after
puberty and before menopause. SLE often occur in families with a history of SLE
or others autoimmune disease.4
Systemic lupus erythematosus is a chronic, recurrent, potentially fatal
multisystem inflammatory disorder that can be difficult to diagnose. The disease
has no single diagnostic marker; instead, it is identified through a combination of
clinical and laboratory criteria. Accurate diagnosis of systemic lupus
erythematosus is important because treatment can reduce morbidity and mortality,
particularly from lupus nephritis.5
SLE in pediatric is a more aggressive disease than adult SLE, having a
substantially higher prevalence and severity of nephritis and CNS disease,
requiring higher doses and more sustained need for corticosteroids and other
immunosuppressive medications. Children, especially adolescents, experience a
more negative impact on their physical and psychosocial development. Additional
issues to be considered include the side effects of corticosteroids such as
osteoporosis, growth retardation and poor compliance with drugs.2
 2

In the last decade the outcome of SLE patients have improved remarkably,
but even though many diagnostic and treatment options are similar for adults and
children there are special issues that need to be considered in children and
adolescents with SLE.3 For example, the long term psychological impact of having
a lifelong illness, along with the significant side effects of therapy need to be
addressed, in order to have a smooth transition through adolescence into
adulthood. SLE in pediatric is a challenging disease, both to diagnose and treat. It
is a more severe disease, as compared to adult SLE, having significantly more
renal and CNS involvement.2 Threfore, this article reviews for the diagnosis of
systemic lupus erythematosus by primary care physicians.
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CHAPTER II
THEORY

2.1 Definition
Systemic lupus erythematosus (SLE) is an autoimmune disease with the
formation of antinuclear antibodies (ANA), especially against double-stranded
DNA (anti-dsDNA).4 SLE is the multisystem autoimmune disorder with a broad
spectrum of clinical presentations encompassing almost all organs and tissues. 1
According to Indonesian rheumatologist, SLE is a systemic autoimmune disease
characterized by the presence of autoantibodies against the autoantigen, immune
complex, and dysregulation of the immune system, causing damage of several
organs.6

2.2 Epidemiology
The incidence of SLE in the general population varies according to the
characteristics of the population studied, i.e. age, gender, race, ethnic/national
origin or period of time studied, but also depending on changes in classification
criteria.7 SLE in children represents approximately 15-20% of all SLE patients. It
is more common in females than in males, with a female to male ratio varying
from 3:1 to 9:1. In Caucasian females the incidence of SLE with onset before age
of 19 years is between 6 and 18.9 cases per 100.000, while it is 20-30 per 100.000
in African American females and 16-36.7 per 100.000 in Puerto Rican females. 3
On average, 60% of patients develop pSLE after age 10, 35% between 5 and 10
years, and only 5% before age 5. In studies from Asia, the mean ages at diagnosis
were reported to be 8.6 to 13.5 years. 6 The diagnosis of SLE in children is rare
before the age of 10, and the average age at presentation is 12.1 years. Disease
damage and mortality in pSLE have been linked to different risk factors which
include young age at diagnosis, male sex, and non-white ethnicity (African
American, Asian, and Hispanic).3

2.3 Etiology and Pathogenesis

 4

Although the etiology of SLE remains unknown, but several factors

predisposition may play a role in the pathogenesis of this disease include
hormonal, genetic, and environmental. These factors lead to an irreversible break
in immunological tolerance manifested by immune responses against endogenous
antigens.1,3
Abnormal oestrogen metabolism has been demonstrated in patients with
SLE of both sexes, with an increase in 16 hydroxylation of oestrone, resulting in
significantly raised 16 hydroxyestrone concentrations. The 16 metabolites are
more potent and feminising oestrogens. Women with SLE also have low plasma
androgens, including testosterone, dihydrotestosterone, dehydroepiandrosterone
(DHEA), and dehydroepiandrosterone sulfate. This abnormality might be
explained by increased testosterone oxidation at C-1721 or increased tissue
aromatase activity. The concentrations of androgens correlate inversely with
disease activity. Low concentrations of plasma testosterone and raised luteinising
hormone (LH) values have been found in some men with SLE. Thus, excessive
oestrogenic but inadequate androgenic hormonal activity in both men and women
with SLE might be responsible for the alteration of the immune responses.8
Patients with homozygous deficiencies of the early components of
complement (C1q, C1r, C1s, C4 and C2) are at risk of developing SLE or a lupus-
like disease. Patients deficient in one of the C1-complex or C4 molecule exhibit
the strongest prevalence (> 80 %) and the most severe disease, while strength of
association and disease severity decrease in C2-deficient patients. 3,8 The genes of
the major histocompatibility complex (MHC) have been most extensively studied
for their contribution to human SLE. Population studies reveal that the
susceptibility to SLE involves human leucocyte antigen (HLA) class II gene
polymorphisms.8
Environmental factors that trigger SLE include ultraviolet (UV) light,
infectious viruses, and demethylating drugs. UV light is the most obvious
environmental factor that may exacerbate SLE.1 UV light, especially UVB, is an
important trigger in many patients with SLE. UV light, can cause inflammation,
induce cellular apoptosis, and cause tissue damage. There is good evidence that
 5

exposure of skin to UV light alters the location and/or chemistry of DNA, in

addition to Ro and nRNP antigens, and probably enhances their immunogenicity.
Recent studies have demonstrated that UV light induces the apoptosis of human
keratinocytes, leading to the formation of clusters or blebs on the surface of dying
cells, which contain both nuclear and cytoplasmic antigens. 8 Infectious agents
such as viruses might theoretically initiate or cause a flare in SLE. 1 Viral
infections, including parvovirus B19 and cytomegalovirus (CMV), are common in
patients with SLE. Although chronic viral infection can lead to T cell exhaustion,
viruses have also been implicated in contributing to autoimmunity through
molecular mimicry. Some viral proteins are similar to selfantigens and therefore
illicit specific immune responses that can crossreact with self-antigens. For
instance, the EBV protein EBNA-1 crossreacts with the self-antigen Ro, a
common target of autoantibodies. Molecular mimicry is also observed with
bacterial and parasitic epitopes. Many drugs such as procainamide and
hydralazine, which are aromatic amines or hydrazines, can induce a lupus-like
syndrome in healthy individuals, especially in individuals who are genetically
slow acetylators.8,9 Although the pathogenesis of DIL is not well understood, a
genetic predisposition may play a role in the case of certain drugs. These drugs
may alter gene expression in CD4+ T cells by inhibiting DNA methylation and
induce over-expression of LFA-1 antigen, thus promoting autoreactivity.1

2.4 Diagnosis
The diagnosis of SLE is based on clinical findings, laboratory test results
including inflammatory markers, complement levels, markers of organ
involvement, and specific autoantibodies.10 Clinical characteristics and organ
involvement (Table 1) vary depending on age of onset, gender and race. In general
children with SLE tend to have a more severe disease at onset with higher rates of
organ involvement and a more aggressive clinical course than adult-onset SLE
patients.3

Table 1. Clinical Features of Systemic Lupus Erythematosus5

 6

Affected Organ System Signs and symptoms

Constitutional Fatigue, fever (in the absence of infection), weight loss
Skin Butterfly rash, photosensitivity rash, mucous membrane lesion,
alopecia, Raynauds phenomenon, purpura, urticaria, vasculitis
Musculoskeletal Arthritis, arthralgia, myositis
Renal Hematuria, proteinuria, cellular casts, nephrotic syndrome
Hematologic Anemia, thrombocytopenia, leukopenia
Reticuloendothelial Lymphadenopathy, splenomegaly, hepatomegaly
Neuropsychiatric Psychosis, seizures, organic brain syndrome, transverse
myelitis, cranial neuropathies, peripheral neuropathies
Gastrointestinal Nausea, vomiting, abdominal pain
Cardiac Pericarditis, endocarditis, myocarditis
Pulmonary Pleurisy, pulmonary hypertension, pulmonary parenchymal
disease

The skin is commonly involved in SLE. Different cutaneous manifestations

have been reported in children during the course of their disease including: malar
rash which is a hallmark of SLE, oral ulcers, vasculitic rash, photosensitivity,
alopecia, discoid lesions and Raynauds phenomenon.3 Mucocutaneous
involvement includes the typical butterfly or malar rash, an erythematous rash
in the malar distribution sparing the nasolabial folds (Figure 1). Diffuse hair loss
is commonly seen in children with active disease. In addition, children with lupus
can present with a photosensitive rash, exacerbated in sun-exposed areas including
upper arms, neckline, and face; Raynauds phenomenon of fingers and toes; a
vasculitic skin rash, which is often raised and painful affecting the fingers and
toes; and oral and/or nasal ulcers. Oral ulcers are typically located on the hard
palate and are painless (Figure 2). Uncommon skin manifestations include discoid
lupus lesions, which heal with scarring.10
 7

Figure 1. Malar rash of a 14-year-old patient with pediatric sys-

temic lupus erythematosus. The diseases typical butterfly or
malar rash is an erythematous rash in the malar distribution,
which includes the cheeks and crosses the nasal bridge but spares
the nasolabial folds.10

Figure 2. Oral ulcers in a 16-year-old girl with active pediatric

systemic lupus erythematosus. Characteristic oral ulcers and
hyperemia in children with lupus can be found on the hard palate.
These ulcers are typically painless.10

Musculoskeletal manifestations Arthritis occurs in more than 3/4 of

pediatric patients with SLE. Characteristically, it is polyarticular, usually presents
as a symmetric non-erosive, very painful polyarthritis involving both, large and
small joints and is rarely associated with radiographic changes, episodic and
 8

flitting in nature. In general, SLE arthritis responds well to conventional therapy.

Myalgia and myositis also can be found infrequently among SLE patient.
Musculoskeletal manifestations could be also seen as a side effect of the different
drugs used. Treatment-induced musculoskeletal complications include avascular
necrosis, osteoporosis and growth failure.3,7
Nephritis is the most common major organ manifestation and occurs in
more than 50% of children with pSLE, most commonly at diagnosis. Immune
complex formation/deposition in the kidney results in intra-glomerular
inflammation with recruitment of leucocytes and activation and proliferation of
resident renal cells.1 Children with lupus nephritis present with peripheral edema,
proteinuria, active urine sediment, and hypertension.10 Pathological findings can
not be predicted from the clinical manifestations and therefore a renal biopsy is
required for precise to establish diagnosis and subsequently planning effective
therapy.3
Thirty nine percent of children with SLE will develop hematological
abnormalities, one of the ACR diagnostic criteria for SLE, during the course of
the disease.3 Acute immune hemolytic anaemia with a sharp fall in hemoglobin
(>3 mg), spherocytes on peripheral smear, raised bilirubin and reticulocyte count
and a positive Coombs test confirm hemolytic process. Leucopenia and
lymphopenia correlate with disease flare.Thrombocytopenia may be due to
antiplatelet or antiphospholipid antibodies(APLA). Pancytopenia may be due to
drugs, infections or hemophagocytosis.During isolated hematological flare,
complement levels do not decrease and dsDNA titers are high.11
Neuropsychiatric disease affects about a quarter of children with SLE. 10
Unlike other manifestations of the disease, central nervous system (CNS)
involvement occurs within the first year of disease. Neuropsychiatric involvement
in SLE can range from global cerebral dysfunction with paralysis and seizures, to
mild or focal symptoms such as headache or memory loss. The diagnosis of
Neuropsychiatric involvement in SLE is difficult since there is a lack of specific
serologic studies for its diagnosis and monitoring. Although neuroimaging is a
 9

clinically useful tool, CSF analysis, EEG, CT scan, and even MRI may be normal
in these patients.3,10
Cardiac manifestations . It encompasses 4 major types of manifestations:
pericarditis (the most common form of cardiac involvement), myocarditis,
valvular disease, and coronary artery disease due to either coronary arteritis or
atherosclerosis.3 Cardiac manifestations are chest pain due to pericarditis or
coronary angitis. Myocarditis presents with unexplained tachycardia, arrhythmias
and cardiomegaly with or without cardiac failure. Doppler echocardiography may
demonstrate diastolic dysfunction or pericardial effusion.11
Pulmonary manifestations vary from sub-clinical abnormalities to life-
threatening disorders. The clinical spectrum includes pleuritis (the most common),
pneumonitis, pneumonia, pneumothorax, diffuse interstitial disease, pulmonary
hypertension and pulmonary hemorrhage, a relatively uncommon and potentially
lethal complication. In the majority of children, pulmonary symptoms (Table 2)
are present at some point during their disease course.3

Table 2. Pulmonary manifestations of SLE1

Pleuropulmonary
Frequency
Manifestation
Pleuritic chest pain or Common, with or without effusion or friction
pleurisy rub
Pleural effusion Exudate; unilateral or bilateral
Acute pneumonitis Not common; presentation includes: fever, non-
productive cough, infiltrates, hypoxia; high
mortality rates
Interstitial lung disease Insidious onset of dyspnoea on exertion, non-
productive cough, pleuritic chest pain

Pleuropulmonary
Manifestation
Bronchiolitis obliterans with
organising pneumonia
Frequency
Can be difficult to diagnose; requires biopsy;
responds to corticosteroids
 10

Pulmonary capillaritis or Rare, associated with antiphospholipid

diffuse alveolar haemorrhage
Shrinking lung syndrome
Pulmonary embolism or
infarction
Pulmonary hypertension
Lymphadenopathy
Infection
Malignant tumour
antibodies; poor prognosis
Occurs in patients with longstanding SLE;
possible cause: diaphragmatic weakness
Common in patients with antiphospholipid
antibodies
Insidious onset of dyspnoea on exertion,
chronic fatigue, weakness, palpitations, oedema
Massive mediastinal lymphadenopathy
uncommon in patients with SLE alone.
Cervical and auxiliary common, correlates with
disease activity
Typical and atypical pathogens. Due to immune
dysfunction and immunosuppressive
medications
Lung cancer; lymphoma more common

The heterogeneous nature of lupus can result in a diagnostic challenge for

physicians. Since there is not a single symptom or finding that in itself is
sufficient for making the diagnosis of SLE, the ACR (Table 3) has developed
different criteria that can be useful as general clinical guidelines for the initial
assessment of patients with suspected SLE. The guidelines combine 11 criteria
(clinical and laboratory) and a diagnosis can be made when four or more of these
criteria are present.3 The presence of 4 of 11 ACR classification criteria was found
to have a sensitivity of 96% and a specificity of 100%.10

Table 3. Revised ACR Criteria for the1

Criteria Definition
Malar rash Fixed erythema, flat or raised, over the malar eminences,
tending to spare the nasolabial folds
Discoid rash Erythematous raised patches with adherent keratotic scaling
and follicular plugging; atrophic scarring occurs in older
lesions
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Photosensitivity Skin rash as a result of unusual reaction to sunlight, by

patient history or physician observation
Oral ulcers Oral or nasopharyngeal ulceration, usually painless,
observed by a physician
Arthritis Non-erosive arthritis involving two or more peripheral
joints, characterised by tenderness, swelling or effusion
Serositis a. Pleuritis: convincing history of pleuritic pain or rub
heard by a physician or evidence of pleural eff usion or
b. Pericarditis: documented by ECG or rub or evidence of
pericardial eff usion
Renal disorder a. Persistent proteinuria >0.5 g per day or >3+ if
quantitation is not performed or
b. Cellular casts: may be red cell, haemoglobin, granular
tubular, or mixed
Neurological a. Seizures: in the absence of off ending drugs or known
disorder metabolic derangements (eg, uraemia, acidosis, or
electrolyte imbalance) or
Psychosis: in the absence of off ending drugs or known
metabolic derangements (eg, uraemia, acidosis, or
electrolyte imbalance)

Criteria Definition
Haematologic a. Haemolytic anaemia with reticulocytosis, or
b. Leucopenia: <4000/mm3, or
disorder
c. Lymphopenia: <1500/mm3, or
Thrombocytopenia: <100 000/mm3 in the absence of
offending drugs
Immunologic a. Anti-DNA: antibody to native DNA in abnormal titre,
disorder or
b. Anti-Sm: presence of antibody to Sm nuclear antigen,
or
c. Positive finding of antiphospholipid antibodies based
on:
 12

an abnormal serum concentration of IgG or IgM

anticardiolipin antibodies,
a positive test result for lupus anticoagulant using a
standard method, or a false positive serologic test
for syphilis known to be positive for at least 6
months and confirmed by Treponema pallidum
immobilisation or fl uorescent treponemal antibody
absorption test
Antinuclear An abnormal titre of antinuclear antibody by
antibody immunofluorescence or an equivalent assay at any point in
time and in the absence of drugs known to be associated
with drug-induced lupus syndrome

Figure 3. Algorithm for the diagnosis of systemic lupus erythematosus

(SLE). (ANA = antinuclear antibody; ACR = American College of
 13

Rheumatology; anti-dsDNA = antibody to doublestranded DNA antigen;

anti-Sm = antibody to Sm nuclear antigen).5

2.5 Risk Factor

1. Genetic
Various gene contributes in abnormal immune response that triggers
overproduced autoantibody. The study of the genome has identified a
group of genes that have correlation with SLE. (MHC Major
Histocompatibility Complex) class II especially HLA- DR2 (Human
Leukocyte Antigen-DR2), has been associated with the emergence of SLE.
In addition, the lack in the structure of components of the complement is
one highest risk factors that can cause SLE. Around 90% of people with
deficiency of C1q homozygote are at risk of suffering from SLE.12
2. Immunology factor

a. Antigen
Normally macrophages, form of APC (Antigen Presenting Cell) will
introduce antigens to the T cells. In SLE, few receptors that are located on
the surface of the T cells changes its structure and function until the
redirection of normal information could not be identified. This causes the
receptor on the surface of the T cells misinterpreted information from T
cell.
b. Intrinsic abnormalities of T cells and B cells.13
T cells are the B cells will unknownly activated into autoreactive cells,
also known as lymphocytes (receptor for autoantigen and stimulate
autoimmune response), which causes apoptosis of T and B cells to fail and
lead the abnormality in production of immunoglobulin.14
c. Disorders/Anomalies of antibodies
In SLE, as substrate antibodies were produced too much, idiotype known
as antigens. These trigger, overproduced autoantibodies by T lymphocytes
(T cells affect increased autoantibodies and complex immune easily
deposited in the tissues).13

3. Hormonal factors
 14

Elevated hormones also triggered SLE. Few studies found the correlation
between an increased risk of lupus and high level of estrogen. Other
studies also shown, the metabolism of estrogen the abnormal tissue can be
considered as a risk factor for SLE.

4. Environmental factors (plays a role in SLE)

a. The virus and bacterial infections

Viruses and bacteria, may have a role in the emergence of SLE. For
example, Epstein Barr Virus (EBV),Streptococcus and Clebsiella.
b. Exposure to UV ray
UV ray reduce the emphasis of the immune system, until the therapy
become less effective and also increase chance of recurrence or
complication. This causes the skin cells produce cytokines and
prostaglandin (local inflammation and systemic through the circulation of
blood vessels).15
c. Stress
Overstress triggered the SLE in patients who already have tendency of
SLE. The immune response will be interrupted when a person in stress.
Stress itself will not precipitate SLE on someone autoantibodinya system
no disruption since the beginning.

d. Drugs

Drug Induced Lupus Erythematosus (DILE).The classic medications that

induce drug-induced lupus (DIL) include minocycline, procainimide,
hydralazine, penicillamine, isoniazid, quinidine, phenytoin, and
carbamazapine. Antitumor necrosis factor agents, such as infliximab,
adalimumab, and etanercept have also been implicated in DIL. The
prevalence of DIL is equal in males and females, although minocycline-
induced lupus is usually seen in adolescent girls using the medication for
treatment of acne. Chronic use of the medication is required to develop
DIL.16

2.6 Therapy and Management

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1. Inpatient Treatment

Inpatient care in patients with systemic lupus erythematosus (SLE) is

required for severe hematologic, nephrologic, neurologic, or psychiatric
disease or for complications from these (eg, severe anemia, renal failure,
stroke, seizure), including the use of intravenous (IV) high-dose
corticosteroids or chemotherapy as required. Hospitalization may also be
required for severe hypertension. Inpatient care is appropriate for the
patient with unexplained fever to provide sepsis evaluation and treatment,
as well as to evaluate the patient for disease flare and to treat him or her
accordingly.21

2. Diet

Dietary restrictions are driven by the patients medical therapy. Most

patients require a course of corticosteroids and should be on a no-added-
salt, low-fat, c alcium-sufficient diet. Recognize that patients frequently
try nontraditional medical remedies and food supplements. These remedies
should be met with an open and supportive response. Monitoring
nontraditional remedies and food supplements is important, because they
may alter metabolism of more traditional medications, such as warfarin
sodium, or they may have a negative effect. Of note, L-canavanine in
alfalfa sprouts has been implicated in causing lupus, and excess use should
be avoided.

3. Medication
a) Antimalarial agents

Rash and other minor symptoms, including musculoskeletal symptoms,

can be treated with hydroxychloroquine 3-7 mg/kg/d, usually no more than
400 mg/d orally. Hydroxychloroquine may also decrease the risk of
thrombotic events. The long-term use of this medication requires
monitoring for retinal pigment changes every 6 months. Adverse effects
are infrequent and include eye changes, GI symptoms (of which diarrhea is
 16

most prominent), and CNS changes. Antimalarial drugs inhibit the

synthesis of DNA, ribonucleic acid (RNA), and proteins by interacting
with nucleic acids. Antimalarial drugs have various immunosuppressive
effects, can act as antioxidants, and interfere with prostaglandins.21

b) Corticosteroids

These agents elicit anti-inflammatory and immunosuppressive properties,

cause profound and varied metabolic effects, and modify the body's
immune response to diverse stimuli.

Treat children who have evidence of severe renal, CNS, or hematologic

disease with corticosteroids. The dose varies with the intensity of SLE on
the organ system involved and in select individuals with serologic disease
activity. Consider initiating therapy with daily prednisone (1 mg/kg/d) or
higher-dose alternate-day prednisone (5 mg/kg/d, not to exceed 150-250
mg, depending on the size of the patient). Alternatively, lower-dose daily
prednisone (0.5 mg/kg) may be used in conjunction with intermittent high-
dose IV methylprednisolone (30 mg/kg/dose, not to exceed 1 g) on a
weekly basis.18

Prednisone decreases inflammation by suppression of the immune system,

which it does by (1) decreasing lymphocyte volume and activity, (2)
decreasing polymorphonuclear (PMN) leukocyte migration, and (3)
decreasing or reversing capillary permeability. High doses, especially over
periods of more than 2-3 weeks, suppress adrenal function. Children who
are systemically ill with renal, neurologic, severe hematologic, cardiac, or
pulmonary disease are begun on high-dose daily prednisone 2 mg/kg/d
(not to exceed 80 mg/d) in divided doses, which are consolidated after
serologic disease activity is controlled and finally switched to alternate-
day prednisone.

Methylprednisolone decreases inflammation by suppression of the

immune system in much the same manner as prednisone, but it has less
 17

mineralocorticoid effects. Intravenous high-dose steroids can be given in a

hospital setting but also by home health teams. Intermittent high-dose IV
methylprednisolone is now known to extinguish the interferon signature of
systemic lupus for up to 7 days.18

c) Immunosuppressive agents

Evaluate children with signs of active nephritis to determine the WHO

classification category of their nephritis. Patients with class IV nephritis
and some patients with class III nephritis should be treated with
corticosteroids and cyclophosphamide. Mycophenolate mofetil has
become an alternative therapy for lupus nephritis. Azathioprine is used for
more mild nephritis. Consider cyclophosphamide for severe systemic
involvement of other vital organs, especially the brain. Other agents (eg,
mycophenolate mofetil, cyclosporine, methotrexate) are considered when
standard therapies have failed.

Cyclophosphamide, which is chemically related to nitrogen mustards,

interferes with the normal function of DNA by alkylation and cross-linking
the strands of DNA and by possible protein modification. As an alkylating
agent, the mechanism of action of active metabolites may involve cross-
linking of DNA, which may interfere with the growth of normal and
neoplastic cells.18

Mycophenolate inhibits inosine monophosphate dehydrogenase (IMPDH)

and suppresses de novo purine synthesis by lymphocytes, inhibiting their
proliferation. It inhibits antibody production by inhibiting T-cell and B-cell
proliferation, cytotoxic T-cell generation, and antibody secretion.

Methotrexate is an antimetabolite that binds to dihydrofolate reductase,

blocking the reduction of dihydrofolate to tetrahydrofolic acid; depletion
of tetrahydrofolic acid leads to depletion of DNA precursors and inhibition
of DNA and purine synthesis. This reduces the metabolism of activated T
and B cells, reducing the inflammatory response. Adjust the dose gradually
 18

to attain a satisfactory response. Consider SC route for patients who do not

respond to PO methotrexate or who have GI intolerance to PO dosing.18

d) Calcium and vitamin D supplements

All patients with systemic lupus erythematosus (SLE) who are on

corticosteroids or who have arthritis are at increased risk for osteopenia
and its complications. Diet and appropriate supplementation with vitamin
D and calcium are important tools for bone health in these patients. Of
note, natural production of vitamin D involves skin exposure to sun, which
is discouraged in the SLE population, increasing the risk of vitamin D
deficiency.

e) Nonsteroidal anti-inflammatory drugs (NSAIDs)

A child who presents with mild disease with no evidence of nephritis,

hypocomplementemia, and elevated antidouble-stranded DNA antibodies
is treated symptomatically and is monitored closely for signs of disease
progression. Arthritis is treated with NSAIDs. Select a specific agent based
on patient response to medication, history of previous drug allergy or
reaction, and ease of use.

Administer NSAIDs with caution in any patient with renal or liver disease
and avoid administering NSAIDs during pregnancy. NSAIDs have various
adverse effects that should be monitored, including gastritis, bone marrow
suppression, hepatitis, interstitial nephritis, and CNS changes.
Occasionally, a patient with systemic lupus erythematosus (SLE) has a
hypersensitivity reaction to NSAIDs; this is most often characterized by
hepatotoxicity, but the reaction can include other symptoms and must be
kept in mind.

2.7 Complications
 19

Children with lupus may have hematologic abnormalities, including hemolytic

anemia, thrombocytopenia, leukopenia, or lymphopenia. Patients with immune
complex disease in the kidneys may present with nephritis or nephrotic syndrome.
Numerous neurologic abnormalities, from psychosis and seizure to cognitive
disorders to peripheral neuropathies, may also occur. Their exact relationship to
the presence of immune complexes and autoantibodies remains unclear.17
As stated, corticosteroids are a mainstay of treatment for SLE. Unfortunately, a
number of adverse effects can affect the patient not only medically but from a
psychosocial standpoint as well. Once corticosteroids are started, patients can gain
weight easily, become hirsute, and develop acne, striae, and a cushingoid facies.
These effects tempt all patients, but particularly the adolescent girl, to abandon
adherence to their medical regimen. Infection, thinning of the skin, short stature,
personality changes, sleep disturbance, irregular menses, and mood swings may
also occur. Hypertension, glaucoma, and cataracts can develop and should be
screened for regularly. Patients may develop avascular necrosis of any bone but
this complication is particularly common in the femoral head. Any patient with
SLE on corticosteroids suffering from hip pain, with or without limited hip
motion, should have imaging studies done to rule out avascular necrosis.

2.8 Prognosis
Systemic lupus erythematosus (SLE) is a high-risk disease with the possibility of
end-organ damage to any vital or nonvital organ. This damage can severely affect
organ function and can lead to decreased quality of life. In addition, the treatment
of SLE is fraught with potential complications from the adverse effects of
steroids, infection from immunosuppression and poorly controlled disease, and
cardiovascular disease leading to early myocardial infarction.
Current mortality figures suggest that patients have a 95% rate of survival at 5
years. Some clinicians report a 98-100% survival rate at 5 years. These figures
depend on disease severity and compliance with therapy. Mortality rates rise over
time, with the major causes of death being infection, nephritis, central nervous
system (CNS) disease, pulmonary hemorrhage, and myocardial infarction. One
 20

indicator of morbidity and mortality risk is frequency of emergency department

visits.19
The 5-year survival rate for children with SLE is more than 90%. Most deaths in
children with SLE are the result of infection, nephritis, renal failure, neurologic
disease, or pulmonary hemorrhage. 20
The disease course is milder and survival rate higher in persons with isolated skin
and musculoskeletal involvement than in those with renal disease and CNS
disease. It is important to distinguish between the disease activity and the damage
index (irreversible organ dysfunction). Although the most effective instrument to
measure SLE disease activity is still open to debate, there are several validated
measures, including the Systemic Lupus Activity Measure (SLAM), SLEDAI,
Lupus Activity Index (LAI), European Consensus Lupus Activity Measurement
(ECLAM), and British Isles Lupus Activity Group (BILAG) Index.17

Prognostic factors from the 2008 European League Against Rheumatism

(EULAR) recommendations included the following [61] :

Clinical findings: Skin lesions, arthritis, serositis, neurologic

manifestations such as seizures and psychosis, and renal involvement

Diagnostic study results: Anemia, thrombocytopenia, leukopenia,

increased serum creatinine levels

Immunologic test results: Serum C3 and C4 concentration (which may be

low), as well as the presence of antidouble-stranded DNA (anti-dsDNA),
anti-Ro/ Sjgren syndrome A (SSA), anti-La/Sjgren syndrome B (SSB),
and antiphospholipid (aPL), and anti-ribonucleoprotein (anti-RNP).22

2.9 Patient Education

The patient and his or her family must have a thorough understanding of systemic
lupus erythematosus (SLE), its potential severity, and the complications of the
disease and its therapy.
 21

Treatment is difficult, especially for adolescent patients. The physician and

parents should expect issues, including depression and noncompliance, to arise.
The best method for deterrence is to thoroughly educate the patient and family
through discussion, support groups, and literature.5

Stress the importance of adherence to medications and follow-up appointments for

detection and control of SLE disease. Instruct patients with SLE to seek medical
care for evaluation of new symptoms, including fever. Advise them regarding
their heightened risks for infection and cardiovascular disease. Instruct patients
with SLE to avoid exposure to sunlight and ultraviolet light. Also, encourage them
to receive nonlive vaccines during stable periods of disease, to quit smoking, and
to carefully plan pregnancies.2

2.10 Prevention

Patients with SLE should be educated to avoid triggers for flare. Persons with
SLE should avoid ultraviolet light and sun exposure to minimize worsening of
symptoms from photosensitivity. Diet modification should be based on the disease
activity. A balanced diet is important, but patients with SLE and hyperlipidemia,
for example, should be placed on a low-fat diet. Many patients with SLE have low
levels of vitamin D because of less sun exposure; therefore, these patients should
take vitamin D supplements. Exercise is important in SLE patients to avoid rapid
muscle loss, bone demineralization, and fatigue. Smoking should also be avoided.2

Preventive measures are necessary to minimize the risks of steroid-induced

osteoporosis and accelerated atherosclerotic disease. The American College of
Rheumatology (ACR) Guidelines for the prevention of glucocorticoid-induced
osteoporosis suggest the use of traditional measures (eg, calcium, vitamin D) and
the consideration of prophylactic bisphosphonate therapy.2

The European League Against Rheumatism (EULAR) vaccination

recommendations for rheumatic diseases, including lupus, advocate baseline
assessment and delivery of nonlive vaccines during stable disease.
 22

[150] Particularly important is immunization against encapsulated organisms,

such as meningococcal vaccine, pneumococcal vaccine, and routine Haemophilus
influenzae childhood vaccination. Annual influenza vaccine is also encouraged.2

CHAPTER III
MEDICAL REPORT

3.1 Objective
The aim is to report a case of 13 years and 5 months old girl diagnosed with
systemic lupus erythematosus (SLE).

3.2 Case
RS, 13 years and 5 months years old girl with a 30 kg of BW and 135 cm of
BH presented with high fever was admitted to emergency department in Haji
Adam Malik General Hospital Medan on 27th January 2017 at 02.20 a.m.

3.3 History of Disease

 23

- Patient RS presented with high fever since 4 days ago.The fever included
with headache and dizziness. It also showed a decrement in temperature by
giving anti-pyretic medicine. The parents reported, she always had low fever
(relapsing fever) in this past 2 month and it goes away with administration
of anti-pyretic medicine.
- The history of cough,nause,vomitting,chills and night-sweating was not
found. The history of seizure related to fever was not found.
- Patient was complaining face rash that comes along with onset of fever 2
month ago. The rash was found around cheeks area and nasal bridge and
occasionally painful but not pruritic. It also came when she was exposed
(direct contact) to the sunlight. No medication was given according to the
parent.
- She complained her vision and skins were very sensitive towards the
sunlight until she had to wear a sunglasses and a cap for outdoor activities.
It also started few month ago.
- Patient also had a history of oral ulcers few month ago around buccal area,
tongue and lower lip. Her parent gave a herbal medication for the ulcers and
it goes away.
- Previously, December 2016, patient have been admitted in Adam Malik
hospital and was diagnosed with (SLE) by a paediatrician.
- The history of allergies and atopic was not found.

History of drugs : Paracetamol tablet

History of family : There was no history of the same disease in family.
History of parents medication : Unclear
History of Pregnancy : The age of mother was 25 years old during second
pregnancy. The gestation was 40 weeks.
History of Birth : Birth was assisted by a midwife. The patient was
born normally pervaginal and cried immediately
after birth. Body weight at birth was 3300 gram,
body length was 51 cm and the head circumference
was unclear.
 24

History of feeding : Patient was breastfeeding from birth to 4 months,

then given formulated milk from 4 months until 3
years old. Porridge strain from 6 months to 12
months and family meals since the age of 1 year
until now.
History of immunization : Not completed.
History of growth and development : The patients mother reported that RS
grew normally. RS had developed
talking, crawling, and walking skills on
time. Patient developed normal social
skills and history of mental illness was
not found.

Physical Examination:
Present : Level of consciousness: Compos Mentis (E=4 V=5 M=6)
BW: 30 kg, BH: 135 cm.
Vital Sign : BP: 110/70 mmHg, HR: 110 x/min, RR: 20 x/min, T: 39,2C
Localized Status :
Head : - Anemic (+/+), ikteric (-/-), dyspnea (-), cyanosis(-), edema (-)
- Old mans face (-)
- Eye : light reflex +/+, isochoric pupil, conjunctiva
palpebra inferior pale (-/-)
- Ear : normal
- Nose : normal
- Mouth : oral ulcers within lower lips
- Face : malar rash (cheeks and nose bridge area)

Neck : - Jugular vein pressure : (R+2) cmH2O

- Stiff neck : (-)
- Enlarged lymph nodes : (-)
 25

Thorax : - Symetrical fusiformis, retractions (-)

- RR: 20x/minute, regular, respiratory sound: vesicular, ronchi
(-/-), wheezing (-/-)
- HR: 110 x/minute, regular, murmur (-)

Abdomen : - Soepel
- Peristaltic (+) normal
- Hepar and Lien: unpalpable

Extremities : - Pulse: 110 x/minute, regular, tension/volume: enough

- Warm acral
- CRT < 3
- Edema pretibial (-), Plantar palmar et pedis pales (+/+)
- Blood pressure: 110/70 mmHg.

Laboratory Findings: 27th January 2017

Complete blood count:

Test Result Unit Referal

Hemoglobin 12.7 g% 11.7-15.5
Erythrocyte 4.49 106/mm3 4.20-4.87
Leucocyte 4.47 103/mm3 4.5-11.0
Thrombocyte 154 103/mm3 150-450
Hematocrite 38 % 38-44
Eosinophil 0.20 % 1-6
Basophil 0.20 % 0-1
Neutrophil 51.10 % 37-80
Lymphocyte 31.00 % 20-40
 26

Monocyte 17.50 % 2-8

Neutrophil 103/L
2.42 2.7-6.5
absolute
Lymphocyte 103/L
1.47 1.5-3.7
absolute
103/L
Monocyte absolute 0.83 0.2-0.4

103/L
Basophyl absolute 0.01 0-0.1

MCV 85 fL 85-95
MCH 28.3 Pg 28-32
MCHC 33.2 g% 33-35
Random
100 mg/dL <200
Blood Glucose
Electrolytes:
Sodium 129 mEq/L 135-155
Kalium 5.0 mEq/L 3.6-5.5
Chloride 98 mEq/L 96-106
Liver Function :
ALP 99 U/L 40-15-
AST/SGOT 680 U/L 5-34
ALT/SGPT 537 U/L 0-55

Kidney Function : 12
BUN 26 mg/dL 7-19
Ureum 0 mg/dL 15-40
Creantinin mg/dL 0.6-1.1
.61
Procalcitonine 0.41 Ng/mL

Differential Diagnosis : 1. Systemic Lupus Erithematosus + Hepatitis

2. Measles + Hepatitis
 27

3. Dengue Fever + Hepatitis

Working Diagnosis : Systemic Lupus Erithematosus + Hepatitis

Therapy : - Bed rest

- IVFD NaCl 0.9% 20cc/ hour (microdrips)
- Inj Paracetamol 30cc/6 hour

Work up : - Autoantibody Test

- Urinalysis
- Consult to gastroenterohepatology division

FOLLOW UP

27th January 2017 1st day

S O A P
Pale Sens : CM SLE + - Urdafalc 3x100 mg
- Methylprednisolon tab
(-), T : 39,2 Hepatitis
Hematuria 3-3-2
- Inj Paracetamol 30 cc/
(-)
Head : 6 hour
- Inj. Mehylprednisolon
- Eye reflect (+/+), isochoric
(30 mg/kg BB) 870
pupil
- Conjunctiva palpebral mg in 100 cc NaCl
inferior pale (-/-) 0,9% in an hour (for 3
- Mouth/nose/ear: normal
days) 1st day
Nect :
- Jugular Vein Pressure: R+2
cmH2O
- Enlarged lymph nodes (-/-)
 28

Thorax :
- Symetrical fusiform,
retraction (-)
- RR: 20 x/min, regular,
respiratory sound:
vesicular, ronchi (-/-)
- HR: 80 x/min, regular,
murmur (-)

Abdomen :
- Soepel
- Peristaltic (+) normal
- Hepar/Lien: unpalpable

Extremities :
- Pulse: 80 x/min, regular,
adequate
- Warm acral
- CRT < 3
- Pretibial edema (-/-)
- BP: 110/70 mmHg

28th January 2017 2nd day

S O A P
Pale (-), Sens : CM SLE + - Urdafalc 3x100 mg
Hematuria - Methylprednisolon tab
T : 36,7 Hepatitis
(-) 3-3-2
- Inj. Mehylprednisolon
Head : (30 mg/kg BB) 870
- Eye reflect (+/+), isochoric mg in 100 cc NaCl
pupil 0,9% in an hour (for
- Conjunctiva palpebral
3 days) 2nd day
inferior pale (-/-)
- Mouth/nose/ear: normal

Nect :
 29

- Jugular Vein Pressure: R+2

cmH2O
- Enlarged lymph nodes (-/-)

Thorax :
- Symetrical fusiform,
retraction (-)
- RR: 20 x/min, regular,
respiratory sound:
vesicular, ronchi (-/-)
- HR: 82 x/min, regular,
murmur (-)

Abdomen :
- Soepel
- Peristaltic (+) normal
- Hepar/Lien: unpalpable

Extremities :
- Pulse: 82 x/min, regular,
adequate
- Warm acral
- CRT < 3
- Pretibial edema (-/-)
- BP: 110/80 mmHg

29th January 2017 3rd day

S O A P
Pale (-), Sens : CM SLE + - Urdafalc 3x100 mg
Hematu - Methylprednisolon tab
T : 37 Hepatitis
ria (-) 3-3-2
- Inj. Mehylprednisolon
Head : (30 mg/kg BB) 870
- Eye reflect (+/+), isochoric mg in 100 cc NaCl
pupil 0,9% in an hour (for
- Conjunctiva palpebral
3 days) 3rd day
 30

inferior pale (-/-)

- Mouth/nose/ear: normal

Nect :
- Jugular Vein Pressure: R+2
cmH2O
- Enlarged lymph nodes (-/-)

Thorax :
- Symetrical fusiform,
retraction (-)
- RR: 20 x/min, regular,
respiratory sound:
vesicular, ronchi (-/-)
- HR: 90 x/min, regular,
murmur (-)

Abdomen :
- Soepel
- Peristaltic (+) normal
- Hepar/Lien: unpalpable

Extremities :
- Pulse: 90 x/min, regular,
adequate
- Warm acral
- CRT < 3
- Pretibial edema (-/-)
- BP: 110/70 mmHg

30th January 2017 4th day

S O A P
Pale (-), Sens : CM SLE + - Urdafalc 3x100 mg
Hematu - Methylprednisolon tab
T : 36,8 Hepatitis
ria (-) 3-3-2

Head :
 31

- Eye reflect (+/+), isochoric

pupil
- Conjunctiva palpebral
inferior pale (-/-)
- Mouth/nose/ear: normal

Nect :
- Jugular Vein Pressure: R+2
cmH2O
- Enlarged lymph nodes (-/-)

Thorax :
- Symerical fusiform,
retraction (-)
- RR: 20 x/min, regular,
respiratory sound:
vesicular, ronchi (-/-)
- HR: 95 x/min, regular,
murmur (-)

Abdomen :
- Soepel
- Peristaltic (+) normal
- Hepar/Lien: unpalpable

Extremities :
- Pulse: 95 x/min, regular,
adequate
- Warm acral
- CRT < 3
- Pretibial edema (-/-)
- BP: 110/70 mmHg

31st January 2017 5th day

S O A P
Pale Sens : CM SLE + - IVFD D5% NaCl
 32

(-), T : 37 Hepatitis 0,45% 20 gtt/i micro

Hematu - Urdafalc 3x100 mg
- Methylprednisolon tab
ria (-)
Head : 3-3-2
- Chemotheraphy
- Eye reflect (+/+), isochoric
cyclophospamide
pupil
postponed until result
- Conjunctiva palpebral
of SGPT at normal
inferior pale (-/-)
- Mouth/nose/ear: normal range for (3
times)
Nect : - R/: Repeat SGPT/
- Jugular Vein Pressure: R+2 SGOT on 2/2/2017
cmH2O
- Enlarged lymph nodes (-/-)

Thorax :
- Symetrical fusiform,
retraction (-)
- RR: 22 x/min, regular,
respiratory sound:
vesicular, ronchi (-/-)
- HR: 100 x/min, regular,
murmur (-)

Abdomen :
- Soepel
- Peristaltic (+) normal
- Hepar/Lien: unpalpable

Extremities :
- Pulse: 100 x/min, regular,
adequate
- Warm acral
- CRT < 3
- Pretibial edema (-/-)
- BP: 110/80 mmHg
 33

1st February 2017 6th day

S O A P
Pale Sens : CM SLE + - IVFD D5% NaCl
(-), T : 37 Hepatitis 0,45% 20 gtt/i micro
Hematuria - Urdafalc 3x100 mg
- Methylprednisolon tab
(-)
Head : 3-3-2
- Eye reflect (+/+), isochoric - CPA postponed
- R/: Repeat SGPT/
pupil
- Conjunctiva palpebral SGOT on 2/2/2017
inferior pale (-/-)
- Mouth/nose/ear: normal

Nect :
- Jugular Vein Pressure: R+2
cmH2O
- Enlarged lymph nodes (-/-)

Thorax :
- Symerical fusiform,
retraction (-)
- RR: 20 x/min, regular,
respiratory sound:
vesicular, ronchi (-/-)
- HR: 98 x/min, regular,
murmur (-)

Abdomen :
- Soepel
- Peristaltic (+) normal
- Hepar/Lien: unpalpable

Extremities :
- Pulse: 98 x/min, regular,
adequate
 34

- Warm acral
- CRT < 3
- Pretibial edema (-/-)
- BP: 110/80 mmHg

2nd February 2017 7th day

S O A P
Pale (-), Sens : CM SLE + - IVFD D5% NaCl
Hematu
T : 37 Hepatitis 0,45% 20 gtt/i micro
ria (-) - Urdafalc 3x100 mg
- Methylprednisolon tab
Head : 3-3-2
- Eye reflect (+/+), isochoric - CPA postponed
- R/: Repeat SGPT/
pupil
- Conjunctiva palpebral SGOT on 2/2/2017
inferior pale (-/-)
- Mouth/nose/ear: normal

Nect :
- Jugular Vein Pressure: R+2
cmH2O
- Enlarged lymph nodes (-/-)

Thorax :
- Symerical fusiform,
retraction (-)
- RR: 16 x/min, regular,
respiratory sound:
vesicular, ronchi (-/-)
- HR: 90 x/min, regular,
murmur (-)

Abdomen :
- Soepel
- Peristaltic (+) normal
- Hepar/Lien: unpalpable
 35

Extremities :
- Pulse: 90 x/min, regular,
adequate
- Warm acral
- CRT < 3
- Pretibial edema (-/-)
- BP: 110/80 mmHg

3rd February 2017 8th day

S O A P
Pale Sens : CM SLE + - IVFD D5% NaCl
(-), T : 36,8 Hepatitis 0,45% 20 gtt/i micro
Hematuria - Urdafalc 3x100 mg
- Methylprednisolon tab
(-)
Head : 3-3-2
- Eye reflect (+/+), isochoric - CPA postponed

pupil
- Conjunctiva palpebral
Lab:
inferior pale (-/-)
Liver
- Mouth/nose/ear: normal
- ALP/SGOT/SGPT:
Nect : 50/188/232
- Jugular Vein Pressure: R+2
Urinalisis:
cmH2O
- Enlarged lymph nodes (-/-) - Color:
kuning
Thorax :
jernih
- Symerical fusiform, - Glukosa
retraction (-) :-
- RR: 20 x/min, regular, - Billirubin
respiratory sound: :-
- Keton
vesicular, ronchi (-/-)
- HR: 90 x/min, regular, :
murmur (-) -
- Berat jenis
Abdomen : : 1.001
 36

- Soepel - PH :
- Peristaltic (+) normal
6,5
- Hepar/Lien: unpalpable
- Protein
:
Extremities :
-
- Pulse: 90 x/min, regular,
- Nitrit
adequate
:-
- Warm acral
- Leukosit
- CRT < 3
- Pretibial edema (-/-) :-
- BP: 110/80 mmHg - Darah
:
+

FCM
- Eritrosit
: 10,2
- Leukosit
: 7,1
- Epitel
: 1,6
- Cast
:0
- Kristal
:
0,1
- Bakteri
:
64,5
- Path Cast
:0

4th February 2017 9th day

 37

S O A P
Pale Sens : CM SLE + - IVFD D5% NaCl
(-), T : 37 Hepatitis 0,45% 20 gtt/i micro
Hematuria - Urdafalc 3x100 mg
- Methylprednisolon tab
(-)
Head : 3-3-2
- Chemotheraphy
- Eye reflect (+/+), isochoric
Cyclophosphamide
pupil
CPA 500 mg in 250
- Conjunctiva palpebral
cc NaCl 0,9% with
inferior pale (-/-)
- Mouth/nose/ear: normal manitol 35 cc in 500
cc NaCl 0,9% for 24
Nect :
jam (start: 14.30)
- Jugular Vein Pressure: R+2
cmH2O
- Enlarged lymph nodes (-/-)

Thorax :
- Symerical fusiform,
retraction (-)
- RR: 20 x/min, regular,
respiratory sound:
vesicular, ronchi (-/-)
- HR: 98 x/min, regular,
murmur (-)

Abdomen :
- Soepel
- Peristaltic (+) normal
- Hepar/Lien: unpalpable

Extremities :
- Pulse: 98 x/min, regular,
adequate
- Warm acral
- CRT < 3
 38

- Pretibial edema (-/-)

- BP: 110/80 mmHg

5th February 2017 10th day

S O A P
Pale Sens : CM SLE + - IVFD D5% NaCl
(-), T : 37 Hepatitis 0,45% 20 gtt/i
Hematuria
micro
(-) - Urdafalc 3x100 mg
Head : - Methylprednisolon
- Eye reflect (+/+), isochoric tab 3-3-2
- Continue
pupil
- Conjunctiva palpebral chemotheraphy
Cyclophosphamide
inferior pale (-/-)
- Mouth/nose/ear: normal CPA 500 mg in
250 cc NaCl 0,9%
Nect :
with manitol 35 cc
- Jugular Vein Pressure: R+2
in 500 cc NaCl
cmH2O
0,9% for 24 jam
- Enlarged lymph nodes (-/-)
(finish: 14.30)
Thorax :
- Symerical fusiform,
retraction (-)
- RR: 20 x/min, regular,
respiratory sound:
vesicular, ronchi (-/-)
- HR: 98 x/min, regular,
murmur (-)

Abdomen :
- Soepel
- Peristaltic (+) normal
- Hepar/Lien: unpalpable
 39

Extremities :
- Pulse: 98 x/min, regular,
adequate
- Warm acral
- CRT < 3
- Pretibial edema (-/-)
- BP: 110/80 mmHg

CHAPTER IV
DISCUSSION

December 2016. SLE in children represents approximately 15-20% of all

SLE patients. It is more common in females than in males, with ratio varying from
3:1 to 9:1.3 On average, 60% of patients develop SLE after age of 10, 35%
between 5 and 10 years, and only 5% before age 5. In studies from Asia, the mean
ages at diagnosis were reported to be 8.6 to 13.5 years.6
 40

Patient presented with high fever since 4 days ago included with headache
and dizziness. It also showed a decrement in temperature by giving anti-pyretic
medicine. Patient had a history of (relapsing fever) in this past 2 month. Patient
was complaining face rash that comes along with onset of fever 2 month ago. The
rash was found around cheeks area and nasal bridge and occasionally painful but
not pruritic. It also came when she was exposed (direct contact) to the sunlight.
She also complained of photosensitivity that started few month ago. Patient also
had a history of oral ulcers (painless) few month ago around buccal area, tongue
and lower lip.
The skin is commonly involved in SLE. Skin manifestations mainly have
been reported in children during the course of their disease including: malar rash
which is a hallmark of SLE and oral ulcers which is found in this patient. 3 The
typical butterfly or malar rash, an erythematous rash in the malar distribution
sparing the nasolabial folds. In addition, children with lupus can present with a
photosensitive rash, exacerbated in sun-exposed areas. Oral ulcers are typically
located on the hard palate and are painless.10
Since there is not a single symptom or finding that in itself is sufficient for
making the diagnosis of SLE, the ACR (Table 3) has developed different criteria
that can be useful as general clinical guidelines for the initial assessment of
patients with suspected SLE. The guidelines combine 11 criteria (clinical and
laboratory) and a diagnosis can be made when four or more of these criteria are
present.3 The presence of 4 of 11 ACR classification criteria was found to have a
sensitivity of 96% and a specificity of 100%.10
Management and theraphy in SLE mainly includes the use of corticosteroids
or chemotherapy as required. Corticosteroids, agents elicit anti-inflammatory and
immunosuppressive properties and modify the body's immune response to diverse
stimuli. Methylprednisolone decreases inflammation by suppression of the
immune system in much the same manner as prednisone, but it has less
mineralocorticoid effects. Cyclophosphamide, which is chemically related to
nitrogen mustards, interferes with the normal function of DNA by alkylation and
cross-linking the strands of DNA and by possible protein modification. As an
 41

alkylating agent, the mechanism of action of active metabolites may involve

cross-linking of DNA, which may interfere with the growth of normal and
neoplastic cells.5,17
- Consider initiating therapy with daily prednisone (1 mg/kg/d) or higher-dose
alternate-day prednisone (5 mg/kg/d, not to exceed 150-250 mg).
- Alternatively, the patient may be treated with IV pulse methylprednisolone
therapy (3 d of high-dose IV corticosteroids) and then switched to
intermittent high-dose IV corticosteroids with lower daily prednisone doses,
depending on disease severity.20
- Cyclophosphamide, 500-750 mg/m IV monthly; not to exceed 1 g/m

Meanwhile according to physical examination and lab analysis, patient RS was

administered with management of:

- IVFD D5% NaCl 0,45% 20 gtt/min micro

- Methylprednisolon tab 3-3-2
- Inj. Methylprednisolon (30 mg/kg BB) 870 mg in 100 cc NaCl 0,9% in an
hour (for 3 days)
- Urdafalc 3x100 mg tablet
- Chemotheraphy, cyclophosphamide postponed until result of SGPT at
normal range for (3 times)

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 43

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