CASE REPORT

ALLERGY DIVISON

JUVENILE IDIOPATHIC OLIGOARTICULAR

Rusmelani Sain

Department of Pediatric Health, Medical Faculty of Hasanuddin University,

Dr. Wahidin Sudirohusodo Hospital, Makassar.

INTRODUCTION.

Juvenile idiopathic arthritis is not a single disease, but a term that

encompasses all forms of arthritis that begin before the age of 16 years, persist for

more than 6 weeks, and are of unknown cause. The term represents, therefore, an

exclusion diagnosis that includes all forms of childhood chronic arthritis of unknown

cause. The term Juvenile Idiopathic Arthritis has replaced the older terms Juvenile

Rheumatoid Arthritis (JRA) and Juvenile Chronic Arthritis (JCA) proposed by the

International League of Associations for Rheumatology (ILAR) to diagnose chronic

arthritis in children. This disease is an active disease that can continue until adulthood

with the potential to cause functional limitations and reduce the quality of life. Until

now the cause of JIA is unknown, but the available evidence shows the influence of

genetic factors and abnormal autoimmune responses resulting in progressive

inflammation and joint destruction. (Kenan Barut 2017, Ravelli 2007).

The disease is divided into several subgroups, according to demographic

characteristics, clinical features, treatment modalities and disease prognosis. Systemic

juvenile idiopathic arthritis, which is one of the most frequent disease subtypes, is

1
characterized by recurrent fever and rash. Oligoarticular juvenile idiopathic arthritis,

common among young female patients, is usually accompanied by anti-nuclear

antibodie positivity and anterior uveitis. Seropositive polyarticular juvenile idiopathic

arthritis, an analogue of adult rheumatoid arthritis, is seen in less than 10% of

paediatric patients. Seronegative polyarticular juvenile idiopathic arthritis, an entity

more specific for childhood, appears with widespread large- and small-joint

involvement. Factors that may be associated with some types of JIA include girls

predominantly, family history of uveitis , ankylosing spondylitis, inflammatory

bowel disease, and family history of psoriasis.(Kenan Barut 2017, Arwin AP Akib

2003)

Juvenile idiopathic arthritis is an important cause of short-term and long-term

disability. Studies in developed countries have reported a prevalence that varies

between 16 and 150 per 100 000. A community-based survey in Australia reported a

prevalence of 400 per 100 000 on the basis of clinical examination of school children

by a paediatric rheumatologist. Although in western European countries the most

common subtype is represented by oligoarthritis, this same subtype is rare in

countries such as Costa Rica, India, New Zealand, and South Africa, where

polyarthritis predominates. (Ravelli 2007).

Disease complications can varied from growth retardation and osteoporosis

secondary to treatment and disease activity, to life-threatening macrophage activation

syndrome with multi-organ insufficiency. With the advent of new therapeutics over

the past 15 years, there has been a marked improvement in juvenile idiopathic

arthritis treatment and long-term outcome, without any sequelae. The treatment of

2
juvenile idiopathic arthritis patients involves teamwork, including an experienced

paediatric rheumatologist, an ophthalmologist, an orthopaedist, a paediatric

psychiatrist and a physiotherapist. The primary goals of treatment are to eliminate

active disease, to normalize joint function, to preserve normal growth and to prevent

long-term joint damage. Timely and aggressive treatment is important to provide

early disease control. (Kenan Barut, 2017)

This paper will report a case of juvenile idiopathic arthritis type

oligoarthritis in a girl aged 11 years 1 month.

3
CASE REPORT

I. PATIENT IDENTITY.

A Girl N, 11 years 1 month old was admitted to Wahidin Sudirohusodo

Hospital on October 10, 2019, was referred from the Unhas hospital with a diagnosis

of juvenile rheumatoid arthritis differential diagnosis acute rheumatic fever.

Name : Ms. N

Gender : Female

Date of birth : 19-8-2008

Age when made a case : 11 years 1 month

Hospitalized : 10 October 2019

Medical record : 898017

Starting to be accepted as a case : October 2019

Patient’s pictures

4
 PARENTS IDENTITY

Father Mother

Name Mr. L Mrs. W

Age 48 years 39 years

Occupation PNS IRT

II. HISTORY TAKING (SUBJECTIVE)

Based on heteroanamnesis from his mother.

Main complaint: Swelling in the right knee accompanied by pain.

1. History of current illness

Swelling in the right knee since 10 days before being admitted to the hospital

accompanied by pain disappearing and moving. She has difficult to walking.

No tightness, no cough. No vomit, She is lazy to eat and drink. Urination:

normal. Defecation: normal

2. History of previous illnesses

History of elbow and knee joint pain 1 year ago, 5 months later

(March 2019) there was swelling in the right knee accompanied by pain, the

patient was taken for treatment to doctor but the her parents don't remember the

kind of medication given at the time. History of frequent fever since the last 6

months.

History of treatment at Siloam Buton Hospital 3 days before being

admitted to the hospital with the same complaint and receiving amoxan, methyl

5
prednisolone, KSR and ibuprofen therapy, pain decreased after taking the drug

but over time the pain reappeared. History of trauma: slipped 1 year ago.

3. Family health history

There was no history of suffering from the same complaint in the family.

4. Patient's personal or social history

a). Maternal pregnancy history

The patient is the first child. This pregnancy is desired pregnancy.

Every month the mother checks her pregnancy to the midwife

regularly, getting vitamins and blood boosting tablets. Mother never

consumes herbs. A history of itchy and refined vaginal discharge.

History of high blood pressure and diabetes is absent.

b). Birth history

The patient was born by normal labor in a hospital, appropriare

gestational age, immediately cried. The patient was born with a birth

weight of 4000 grams. But her mother forgets the length of the body

and head circumference at birth.

c). Post-birth history

Immediately after birth, patients get injections of vitamin K, Hepatitis

B0 and Polio immunization. The condition of the patient after birth

according to the mother was good, the patient was never yellow, never

seizures, cyanotic or bleeding.

d). Nutritional history

Patients get breast milk from birth until the age of 2 years.

6
 e). Growth and development history

Normal child development.

f). Immunization history

The patient had received complete basic immunization up to polio at the

age of 9 months.

g). History of children's basic needs

Physical-biomedical needs.

Patients get breast milk from birth until the age of 2 years. The patient

gets basic immunization at birth. Parents meet food and clothing needs.

Asih (psychosocial)

The patient gets enough love from both parents and siblings. The child is

born from the parents' first marriage and is the expected child.

Mental stimulation needs.

From birth, parents pay full attention to the growth and development of

patients.

III. PHYSICAL EXAMINATION

a). General condition : moderate illness

Consciousness : Glasgow coma scale 15(E4M6V5)

Status present Vital sign

Blood pressure : 100/70 mmHg

Heart rate : 104 time per minute, regular, adequate volume

Respiratory rate : 20 breaths per minute, regular

7
 Temperature : 36,7oC

Pain scale : 2 NRS

b). General status

Organ Description

Head Normosefal, mesosefal

Hairs Black, even distribution, not easily revoked

Face There is no cranial nerve paresis, no dysmorphic

appearance, no moon face, no facies colley.

Eyes Anemis in conjunctiva, no sclera jaundice, no palpebral

edema. Eyeball motion in all directions Pupil isocor

diameter 2,5 mm/2,5 mm, light reflex +/+ normally

Nose There was depressed nasal bridge. No atresia choana

Telinga There are no secretions, intact tympani membrane

Mouth T1-T1 size tonsils, not hyperemia. Pharynx is not

hyperemia. There is no oral ulcer

Neck There is no jugular venous pressure elevation, there is no

lymphadenopathy

Thoraks Symmetrical shape and movement

Lung Symmetrical movement, bronchovesicular breath sounds in

both lung fields, there is no additional sound of wheezing

or wheezing.

Heart I-II heart sounds are normal, murmur normally.

8
 Abdomen Peristaltic normal, there is no hepatosplenomegali

Ekstremitas There is swelling in the right knee, reddish, warm feeling.

Strength 5/5/4/5 and tone are within normal limits,

physiological reflexes are normal impression, pathological

reflexes are not found.

lymph node There is no enlarged lymph node

Back There are no gibbus or scoliosis

c). Clinical nutrition

Body weight (BW) : 33 kg

Body Height (H) : 145 cm

Head circumference : 51 cm (Normal: 50-55,5 cm)

Upper arm circumference (LILA) : 14 cm

BW/H = 33/37 x 100% = 89 % ( Under Nourished)

BW/A =145/145 x 100% = 100 % ( Normal)

BB/A =33/38 x 100% = 86 % (Under Nourished)

Jones criteria :

- Major criteria : -

- Minor criteria : fever, athralgia, increasing of ESR

LABORATORY FINDINGS

a. Complete Blood Count (RS Wahidin , 11/10/2019)

Parameter Result Normal value

Hb 13,7 11,5– 14,5 g/dl

9
 HCT 39,5 37 – 45 %

MCV 83 77 – 91 fl

MCH 28,8 24 – 30 pg

MCHC 34,6 32 – 36 gr/dl

Leukocytes 7.500 4.500 – 13.500 /mm3

Trombosit 388.000 200.000 – 400.000/mm3

GDS 103 140 mg/dl

Ureum 19 10 – 50 mg/dl

Kreatinin 0,59 P < 1,1 mg/dl

SGOT 26 < 38 U/L

SGPT 29 < 41 U/L

Natrium 139 136 – 145 mmol/L

Kalium 3,8 3,5 – 5,1 mmol/L

Klorida 105 97– 111 mmol/L

ESR Hours I : 27 < 10 mm

Hours II : 52

b. Urine Routine (RSWS, 10/11/2019)

Warna Kuning Protein Negative

pH 6,5 Sedimen Leukocytes 1

BJ 1,010 Glukose Negative

Bilirubine Negative Erythrocytes 0

10
 Urobilinogen Normal Keton Negative

Blood Negative Leukocytes Negative

c. Feces Routine

Macroscopic Microscopic

Consistency Solid Erythrocytes Negative

Colour Brown Leukocytes Negative

Mucus Negative Amoeba Negative

Blood Negative Worm eggs Negative

Worm Ancylostoma Duodenale

d. Radiology Finding.

Photo of Knee joint AP + lateral D / S

(11-10-20190).

Impression: There are no radiologic

abnormalities on this genu photo.

11
 Photo of Elbow joint D / S (11-10-

2019).

Impression: There are no radiologic

abnormalities on this elbow photo.

Thoraks AP / PA Photo (11-10-2019).

Impression: There are no radiological

abnormalities on this chest radiograph.

e. Elektrokardiografi : sinus ritme

f. Echocardiografi (11-10-2019)

LV systolic function effects good with EF 70% .

III. RESUME

Swelling in the right knee since 10 days before being admitted to the

hospital accompanied by pain disappearing and moving. She has difficulty walking.

History of elbow and knee joint pain 1 year ago, 5 months later (March 2019) there

was swelling in the right knee accompanied by pain, the patient was taken for

12
treatment to doctor but the her parents don't remember the kind of medication given

at the time. History of frequent fever since the last 6 months.History of treatment at

Siloam Buton Hospital 3 days before being admitted to the hospital with the same

complaint and receiving amoxan, methyl prednisolone, KSR and ibuprofen therapy,

pain decreased after taking the drug but over time the pain reappeared. History of

trauma: slipped 1 year ago. On physical examination : Vital sign within normal

except pain scale 2 NRS. Ekstremitis : there was swelling on the right knee, looks

reddish, warm touch. Motoric strength 5/5/4/5 with physiologhic reflexs within

normal.

IV. DIAGNOSIS

1. Juvenile idiopathic artritis differential diagnosis Acute rheumatic fever

2. Undernourished

3. Ankylostomiasis

13
 V. FOLLOW UP

Follow Up Subyektif Obyektif Assesment Planning

1st day There is swelling General conditions: moderate illness Working Diagnosis Plain therapy
of the right knee
(11-10-2019) Vital sign : Tensi 100/70 mmHg, Heart  Juvenile Idiopathic Ibuprofen 200 mg/ 6hours/ oral.
accompanied by
Pukul 15.00 rate 98 time/minute, Respiratory rate Arthritis differential Calnic syrup 10 ml/ 12 hours/ oral.
pain.
There is a cough, 24 time/minute , Temperature 36,5 ºC, diagnosis Acute Elkana syrup 5 ml/ 24 hours/ oral.
No dyspneu.
Pain scale 2 NRS. rheumatic fever Ambroksol 15 mg/ 8 hours/ oral.

 Undernourished Enteral : Energy : 1650 kkal,

Defecation and
Micturition was Extremities: there is swelling in the  Ankylostomiasis Protein : 52 gram.
normal right knee, looks reddish, warm touch
Plan for tracking rheumatic fever:
temperature
- Check ASTO, CRP, Procalcitonin.
Motoric strength 5/5/4/5
- Consul of the cardiology department of
Physiologic reflex normal
children.
Instruction Prof. dr. Husen Albar Sp. A (K)

Cefixime 100 mg/ 12 hours/ oral.

KSR 600 mg/ 8 hours/ oral.

Consult to gastroenterohepatology division

14
Follow-up Subjective Objective Working Daignosis Plain Therapy
There is swelling
3rd day General conditions: moderate illness  Juvenile Idiopathic Ibuprofen 200 mg/ 6hours/ oral.
of the right knee
13-102-019 Vital sign : Tensi 100/60 mmHg, Heart Arthritis differential Calnic syrup 10 ml/ 12 hours/ oral.
accompanied by
pain. rate 108 time/minute, Respiratory rate diagnosis Acute Elkana syrup 5 ml/ 24 hours/ oral.
There is a cough,
24 time/minute , Temperature 36,5 ºC, rheumatic fever Ambroksol 15 mg/ 8hours/ oral.
No dyspneu.
pain scale 2 NRS.  Undernourished Enteral : Energi : 1650 kkal,

Defecation and  Ankylostomiasis Protein : 52 gram.

Micturition was
Extremities: there is swelling in the 3. Cefixime 100 mg/ 12 hours/ oral.
normal
right knee, looks reddish, warm touch
KSR 600 mg/ 8hours/ oral.
temperature
Gastroenterohepatologi division:
Motoric strength 5/5/4/5
Physiologic reflex normal Mebendazol 500 mg/ 24 hours/ oral (single

Prodia : dose).

C3 komplemen 168, 6 mg/dl (80-150) Feces routine control 2 weeks later.

15
Follow-up Subjective Objective Working Diagnosis Plain Therapy
There is swelling
4th day General status : moderate illness  Juvenile Idiopathic Ibuprofen 200 mg/ 6hours/ oral.
of the right knee
(14-10-2019) Vital sign : Tensi 100/70 mmHg, Heart Arthritis type Calnic syrup 10 ml/ 12 hours/ oral.

Defecation and rate 90 time/minute, Respiratory rate oligoarthritis Elkana syrup 5 ml/ 24 hours/ oral.
Micturition was
20 time/minute , Temperature 36,5 ºC,  Undernourished Ambroksol 15 mg/ 8hours/ oral.
normal.
pain scale 1 NRS  Ankylostomiasis Enteral : Energy : 1650 kkal,

Protein: 52 gram.

Extremities: there is swelling in the 4. Cefixime 100 mg/ 12 hours/ oral.

right knee, looks reddish, warm touch
KSR 600 mg/ 8hours/ oral.
temperature
Motoric strength 5/5/5/5
Physiologic reflex normal.

Lab RSWS 14-10-2019 :

ASTO 386 IU/ ml (<200)

RF : 32,4 IU/ml (<30)

CRP : 1,2 (<5).

Peripheral smear impression:

Leukocytes with signs of infection

16
Follow up Subjective Objective Working Diagnosis Plain therapy
There is swelling
5th day General status : moderate illness.  Juvenile Idiopathic Ibuprofen 200 mg/ 6hours/ oral.
of the right knee
(15-10-2019) Vital sign : Tensi 110/70 mmHg, Heart Arthritis type Calnic syrup 10 ml/ 12 hours/ oral.

Defecation and rate 90 time/minute, Respiratory rate oligoarthritis Elkana syrup 5 ml/ 24 hours/ oral.
Micturition was
22 time/minute , Temperature 36,5 ºC,  Undernourished Ambroksol 15 mg/ 8 hours/ oral.
normal
pain scale 1 NRS  Ankylostomiasis Enteral : Energy : 1650 kkal,

Protein : 52 gram.

Extremities: there is swelling in the 5. Cefixime 100 mg/ 12 hours/ oral.

right knee colors and sound warranty

everything.
Motorik kekuatan 5/5/4/5
Physiologic reflex normal

Lab RSWS 14-10-2019 :

Hb ; 13,6 gr/dl Wbc : 7.580/ ul.

Plt : 399.000/ul.

17
I. DEFINITIF DIAGNOSIS
1. Juvenile idiopathic artritis tipe oligoartritis.

2. Undernourished

3. Ankilostomiasis

VI. PROGNOSIS

- Ad vitam : dubia et bonam

- Ad sanationam : dubia ad bonam

- Ad functionam : dubia ad bonam

XI. CASE ANALYSIS

Juvenile Idiopathic Arthritis (JIA) is the most common chronic arthritis in

children worldwide. It is a heterogeneous inflammatory disease and defined as

arthritis persisting 6 weeks or longer with onset before the age of 16 years with no

identifiable etiology. The International League of Associations for Rheumatology

(ILAR) classifies JIA based on the pattern of arthritis in the first 6 months after

the onset of disease into 7 subtypes: (MohammadH.Al-Hemairi, 2016)

1. Oligoarticular, involving 4 joints or less during the first 6 months.

2. Polyarticular (RhF negative), involving 5 or more joints, Rheumatoid factor

(RhF) antibodies negative on blood tests.

3. Polyarticular (RhF positive), involving 5 or more joints, Rheumatoid factor

(RhF) antibodies positive on blood tests, this type most closely resembles

rheumatic arthritis in adults.

18
4. Systemic, Fever of ≥2 weeks and arthritis in ≥1 joint with systemic

symptoms such as high fever, episodic erythematous rash, lymphadenopathy

and hepatoslenomegaly

5. Enthesis, formerly known as Juvennile spondyloarthropathy, is a chronic

arthritis that is accompanied by enthesis, namely inflammation of the

insertion of tendons, ligaments or bone fascia.

6. Psoriatic, involving asymmetric large and small joints, there is psoariasis or

evidence of psoriasis diathesis (2 of the following criteria: family history,

nail pits or onycholysis or dactylitis).

7. Undifferentiated arthritis: Does not fit into one category or correspond to

more than one category.

Incidence and prevalence in European and North American populations

range from 2 to 20 and from 16 to 150 per 100,000, respectively. However,

remarkable disparity in the frequency of JIA subtypes has been noticed in

different geographical areas or ethnic groups. In Western countries oligoarthritis

is the most common subtype, while polyarthritis predominates in Costa Rica,

India, New Zealand, and South Africa. In Asia, systemic arthritis accounts for a

greater proportion of childhood arthritis. In India, Mexico, and Canada, a greater

incidence of enthesitis-related arthritis (ERA) has been registered (Gabriella

Giancan, 2016). The descriptive retrospective study Allergy Immunology

Division, Department of Child Health Dr. Hasan Sadikin Hospital Bandung

during January 2006 – October 2011 period. There were 28 patients with JIA

consisted of 10 boys and 18 girls, age ranged 2–14 years, with mean age of onset

of 8.25±3.62 years. Male: female ratio 1: 1.8. There were 14 patients with

19
persistent oligoarthritis type, 6 patients with systemic type, 5 patients with

polyarthritis type and 1 patient with polyarthritis adult type. Laboratory finding

showed 2 patients with positive rheumatoid factor and 14 patients were negative.

(Rheni Ghrahani 2012).

Juvenile idiopathic arthritis is a disease that affects children under the age

of under 16 years, rarely affecting children under 6 months. Generally the highest

peak of onset is found at the age of 1-3 years, while the second peak onset is

found at the age of 8-10 years. Some researchers find the highest peak of onset at

age 2-4 years and the second peak of onset at age 11-13 years. The average onset

of age is 6.9 (SD 3.2) years, the type of oligoarthritis and polyarthritis has a peak

onset according to the literature which is at the peak of the second onset (8-10

years). The general description of all JIA subtypes is olygoarthritis. In this patient

she was 11 years old where the JIA was <16 years. (Minden K 2002, Miller ML

2004).

The aetiopathogenesis of the disease is still unclear. The most acceptable

theory supports the influence of immunogenic mechanisms secondary to different

genetic and environmental factors. Infections, together with stress and trauma, are

considered to be the most responsible aetiological factors. Gut microbiota is

emerging as a relevant factor of autoimmune diseases, including JIA, according to

recent studies. The increased frequency of autoimmune diseases among JIA

patients suggests the genetic basis of the disease. Human Leukocytes antigen

(HLA) B27 and the other HLA tissue types are the most commonly mentioned

genetic factors. Various infections are considered to be responsible for JIA

pathogenesis: enteric infections, parvovirus B19, rubella, mumps, hepatitis B,

20
Epstein-Barr virus, mycoplasma and chlamydia infections. Potential trigger-

induced T-lymphocytes and secreted cytokines lead to joint destruction.

Macrophages, induced by secreted mediators, produce pro-inflammatory

cytokines [interleukin (IL) 1, IL-6, tumour necrosis factor (TNF)-α]. Thus, the

acute phase markers [C-reactive protein (CRP), erythrocyte sedimentation rate

(ESR)] increase and the acute inflammation of joints occurs with an increase in

synovial fluid. Synovial inflammation (synovitis) is characterized by villous

hypertrophy and hyperaemia of the subsynovial tissue. Synovial hypertrophy and

synovitis secondary to chronic inflammation are known as “panni”. The T-

lymphocyte percentage in synovial fluids varies among different JIA subtypes,

possibly explaining the difference in treatment response among JIA subgroups.

(Barut 2017, Youn-soo 2010).

The criteria of arthritis consist of swelling in the joint or there are > 2

conditions as follows: pain, warmth, and limited mobility. Other causes of arthritis

must first be ruled out before diagnosing JIA. Many systemic symptoms occur in

JIA systemic and polyarticular subtypes including fatigue, weight loss, anemia,

anorexia and fever. Inflammation causes joint discomfort, especially joint

stiffness in the morning. Large joints are more affected although all joints can be

involved such as cervical, thoracolumbal and temporomandibular joints. In young

children often joint stiffness in the morning and changes in posture. Oligoarticular

disease develops in at least 50% of children with JRA during the first 6 months of

disease. This subtype is the only form of JRA without an adult equivalent.

Oligoarticular disease affects up to 4 joints at presentation, with the knee joints

mostly affected, followed by the ankles. In contrast, this subtype almost never

21
affects the hips, and rarely the smaller joints of the hands and feet. Oligoarticular

type is characterized by asymmetric arthritis, early onset (before 6 years of age),

female predilection, high frequency of positive ANAs, and a high risk of

iridocyclitis. The ILAR classification distinguishes 2 further categories within the

oligoarthritis subtype: persistent oligoarthritis, in which the disease is confined to

4 or fewer joints, and extended oligoarthritis, in which the arthritis extends to

more than 4 joints after the first 6 months of disease. Up to 50% of oligoarticular

patients develop extended disease, and 30% will do so in the first 2 years after

diagnosis. The risk factors for extended disease include involvement of an upper

limb joint and elevated erythrocyte sedimentation rate (ESR) at onset. (Angelo

Ravelli 2007, Youn-soo 2010).

This patient : female gender, age 11 years (<16 years) with complaints of

pain and swelling in the joints in the knees, elbows, missing arise and move, She

has difficulty walking, less than 4 joints is called oligoarthritis. The results of

investigations found that the rate of sedimentation increased, the rheumatoid

factor increased slightly, and the ANA was positive, C3 complemen decreases.

Inflammatory markers in JIA patients include:

1. C-reactive protein is an acute phase plasma protein that is reactive to the

polysaccharide C in the germ wall. CRP used to assess the presence of non-

specific acute inflammation and its measurement is not affected by time and

other auto components of serum.

2. Rheumatoid factor (RF) is an antibody to the region Fc immunogloblin G,

can be used as a parameter to determine disease activity, although RF

sensitivity is high but its specificity is low because RF can also be found in

22
 other autoimmune patients such as lupus, Sjorgen's syndrome, infectious

diseases (hepatitis, Tuberculosis). If found earlier, it is associated with more

aggressive disease.

3. Measurement of complement levels can be used as a marker of disease

infection.

The laboratory examination of inflammatory indicators may be normal,

although the rate of sedimentation of the blood (ESR) is usually normal or slightly

increased usually found in the acute phase. The presence of anemia or an increase

in the rate of sedimentation of the blood (ESR) or C-reactive protein (CRP)

increases the risk of the progression of extended oligoarthritis or polyarticular

disease. In these patients get normal levels of hemoglobin and CRP but the ESR

increase ie ESR : Clock 1 = 27 mm, Clock II LED = 52 mm. (Kenan Barut 2017).

Initially based on the main symptoms of joint pain this patient was

diagnosed with acute rheumatic fever. On physical examination found no heart

abnormalities. On investigations, a normal chest radiograph was obtained,

electrocardiography was not obtained by lengthening the PR interval,

echocardiography was not found for valve abnormalities, only ASO was increased

(386 IU), ESR were increased but CRP was normal. Indeed, the diagnosis of acute

rheumatic fever is difficult especially with the absence of pathognomonic signs

and specific laboratory tests. In the past 50 years this diagnosis has been guided

by Jones criteria through observing a combination of variable signs and symptoms

that occur after oropharyngeal streptococcal infection. Based on Jones criteria:

only minor criteria: fever, atralgia, increased ESR. Indeed, most people with acute

rheumatic fever experience arthritis as an initial manifestation. The main criteria

23
for the diagnosis of recent evidence of streptococcal infection include detection of

antistreptococcal antibodies such as antistreptolysin-O and antideoxyribonuclease

B. However, there are several factors that may be confusing and may hinder

interpretation. First, school-age children have more frequent streptococcal

infections and can show higher ASO values due to concurrent infections but are

not related to diagnosis. Second, patients with rheumatic fever who have isolated

chorea manifestations can show normal ASO levels due to latency of 1 to 6

months between infection and clinical manifestations.(Claudia saad 2001).

JIA female patients with early disease onset. Since these patients are at

an especially high risk of uveitis, the ophthalmological examination should be

performed every three months. The most frequently reported type of uveitis is

chronic anterior uveitis (68.3%), followed by acute anterior disease (16.2%),

recurrent anterior disease (12%) and panuveitis (3.5%). Among 1081 JIA cases,

the uveitis percentage was reported as 13.1%. The onset of uveitis in

oligoarticular JIA patients is often insidious and usually asymptomatic, although

half of the children could have some symptoms related to uveitis (pain, redness,

headache, photophobia, change in vision). Uveitis is characterized by attacks of

unilateral or bilateral acute, painful, photophobic iritis and with hyperaemia of the

sclera and conjunctiva. Patients with HLA B27 positivity are more prone to

developing acute anterior uveitis. (Kenan Barut 2017, Youn soo 2013).

Tabel : Features of three of the major subtypes of juvenile idiopathic arthritis

(JIA)

Systemic JIA Polyartricular JIA Oligoartcular JIA

Percent of JIA 10-15 30-40 50

24
patients
Sex F=M F>M F>M
Age Any <17 years Peak : 2-3, 10-14 Peak : 2-3 years,
years rare >10 years
Joints Any number and Any, usually Large joints, but
any joint symmetrical and rare rarely hips
o start in hips
Fever, rash, Yes No No
lymphadenopathy,
hepatosplenomegaly
Uveitis Rare Less frequently seen 20 %, most
than in oligoarticular common in patients
JIA who are ANA
positive
Leukocytsosis Marked No No
Anemia Marked Mild No
Elevated ESR Marked Mid Mild
ANA Absent Low titer commo in Low titer common
younger
Rheumatoid factor Rare 10-20% in those >10 Absent
years
Elevated ferritn Marked Mild No
Artritis destruktif >50 % >50 % Rare

Conventional radiography remains the gold standard for the detection of

structural joint damage and growth and maturation disturbances of bones in JIA

patients. In recent years, a great deal of effort has been made to develop new

radiographic scoring systems or to adapt adult methods for use in JIA. However,

the poor sensitivity of plain radiographs in identifying active synovitis and its

limited ability to disclose erosive changes early in the disease course has raised

25
interest in alternative imaging methodologies. In this patient, no radiological

abnormalities were found (gabrile Giancane, 2016).

Basically, the management of pediatric rheumatic diseases aims to

obtain normal physical and psychological growth and development status in order

to lead an optimal life. Optimal treatment efforts if possible are done by

pediatricians, rheumatologists, nurses, physiotherapists, occupational therapists,

and social workers. In addition, collaboration and consultation with experts in the

fields of ophthalmology, dental-mouth, orthopedics, cardiology, psychiatry,

nephrology, dermatology, and other fields are often requested for cooperation.

Support for patients and families needs to be done by psychologists, psychiatrists,

and other sources in the community.

Management of JIA is a combination of anti-inflammatory,

immunomodulatory with occupational therapy, physiotherapy, surgery, nutrition,

psychosocial and educational partnership between patients and parents. JIA

Medications can use nonsteroidal anti-inflammatory drugs (NSAIDs), disease

modifying antirheumatoic drugs (DMARDs) or immunosuppressive drugs and

systemic corticosteroids, intra-articular corticostreoid and modifying biologic

agents. Standard treatment for JIA consists of: Nonsteroid anti-inflammatory

drugs (NSAIDs) such as Ibuprofen, Diclofenac. Conventional DMARDs such as

Methotrexate, Hydroxycloroquine, DMARDs Biologic Etanercept, TNF

inhibitors, Calcium supplementation 1000 - 1500 mg and Vitamin D 400 IU daily

on ARJ due to the risk of osteoporosis and osteopenia due to corticosteroid

therapy (> 8 weeks) and these patients are given a multivitamin. (Cobb JE, 2014).

26
 NSAIDs are used to treat joint pain, stiffness and fever associated with

systemic arthritis. Anti-inflammatory drugs in arthralgia and arthritis because they

inhibit inflammatory and painful reactions by reducing the activity of

cyclooxygenase thereby reducing prostaglandin synthesis. Patients with JIA type

oligoarthritis remission can be induced by NSAIDs. The main characteristics of

this drug are its analgesic effect in lower doses and anti-inflammatory effects

when used in higher doses. Treatment response is seen in the first 1-3 days with

pain relief. This type of drug is considered tolerable in pediatric patients, despite

stomachaches and headaches which can sometimes be seen as side effects.

NSAIDs are used 3 times a day to monitor serum levels.(Kenan Barut, 2017).

Corticosteroids are given in conditions of severe fever, serositis, and

macrophage activation syndrome or as a connecting therapy so that treatment is

more effective. Long-acting corticosteroids like Triamcinolone injected

intraarticularly have better efficacy when compared intravenously or by mouth.

Prednisone 0.25 - 1 mg / kg / day peroral single / divided dose (max 40

mg) and tapering off after clinical improvement. Methylprednisolone 1-2 mg /

kg / day if systemic symptoms persist. Hexamethonone triamcinolone 20-40 mg

intra-articularly in large joints, if it does not respond to NSAIDs or supportive

therapy for inflammation and joint contractures. Research conducted by Beth S.

Gothlieb et al showed that 70% of patients with oligoarthritis had no reactivation

of the disease in the intraarticular injected joints for 1 year and 40% after more

than 2 years. The side effect of this intraarticular injection is subcutaneous

atrophy, which can be prevented by injection of a small amount of saline into the

joint and followed by an emphasis on the injection site. Long acting triamcinolone

27
is more effective and more durable than other corticosteroid injections.. (Brewer

EJ, 1977).

The use of aspirin (acetylsalicylic acid) in doses sufficient to maintain

blood levels often alleviates the arthritis and systemic manifestations of the

disease, and in these patients had previously received aspirin therapy.

The benefits for patients are expected with good and continuous

monitoring and intervention of children with JIA can have a better quality of life,

prevent complications and get a better prognosis.The benefit for the family is the

family gets the knowledge and understanding of the child's illness related

conditions, complications, prognosis and management that are applied so that they

can work together in dealing with these sufferers. The role of the family is very

important in the successful management of children with JIA because it requires

patience, patience and support from the whole family so that children continue to

be enthusiastic about undergoing a series of JIA governance.( Schanberg LE,

1999).

IX. PROGNOSIS

Novel biological therapeutics, early aggressive treatment and effective

intra-articular administration of steroids have lead to marked improvement in the

prognosis of JIA. Despite this progress, some patients still have an active disease

with a progressive clinical course. Although the majority of oligoarticular JIA

patients achieve remission, some of them still do not resolve totally. The risk of

uveitis, joint erosions with the development of severe sequelae and extension of

the disease to polyarticular disease makes oligoarticular JIA an entity that requires

28
careful monitoring with a need for aggressive, early treatment in cases of higher

risk. A family history of the disease, early ankle and hip joint involvement and a

higher number of affected joints at disease onset are indicators of poor prognosis

in ERA patients

X. COMPLICATIONS
•

 Impaired growth and development due to early epiphyseal closure

 Other bone and joint abnormalities can also occur such as angkilosis, luxasi

or fracture

 Onset oligoarthritis: uveitis is often asymptomatic.

 Macrophage activation syndrome. (Malleson,2001).

29
 DAFTAR PUSTAKA

1. Barut K, Adrovic A, Şahin S, Kasapçopur O. Juvenile Idiopathic Arthritis.

Balkan Med J 2017;34:90-101. DOI: 10.4274/balkanmedj.2017.0111.
Available at www.balkanmedicaljournal.org

2. Ravelli A, Martini A. Juvenile idiopathic arthritis. Vol 369 March 3,

2007. www.thelancet.com.

3. Akib, Arwin AP. Artritis Idiopatik Juvenil Kesepakatan Baru Klasifikasi

dan Kriteria Diagnosis Penyakit Artritis pada Anak. Jakarta ; Sari
Pediatri, Vol. 5, No.2, September 2003.

4. Al-Hemairi MH, Albokhari SM, Muzaffer MA. ResearchArticle The

Pattern of Juvenile Idiopathic Arthritis in a Single Tertiary Center in Saudi
Arabia. International Journal of Inflammation Volume 2016, Article ID
7802957, 8 pages http://dx.doi.org/10.1155/2016/7802957.

5. Giancane G, Consolaro A, Lanni S, Davı S, Schiappapietra B, Ravelli A.

Juvenile Idiopathic Arthritis: Diagnosis and Treatment. Rheumatol Ther.
DOI 10.1007/s40744-016-0040-4.
http://www.medengine.com/Redeem/89E4 F060559BF6A3.

6. Ghrahani R, Setiabudiawan B, Sapartini G, Puspasari H. Distribusi

Subtipe Juvenile Idiopathic Arthritis di Bandung. MKB, Volume 44 No. 2,
Tahun 2012.

7. 7.Minden K. Long-term outcome in patients with juvenile idiopathic

arthtritis. Arthritis Rheum 2002;46:2392- 401.

8. Miller ML, Cassidy JT. Juvenile rheumatoid arthritis. Dalam: Behrman

RE, Kliegman RM, Jenson HB, penyunting. Nelson’s textbook of
pediatrics. Edisi ke-2. Philadelphia: W.B. Saunders;2004. h. 799-805.

9. Youn-Soo Hahn, Kim JG. Pathogenesis and clinical manifestations of

juvenile rheumatoid arthritis. Korean J Pediatr 2010;53(11):921-930. DOI:
10.3345/kjp.2010.53.11.921

10. Machado, CSM, Ortiz K, Martins ALB, Martins RS, Machado NC.
Antistreptolysin O titer profile in acute rheumatic fever diagnosis. Jornal
de Pediatria. 2001 - Vol. 77, No 2 : 105-11.

11. Brewer EJ. Current proposed revision of JRA Criteria. Arthritis Rheum
1977; 20(2 suppl):195-9.
12. Schanberg LE, Sandstrom MJ. Cause of pain in children with arthtritis.
Rheum Dis Clin North Am 1999;25(1):1-14.

30
 13. Cobb JE, Hinks A, Thomson W. The genetics of juvenile idiopathic
arthritis: current understanding and future prospects. Rheumatology
(Oxford). 2014 Apr;53(4):592-9. doi: 10.1093/rheumatology/ ket314.
Epub 2013 Sep 18. Review. Citation on PubMed:
https://www.ncbi.nlm.nih.gov/pubmed/24049100.

14. Malleson PN, Beauchamp RD, Esdaile JM. Diagnosing musculoskeletal

pain in children. CMAJ 2001;165:183-8.

CASE REPORT
ALLERGY DIVISION

31
 JUVENILE IDIOPATHIC OLIGOARTICULAR

RUSMELANI SAIN

NIM C110215207

DEPARTMENT OF PEDIATRIC HEALTH

MEDICAL FACULTY HASANUDDIN UNIVERSITY

MAKASSAR

2019

32