juvenile reumatoid atritis dan tatalaksananya.ppt

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1. Juvenile
Rheumatoid
Arthritis
AHMAD MUKHLIS

2. Definition - JRA
•JRA adalah istilah umum untuk semua jenis
arthritis dan kondisi terkait yang terjadi (Arthritis
Foundation 2009). Patologi utama terjadi adalah
adanya peradangan pada jaringan ikat yang
ditandai dengan adanya pembengkakan dan
nyeri.

3. JUVENILE RHEUMATOID ARTHRITIS
American College of Rheumatology Revised Criteria
•Usia < 16 tahun
•Atritis pada satu atau lebih sendi
•Durasi Penyakit > 6 minggu
•Kondisi lain yang muncul pada arthritis
pada masa kanak-kanak harus
disingkirkan.

4. JUVENILE RHEUMATOID ARTHRITIS
Epidemiology
•Terdapat di semua ras dan wilayah
geografis
•Insiden: 6 – 19,6 kasus/100.000 anak
•Prevalensi: 16-150/100.000
•Wanita > Pria 2:1

5. 5
Clinical Manifestations of JRA

6. Symptoms and Types
•Juvenile arthritis adalah perubahan besar pada
persendian termasuk peradangan, kontraktur,
dan kerusakan sendi yang memengaruhi
mobilitas, kekuatan, dan daya tahan.

7. •Dampak psikologis dan sosial bersifat
multidimensi, nyeri dan kekakuan sendi menjadi
gangguan, subtipe dibedakan berdasarkan
jumlah sendi yang terlibat dalam 6 bulan pertama
sejak timbulnya penyakit.

8. •Polyarticular arthritis: radang sendi pada lima
sendi atau lebih dengan gejala utama nyeri pada
lutut, pergelangan kaki, pergelangan tangan, jari
tangan, siku, dan bahuArtritis
•Pauciartikular: radang sendi pada empat sendi
atau kurang dalam 6 bulan pertama sejak
timbulnya penyakit; sendi besar lutut,
pergelangan kaki, siku, dan pergelangan tangan.

9. ILAR Proposed Classification
Criteria
Juvenile Idiopathic Arthritis (JIA)
•Systemic
•Polyarticular RF+
•Polyarticular RF-
•Oligoarticular
•Persistent
•Extended
•Pesoriatic arthritis
•Enthesitis-related arthritis
•Other arthritis

10. JUVENILE RHEUMATOID ARTHRITIS
Laboratory Studies: Pauciarticular Disease
•CBC: normal
•ESR: usually normal
•ANA: frequently positive
•RF: usually negative
•Synovial fluid: class II (inflammatory)
•X-ray findings: soft tissue swelling, periarticular
osteoporosis, growth disturbance, loss of joint
space

11. JUVENILE RHEUMATOID ARTHRITIS
Clinical features: systemic disease
•10-20% of patients with JRA
•Prominent systemic symptoms: fever, rash,
lymphadenopathy, hepatosplenomegaly,
pericarditis, pleuritis
•Uveitis uncommon

12. JUVENILE RHEUMATOID ARTHRITIS
Laboratory studies: systemic disease
•WBC   , Hgb , platelets  to  ,
•ESR   to   
•ANA and RF usually negative
•X-rays : soft tissue swelling

13. JUVENILE RHEUMATOID ARTHRITIS
Extra-articular Manifestations
•Generalized or local growth disturbances
•Delayed puberty
•Pericarditis, myocarditis, rarely endocarditis
•Plural effusion, rarely
•Pneumonitis, pulmonary fibrosis
•Hepatitis
•Hematuria

14. 14
Prognosis of JRA
•Pauciarticular JRA
–Boys may be affected in older childhood or
adolescence; this may represent an early
manifestation of a spondyloarthropathy.
–Leg length discrepancy from asymmetric knee
synovitis and bone growth may cause flexion
contractures, gait abnormalities and long-term
growth abnormalities.

15. –Eye involvement as anterior uveitis, may lead
to scarring or blindness in ~ 15-20% of
children.
Prognosis of JRA

16. 16
•Polyarticular JRA and Systemic JRA
–Active arthritis into adulthood: 50% to 70% of
polyarticular or systemic onset JRA;
–Long-term disabilities: 30% to 40% of children
•Unemployment: 25% to 50% of adult JRA
patients;
•May need major surgery (joint replacement).
–Mortality rate: 0.4% to 2% (greater risk with
systemic JRA than with polyarticular JRA).
Prognosis of JRA

17. 17
Before the 1990s …
Pyramid Approach
Traditional Approach to the
Treatment of JRA
Cytotoxic Drugs
Disease Modifying
Anti-Rheumatic Drugs
(DMARDs)
Intra-Articular/Oral Corticosteroids
Non-Steroidal Anti-Inflammatory Drugs
(NSAIDs)

18. 18
Paradigm shift….
The trend in managing JRA is much more
aggressive treatment earlier in the disease
course with the goal of preventing joint damage
and slowing progressive articular damage.
Evolving Treatment of JRA
Since the 1990s and
into the 2000s…

19. 19
•Non-Selective NSAIDs
–Aspirin, tolmetin sodium, ibuprofen, naproxen
–Naproxen [Tablets and Suspension]
•Indicated for patients 2 years and older with
juvenile arthritis.
•Daily dose: approximately 10 mg/kg/day as a
BID dose (5 mg/kg given twice-a-day). Total
daily dose is not to exceed 15 mg/kg/day.
Treatments with Indications
for JRA

20. •Adverse events: gastrointestinal, central
nervous system (headache, dizziness,
drowsiness, vertigo), rash (ecchymoses,
purpura), pruritus, sweating, special senses
(tinnitus, visual disturbances, hearing
disturbances), cardiovascular (edema,
palpitations) prolonged bleeding times.
Treatments with Indications
for JRA

21. 21
•Non-Selective NSAIDs/COX-2 Selective
Inhibitors
–MOBIC (meloxicam) [Tablets and Suspension]
•Indicated for the relief of the signs and
symptoms of pauciarticular and polyarticular
course JRA in patients 2 yrs and older.
•0.125 mg/kg once daily up to a maximum of 7.5
mg.
Treatments with Indications
for JRA

22. •Adverse events: abdominal pain/upper, vomiting,
diarrhea, headache, infection (rhinitis), cough,
pyrexia, rash. urticaria, slight increases in
systolic blood pressure.
–VIOXX (rofecoxib) [Tablets and Suspension]
•Withdrawn from the global market September
2004.
•Indicated for the relief of the signs and
symptoms of juvenile rheumatoid arthritis in
patients 2 years and older.
Treatments with Indications
for JRA

23. 23
Treatment of JRA
•Corticosteroids
–Used for uncontrolled or life-threatening systemic
disease;
–Treatment of chronic uveitis as local ophthalmic
drops; or
–Intra-articular agents (Pauci- and polyarticular
JRA)
–Intermediate-acting corticosteroids: Prednisone;
methyl-prednisolone (Intravenous pulse therapy
for severely active JRA).

24. Treatment of JRA
•Prednisone low-dose as 0.1 to 0.2 mg/kg;
higher-dose 0.25 to 1.0 mg/kg/day (maximum
single dose 40 mg)
•Adverse events: hypertension, iatrogenic
Cushing’s syndrome, growth suppression,
fractures, cataracts, increased susceptibility to
infection.

25. 25
•DMARDs and Biologic DMARDs
–Methotrexate (MTX): used when NSAIDs fail to
bring relief.
•Indicated for polyarticular JRA. MTX is the most
widely used DMARD for JRA treatment.
•Starting dose 7.5 mg/m
2
per week; maximum dose
of 15 mg/m
2
per week.
Treatment of JRA

26. •Adverse events: stomatitis, leukopenia, nausea/
abdominal pain, gastrointestinal bleeding,
anorexia, malaise, fatigue, chills and fever,
headache, alopecia, rash, decreased resistance to
infection, elevated hepatic enzymes.
Treatment of JRA
–Sulfasalazine
•Indicated for polyarticular JRA who have
responded inadequately to salicylates or other non-
steroidal anti-inflammatory drugs.

27. 27
•Children 6 yrs and older: 40 - 60 mg/kg/day
divided into 3 to 6 doses.
•Maintenance dose: 30 mg/kg/day divided into 4
doses.
•Adverse events: anorexia, headache, vomiting,
gastric distress, rash, urticaria, hemolytic anemia.
Treatment of JRA
–ENBREL (etanercept):
•Indicated for moderate to severe polyarticular
course JRA patients 4 to 17 years of age who had
an inadequate response to one or more DMARDs.

28. 28
•Dosage: 0.4 mg/kg/week (maximum 25 mg/ dose
given twice weekly) as subcutaneous injection pre-
filled syringe, 72-96 hrs. apart.
•Adverse events: headache, nausea, abdominal
pain, and vomiting. Infection was reported in 43 of
69 (62%) of JRA patients during the 3-month
(open-label phase).
Treatment of JRA

29. 29
•Pauciarticular
–25% to 33% will respond to NSAIDs;
–Patients not responsive to NSAIDS after 4 - 6
weeks with flexion contractures or leg length
discrepancy  intra-articular corticosteroids.
–Patients with extended pauciarticular JRA or small
joint involvement  treat as polyarticular JRA.
Modified from Laxer R, Hashkes PJ. Medical Treatment of Juvenile Idiopathic Arthritis. JAMA, October 5, 2005. Vol
294, No. 13, pp 1671-1684.
Treatment of JRA in 2008

30. 30
•Polyarticular
–RF (-) or (+), NSAID (symptom control) alone is
usually not as effective as a NSAID + DMARD.
–NSAID trial for several weeks  add oral MTX.
–If oral MTX is not effective  parenteral route MTX.
–If NSAID + MTX (oral or parenteral) is not effective
 anti-TNF medication.
•No current evidence whether a combination of
MTX + anti-TNF medication are more effective
than only anti-TNF medication.
Treatment of JRA in 2006
Modified from Laxer R, Hashkes PJ. Medical Treatment of Juvenile Idiopathic Arthritis. JAMA, October 5, 2005. Vol
294, No. 13, pp 1671-1684.

31. 31
•Systemic
–NSAIDs 2 to 3 weeks with caution  risk of
Disseminated Intravascular Coagulation (DIC),
(macrophage activation syndrome);
–Intravenous pulse methylprednisolone;
–Oral corticosteroids 
•Lowest effective dose;
•Steroid sparing  immunomodulatory approach
is under evaluation for steroid sparing effects.
Modified from Laxer R, Hashkes PJ. Medical Treatment of Juvenile Idiopathic Arthritis. JAMA, October 5, 2005. Vol
294, No. 13, pp 1671-1684.
Treatment of JRA in 2006

32. TERIMA KASIH