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JUVENILE_PSORIATIC_ARTHRITIS.ppt
1. JUVENILE PSORIATIC ARTHRITIS
SUPERVISOR :
Prof. Dr. Dr. Harsoyo Notoatmojo, DTM&H, Spa
Dr. Wistiani, Spak, Msi.Med
Dr. Galuh Hardaningsih, Msi.Med, Spa
Dipresentasikan oleh :
Sylphana Astharica Lawalata
2. Riwayat Penyakit
8 bulan Lalu
Kedua lutut bengkak, warna kulit sama
seperti sekitar, perabaan hangat dan
sering mengeluh kesakitan. Kadang
disertai dengan demam, namun suhu
tidak diukur. Sudah diperiksakan ke RSUD
dilakukan pemeriksaan foto rontgen lutut
dan diberi obat ibuprofen syrup.
3 bulan Lalu
Rujuk Ke IGD RSDK : arthritis genue
duplek, psoriasis. Mendapat perawatan,
dengan terapi inj methyl prednisolone 20
mg/24 jam (2 mg/kg/hari) ,syrup
ibuprofen 100mg/8jam. Konsul mata
Uveitis anterior (-). Perawatan 5 hari
perbaikan rawt jalan
Poliklinik
Mei 2018
Agustus 2018
Januari 2018
Nyeri dan bengkak pada kedua lutut 1
hari yang lalu, kesulitan berjalan. Tungkai
kanan sulit diluruskan. Anak diberi
ibuprofen syrup ½ cth bila nyeri saja.
3. 2 tahun 7 bln, BB 10,3 kg, PB 79 cm
Berobat di poli imun : 11 Januari 2017
Data : Alloanamnesis dan Catatan rekam medis
Pemeriksaan Fisik
KU : Sadar,
TTV: HR 125 x/ menit
Nadi kuat
RR 24 x/ menit
SpO2 99%
T 36,9 oC (aksiler)
Kepala : mesosefal
Wajah : dismorfik (-)
Mata : anemis (-), sklera ikterik (-)
Telinga : sekret (-/-)
Hidung : napas cuping (-), sekret (-)
Mulut : sianosis (-)
Cor : BJ I-II normal,
bisisng (-)
Thorax :simetris , retraksi (-)
Pulmo: SD vesikuler +/+ +/+
ST hantaran-/- -/-
ronkhi -/- -/-
wheezing -/- -/-
Abdomen: Cembung, supel, Hepar/Lien
Tidak teraba. Ruam
hipopigmentasi dan
hiperpigmentasi diseluruh
tubuh, skuama (+)
Extremitas :
Akral hangat : +/+ +/+
Oedem Genue : +/+ +/+
Inflamasi : -/- -/-
4. Pemeriksaan Penunjang
MRI Genu dex et sin (Agustus 2018)
Kesan :
Tidak tampak join effusion kanan kiri
Tak tampak edema prefemoral, quadriceps dan Hoffa
fat pada kanan kiri
Tak tampak fraktur maupun erosi pada tulang yang
tervisualisasi
Tak tampak tear, penebalan maupun perubahan
intensitas sinyal pada meniscus medalis dan lateralis
kanan kiri
Tak tampak perubahan intensitas sinyal pada
ligamnetum, muskulus maupun tulang
Multiple limfanodi ukuran sub sentimeterregio
poplitea kanan-kiri
Agustus 2018
Rheumatoid Factor : Neg
5. Dignosis dan Tatalaksana
Juvenile Psoriatic Arthritis Ibuprofen syr 1 cth/8jam (10 mg/kg/hr)
Desoximethason zalf, ue/ 12 jam
Program :
Fisioterapi di RS Jepara
Kontrol bulan depan (Februari 2019)
7. Epidemiologi
Prevalence of 0.10% to 0.25% in the United
States and occurs in all ethnic groups
The age at onset of JPsA is biphasic. A first peak
occurs during the preschool years, and a second is
seen during middle to late childhood
Series that recognize patients on the basis of frank
psoriasis, or using ILAR criteria, find that JPsA
represents approximately 7% (range: 0% to 11.3%)
of patients with JIA
J.D. Canete, J.R. Rodriguez, G. Salvador, et al., Diagnostic usefulness of synovial vascular morphology in chronic arthritis: a systematic survey of 100 cases, Sem. Arthritis
Rheum. 32 (2003) 378–387
8. Patologi
• In the proper genetic context, an environmental trigger such as
infection or trauma appears to unleash an inflammatory process
involving infiltration of lymphocytes as well as neutrophils and other
effectors of innate immunity into entheses and synovium.
• The target of this immune response remains unknown. Lymphocytes
likely play a key role, as suggested by clonal expansion of these cells
within the synovium and the requirement for lymphocytes in a
murine model of psoriatic arthritis
• Joint inflammation is accompanied by an exuberant vascular
expansion reminiscent of cutaneous psoriasis, with a
tendency to promote bone formation as well as injury to
cartilage and bone.
9.
10.
11. Laboratory tests are of limited diagnostic value in JPsA.
Inflammatory markers, including ESR and CRP, may exhibit mild to
moderate elevation, but are frequently normal.
Elevation of the platelet count has been noted in younger
patients.
ANA is found in low or moderate titer in 60% of younger patients
and 30% of older patients and is helpful primarily to define uveitis
risk for the purpose of ophthalmologic screening.
Antibodies against extractable nuclear antigens are usually
absent.
Rheumatoid factor (RF) is typically absent, and indeed its
presence excludes a diagnosis of JPsA under ILAR criteria
The presence of psoriasis may be considered incidental in
patients with symmetrical polyarthritis who are positive for RF or
anticyclical citrullinated peptide antibodies
Laboratory and radiology findings
12. Treatment
• Psoriatic synovitis is potentially destructive of cartilage and bone, and
like other types of synovitis may compromise bone growth in the
immature skeleton.
• The goal of therapy is therefore remission, with normalization of
physical findings and laboratory markers of inflammation.
• Nonsteroidal anti-inflammatory drugs (NSAIDs), sulfasalazine,
methotrexate, leflunomide, cyclosporine, and the anti- TNF agents.
• Individual large joints can be treated effectively with glucocorticoid
injection.
• In patients with involvement of multiple joints, disease-modifying
antirheumatic drugs (DMARDs) such as sulfasalazine or methotrexate
are indicated.
• TNF blockade is particularly useful when there is axial disease, since no
other treatment is effective for inflammation of the spine and sacroiliac
joints
Psoriatic synovitis
• Treatment of psoriatic spondylitis is based
primarily on experience with ankylosing
spondylitis.
• Treatment should be considered in
patients who experience axial symptoms or
show substantial or progressive limitation
of spinal mobility.
• Continuous treatment with NSAIDs results
in measurable radiographic improvement,
but the effect is small.
• Standard DMARDs, including sulfasalazine,
methotrexate and leflunomide, are of
minimal benefit.
• Anti-TNF therapy is highly effective for axial
disease as assessed both by symptoms and
by MRI evidence of inflammation.
• However, studies in adults have so far
failed to show a corresponding reduction
in radiographic progression.
Spondilitis
P. Rahman, J.T. Elder, Genetic epidemiology of psoriasis and psoriatic arthritis, Ann. Rheum. Dis. 64 (Suppl. 2) (2005) ii37–ii39. discussion ii40-41
13. Prognosis
The long-term outcome of children with JPsA is incompletely defined.
Patients followed at least 15 years demonstrated worse functional outcome than patients with
oligoarticular or polyarticular JIA, and 33% still required DMARD therapy.
Another study of patients with JPsA followed for at least 5 years demonstrated persistently active
disease in 70% and limitations of physical activity in one-third.
A more recent study with shorter follow-up documented achievement of clinical remission (on
medication) in approximately 60% in both younger and older children, although younger patients
required longer to achieve this endpoint.
Impaired visual function may also occur, especially if uveitis is not discovered promptly
B. Flato, G. Lien, A. Smerdel-Ramoya, et al., Juvenile psoriatic arthritis: longterm outcome and differentiation from other subtypes of juvenile idiopathic arthritis, J.
Rheumatol. 36 (2009) 642–650.
14. Ekelund M, AaltoK, Fasth A, HerlinT, Nielsen S, et all, Psoriasis and associated variables in classification and outcome of juvenile idiopathic arthritis - an eight-year follow-
up Study. Pediatric Rheumatology (2017) 15:13
15. Table 2 presents the development of clinical
features associated with psoriasis during the
first 8 years of disease. Twenty-four children
developed dactylitis during disease course, 15
of them had dactylitis at onset.
Median age at time of onset of JIA in the group
with dactylitis was 2.4 (IQR 1.7–5.4) years
compared with 5.8 (IQR 2.7–9.9) years in the
rest of the cohort (p = 0.007).
Ekelund M, AaltoK, Fasth A, HerlinT, Nielsen S, et all, Psoriasis and associated variables in classification and outcome of juvenile idiopathic arthritis - an eight-year follow-up
Study. Pediatric Rheumatology (2017) 15:13
16. Table 3 also presents the significantly higher cumulative number of active joints in children with psoriasis or
psoriasis-like rash as well as the increased frequency of dactylitis, nail pitting, enthesitis, and first-degree
heredity for psoriasis compared with children without psoriasis or psoriasis-like rash.