CURRICULUM VITAE

Nama : Sumadiono, dr SpA(K)

Tempat/ tanggal lahir : Nganjuk, 9-10-1956
Pekerjaan/jabatan : Staf Bagian Ilmu Kesehatan Anak FKUGM

Riwayat pendidikan formal :

Dokter umum : Fakultas kedokteran Unair, lulus tahun 1982
Spesialis anak : Fakultas kedokteran UGM, lulus tahun 1994
Konsultan Alergi Imunologi Anak: Ikatan Dokter Anak Indonesia 2002

Riwayat pendidikantambahan:
Molecular Biology (ICMR Kobe, Japan, 1996)
Basic Immunology (VU Amsterdam, The Netherland, 1997)
Pediatric clinic/treatment (VU Amsterdam, The Netherlands, 1998 & 1999)
Pediatric Allergy Immunology (Saskatoon, Canada, 2003)
Medical Education: PBL (UM Mastrich, The Netherlands, 2004)
Trends in Medical Education (NUS, Singapore, 2004)
Workshop: Intraarticular Injection (IRA, 2005)

Riwayat Organisasi:
 Gejala Dini
dan
Penatalaksanaan
Penyakit LUPUS
pada ANAK
 SUASANA

DATA dan PERMASALAHAN

PASIEN SLE/IMUN
di Dept IKA RSUP Dr SARDJITO
YOGYAKARTA
 Pasien Imun Dept IKA RSUP Sardjito

RUJUKAN NASIONAL:
- Jateng - Lampung
- Jatim -Pekan Baru
- Tangerang - Kupang
- Pontianak - Palangkaraya

REGISTRASI IMUN:
- EXEL, Tabel, Chart,
- WA GROUP: Data, Foto, Video
- 5/9/2018
Peta 4
5/9/2018 5
Pasien SLE di POLI ANAK RSUP Dr Sardjito, Maret 2015
Nefritis HSP Dermatitis
3%
Nefritis HSP 3%
Dermatitis
HSP Juvenile
3% 3%
2% dermatomyositi
JIA sistemik
s
3% SLE berat 2%
18% SLE berat
Rhinitis Rhinitis
alergi 18% HSP
alergi SLE berat
15% 2%HSP
Juvenile
15% SLE: 28%
Juvenile dermatomyositis
JIA sistemik dermatomyosi
JIA Oligoartikuler

3% SLE tis
JRA/JIA:
2%
Dermatitis atopik

21%
JIA poliartikuler
Alergi JIA

Oligoartikuler
JIA sistemik
HSP: 5%
Rhinitis alergi
Alergi Alergi Nefritis 10%
HSP

23% 23% SLE

Dermatitis
SLE
10% JIA
10%
Oligoartikuler
JIA
JIA Dermatitis
Dermatitis 10%
poliartikuler poliartikuler atopik atopik
8% 8% 3% 3%
Pasien SLE di BANGSAL ANAK RSUP
Dr Sardjito, Maret 2015

SLE: 86%
Nefritis lupus
JRA/JIA:
29%
14% Nefritis lupus
JIA oligoartikuler
SLE berat
SLE berat
57%

JIA
oligoartikuler
14%
Maret 2018
Maret 2015

SLE: 40%
SLE: 28%
JRA/JIA:
28% JRA/JIA:
21%
HSP: 14%
HSP: 5%
 Maret 2015
Maret 2018
SLE: 86%
SLE: 45%
JRA/JIA:
JRA/JIA: 14%
45%
 PROGNOSTIC FACTOR AND SURVIVAL OF SYSTEMIC LUPUS
ERYTHEMATOSUS IN SARDJITO GENERAL HOSPITAL, YOGYAKARTA
2006-2011
Muslikhah Yuni Farkhati , Cahya Dewi Satria, Sunartini Hapsara

 Total: 26 childhood SLE .... sd 2018

 3 (11%): male and 23 (87%): female. L:P = 2: 8
Mortalitas: 20-25%?
 The median age at diagnosis: 11,9 years
  11 (42,31%) were dead, 3 (11,5%): dropped out
  12 (46,3%) survived
 One, 3 and 5 years’ survival rate: 75%, 55% and 50 %

 Anti-dsDNA antibody (p= 0,04), leukopenia (p=0,03) and

neurologic involvement (p=0,03)  worse survival
 Protective effect of interaction between high dose
corticosteroid and IVIG therapy (HR 0,23 p=0,04) .
 Permasalahan Pasien SLE Anak
 Diagnosis:
Data tdk selalu lengkap pada saat datang...perlu follow up
 Management:
Obat: IvIg tdk bisa BPJS?
Kemajuan dalam Managemenet Komprehensif
 Permasalahan khusus:
Tumbuh kembang anak terganggu
Transportasi
Rumah tinggal
Gangguan Psikologis/psikiatri
Gangguan mobilitas/gerakan, rehabilitasi medik
Home visit......sangat komunikatif dan suportif
Diagnosis and Management

LUPUS
In
Children

Sumadiono
Department of Pediatric Fac. of Medicine Gadjah Mada University
Yogyakarta
Systemic Lupus Erythematosus (SLE)
adalah
- Penyakit autoimun
- Kronis, multisistem
- Aktivitas peradangan vaskuler & jar. ikat.

Prevalensi:
- Antara 4 - 250 kasus/100.000 anak
- Di USA: 17,7/100.000 anak.
- 85%: Perempuan. Laki-2 15%
- Nefritis Lupus: 37%
- Di Taiwan: 6,3/100.000 anak. Perempuan 6,2 kali > laki-laki
- Di Asia: 31/100.000.
SLE  menyerupai penyakit lain
(bila malar rash tidak dijumpai)
Diagnosis SLE: tantangan tersendiri

SLE pada anak

- Klinis > berat dibanding pada dewasa
- >>: Ginjal, Neurologi/Ensefalopati, Anemia hemolitik
- 30% progres Renal Insufisiensi
Glomerulonephritis – at least 75%

Penanganan SLE anak:

Mempertimbangkan pengaruh terapi terhadap
perkembangan fisik dan psikologis anak.
 Musculoskeletal Disease

 Incidence: 76%
 Arthralgias
 Arthritis
 Non-erosive
 Involves small joints of the hands, wrists, elbows,
shoulders, knees, ankles

 Myalgias/ muscle weakness

 Usually proximal
Presentasi dr Cahya pada Kongress SIOP ASIA di Yogyakarta

Penyakit yang menyerupai

SLE & Leukemia
 Organomegali

 Arthritis

 Sitopenia

 Demam lama

Nyeri sendi berpindah2  Leukemia  BMP

Penyakit yang menyerupai

Demam Reumatik Akut & SLE

 Polyartritis
 Karditis
 Demam Lama Bagaimana
 KED tinggi Kriteria ARA
 Anemia
Diagnostic criteria of SLE (ARA, 1982, modified in 1997)
1. Malar rash (or: vespertilio, butterfly rash)
2. Discoid rash
3. Photosensitivity If 4 criteria
4. Oral ulcers: oral or nasopharyngeal ulceration are present serially
5. Arthritis: nonerosive athritis or simultaneously,
6. Serositis: (at least one of the following) the diagnosis = SLE
a) pleuritis
b) pericarditis
7. Renal disorder (at least one of the following):
a) persistent proteinuria (>0.5g/day or 3+)
b) cellular casts (ery, hemoglobin, granular, tubular or mixed)
8. Neurological disorder (at least one of the following):
a) seizures
b) psychosis
9. Hematologic disorder (at least one of the following):
a) hemolytic anemia (with reticulocytosis), or
b) leukopenia (<4.0 on 2 or more occasions), or
c) lymphopenia (<1.5 on 2 or more occasions), or
d) thrombocytopenia (<100 in the absence of offending drug)
10. Immunologic disorder (at least one of the following):
a) abnormal titer anti-dsDNA antibody, or
b) antibody to Sm nuclear antigen, or
c) abnormal titer of anticardiolipin antibody
11. ANA positivity
Gambaran Klasik

Frequency: 76%

Malar Rash
(Butterfly Appearance)
Mucous membane ulcers
 - AIHA
- Reynaud
syndrome
-Amputasi
jari
Gagal Jantung
Lupus Nefritis
Kelainan Kulit
Vaskultis?
 Pneumonia Incidence: 5-67%
Spesis
Malnutrisi berat
Atropi 2 kaki
Kelainan Jantung
 Frequency: 20-40%

NPSLE
(NeuroPsychyatric SLE)

SLE.mpg
Lupus Nefritis Tremor.mp4

Pneumonia
Tremor
 Laboratory Findings
 Cytopenias (anemia, thrombocytopenia,
leukopenia)
 Elevated ESR, CRP, Immunoglobulins
 Hypoalbuminemia
 Proteinuria; RBCs, casts in urine
 Decreased creatinine clearance

 Low complement levels (C3/ C4)

 Autoantibodies (ANA, APL, Coombs, anti-platelet
Ab, rheumotoid factor, etc.)
 Antinuclear Antibodies (ANA)

 Sensitive but not specific, 95-98% pts positive

 Against nuclear components of the cell

dsDNA:
 high specificity for lupus (over 80%)
 SLE - Treatment
 MODERATE DISEASE:
 Prednisone 1-2 mg/kg/day, NSAIDS,
Antimalarials, Low dose methotrexate,
Azathioprine, MMF

 SEVERE DISEASE: Severe, life-threatening

organ system involvement
 High dose corticosteroids (2-3 mg/kg/day
or pulse),
 Immunosuppressives (IV pulse
Cyclophosphamide), Plasmapheresis,
Anticoagulation where appropriate
 Initial case reports of IVIg therapy of SLE
manifestations.

 Hjortkjaer et al. [29] 1990 Pericarditis

 Maier et al. [30] 1990 Thrombocyto penia
 Tomer [31] 1992 Psychosis
 Lesprit et al. [32] 1996 Polyneuritis
 Aharon et al. [33] 1997 Myelofibrosis
 Généreau et al. [34]1999 Cutaneous lupus
 Sherer et al. [35] 1999 Cerebritis
 Sherer et al. [36] 1999 Myocarditis
 Meissner et al. [37]2000 Serositis
 Sherer et al. [38] 2000 Antiphospholipid syndrome
 Hoshi et al. [39] 2004 Pulmonary hemorrhage
 Current Standard Therapy

Moderate to Severe SLE Therapy

nephritis corticosteroids
vasculitis cyclophosphamide
pneumonitis azathioprine
CNS cyclosporine
hematologic IVIg
plasmapheresis ???
mycophenylate mofetil
 SLE, DVT (Deep Vein
Trombosis)
Status lokalis :
Bengkak di ekstremitas superior
sinistra disertai hematoma terutama
area intertrigo, tidak hangat, nyeri

Membaik dengan IvIG,

protokol Severe SLE
Gagal Ginjal, Kejang2
Membaik dg IvIg
Kejang2, SLE Berat
Membaik dg IvIg
Paguyuban Keluarga SLE & JRA
KOMPLIKASI

Steroid 
Moon Face
Asites, Alb <<
Lupus Nefritis
Anemia
Striae
Osteoporosis
Prognosis:

- unpredictable disease and may be fatal

- Kidney disease is one of the life threatening

complications of SLE

- Involvement of the blood: bleeding, pallor or

risk of infection

- Involvement of the brain is the most difficult

 Buku
Kisah Nyata
Penderita SLE
Buku Penderita
SLE