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CURRICULUM VITAE
Name : Dr. Nanang Sukmana, SpPD-KAI
Sex : MalePlace of Birth : Pegaden (Subang, West Java, Indonesia)
Date of Birth : August, 3rd 1948
Marital Status : Married
Nationality : IndonesianResidence : Jl. Gambang No. 5 Kelapa Gading
Bangun Cipta Sarana Jakarta 14250
Education
University of Indonesia, Faculty of Medicine
Awarded the degree of Medical Doctor, 1974
University of Indonesia, Faculty of Medicine
Awarded the degree of Internal Medicine, 1987
University of Indonesia, Faculty of MedicineDe artment of Internal Medicine
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Systemic LupusEritematosus
Diagnosis dini dan tata laksana
Nanang Sukmana
Divisi Alergi Imunologi Klinik, Departemen IPD
FK Universitas Indonesia/ RS Dr Cipto Mangunkusumo
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Lupus eritematosus sistemik (LES)
Penyakit autoimun yang melibatkan berbagai organ
dengan manifestasi klinis yang bervariasi dari yang
ringan sampai berat . Pada keadaan awal, seringsekali sukar dikenal sebagai LES, karena
manifestasinya sering tidak terjadi bersamaan.
Sampai saat ini penyebab LES belum diketahui ada
dugaan faktor genetik, infeksi dan lingkungan ikut
berperan pada patofisiologi LES
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Introduction
Systemic Lupus Erythematosus (SLE) is
chronic, often life long, autoimmune disease
It can be mild to severe
Affects mostly women
Systemic is usedbecause the disease
can affect organsand tissue
throughout the body
Lupus is Latinfor wolf
Erythematosus isfrom Greek for red
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Wanita > laki-laki
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Genetic DefectsResearchers estimated that20-100
different genetic factors may be
involved in the alterations of the
immune systems
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Enviromental factors that may be relevant in thepathogenesis of systemic lupus erythematosusChemical/physical factors
Aromatic amines Hydrazines
Drugs (procainamide, hydralazine, chlorpromazine, isoniazid, phenytoin,
penicillamine)
Tobacco smoke
Hair dyes Ultraviolet light
Dietary factors
L-canavanine (alfalfa sprouts) High intake of saturated fats
Infectious agents
?Bacterial DNA/endotoxins ?Retroviruses
Hormones and environmental oestrogens
Hormonal replacement therapy, oral contraceptive pills
Prenatal exposure to oestrogens
Mok CC, Lau CS. Pathogenesis of Systemic Lupus Erythematosus. J. Clin Pathol 2003;56:481-490
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Gambaran klinis LES
LES
SSP
20%
Hepotomepali/
Splenomegali
20%
Sal cerna
18%
Paru
38%
Hematologi
50% Jantung
48%
Vaskulitis
Ginjal
50%
Limphadenopati12-50%
Kelelahan90%
Panas lama
80-82%
BB turun60%
Artritis/Artralgia
90%
Kulit
50-58%
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Constitutional Symptoms
Fatigue
Weight changes
Myalgia
Fever
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Weight changes
Decrease appetite
The side effects of medications
Gastrointestinal disease
Loss of excess fluid due to use of diuretic
medications
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Fever
Fever is seen in most patients with SLE
Fever active SLE or infection
Reflect central nervous or adverse effect of a drug
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Faktor pencetus/eksaserbasi
LES
Obat :
Keguguran
KehamilanTindakan
pembedahan
Infeksi
Sinar UV(320-400 nm)
ProcainamidHidralazin
Metildopa
CPZ
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The 1997 Revised Criteria for
the Classification of SLE
1.Malar rash
2.Discoid rash
3.Serositisa. Pericarditis
b. Pleuritis
4.Oral ulcer
5.Arthritis
6.Photosensitivity
(MD SOAP BRAIN)
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The 1997 Revised Criteria forthe Classification of SLE
7. Blood / Hematologic disordera. Hemolytic anemia ORb. Leukopenia (< 4000/ ml) ORc. Lymphopenia (
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Laboratorium
Blood
Low white blood counts Anemia
Low platelet counts
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www-medlib.med.utah.edu
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Cutaneous vasculitis rash in a patient with SLE
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Oral Ulcers
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Photosensitivity
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Discoid Lupus
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Discoid Lupus
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Erythematous Rash
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AUTOANTIBODIES PRECEDE DISEASE BY YEARS
Some Auto Ab
before Dx: 80%
ANA: 3 yrs (9)
Anti-Ro/La
Anti-DNA: 2 yrs (9)
Anti-PL: (7)
Anti-Sm: 1 yr (7)
Anti-RNP
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General information
To prevent a rapid loss of
muscle & stamina
Influenza and pneumococcalvaccines are safe
Should avoid during periods of
active disease
Exercise
Immunization
Pregnancy
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Diet and nutrition
Condition of Patients
Steroids, increase appetite weight gain
Hyperlipidemia Vitamins are rarely needed in people who eat a
balanced diet
Take 1000 to 1500 mg of calciumper day A supplement with 400 to 800 units of vitamin D
is recommended every day
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Dehidroepiandrosterone (DHEA)
DHEA are major circulating androgens
Use ofDHEA or DHEA sulfate supplements are
not recommended
Crosbie D, Black C, McIntyre L, et al. Dehydroepiandrosterone for systemic lupus erythematosus. CochraneDatabase Syst Rev 2007; CD 005114
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Lannys
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Lanny s
Laboratory aids in distinguishing pre-
eclampsia from lupus nephritis
LUPUS
NEPHRITIS
PRE-
ECLAMPSIA
Urinalysis proteinuria and/or an active
urine sediment (red andwhite cells and cellular
casts)
Proteinuria only
C3, anti
dsDNA
Low C3, increased anti ds
DNA
High C3
Sign of SLE activities Thrombocytopenia,elevated serum levels of
liver enzymes and uric
acid, and decreased
urinary excretion of
calcium
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Drugs In Pregnancy and Lactation
Pregnancy Lactation
NSAIDs Yes (avoid after 32 weeks) Yes
Antimalarials Yes Yes
Corticosteroids Yes Yes
Azathioprine Yes Yes?
Mycophenolate No No
Methotrexate No No
Cyclophosphamide No No
Anti-TNF No No
Warfarin No (with caution after first trimester) Yes
Heparin Yes Yes
AAS (low dose) Yes Yes
NSAIDS, non-steroidal anti-inflammatory drugs; AAS, aspirinLupus and Pregnancy : ten questions and some answers. Gruiz-Irastorza and MA Khamashta. Lupus (2008)17, 416-420
T f ifi
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Treatment of specific organ
involvement
NSAIDs
Antimalarials
Steroid and Immunosuppressive
Other options Stem cell transplantation(bone marrow transplantation)
Anti-B cell antibodies
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Treating mild SLE
Rashes
Fever
Arthritis
Headache
Pleurisy
Mild kidney involvement
Inflammation of the tissue
surrounding the heart
Cream and Sunbloks
NSAIDs
Antimalaria drugs
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Treating severe SLE
Suppress the immune factors, most often first with
corticosteroids and otherimmunosuppressant drugs
Hemolytic anemia
Low platelet count with an accompanying rash
(thrombocytopenia purpura)
Major involvement in the lungs or heart
Significant kidney damage
Acute inflammation of the small blood vessels
in the extremities or gastrointestinal tract
Severe central nervous system symptoms
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Is cancer risk increased?
The relation ofSLE to malignancy is unclear
because conflicting data have been reported
Bernatsky S, Clarke A, Ramsey-Goldman R. Malignancy and systemic lupus erythematosus. CurrRheumatol Rep 2002;4:351
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Prognostic factors
Poor prognostic factors for survival in SLE include: Renal disease
Hypertension
Male sex
Young age
Older age at presentation
Poor socioeconomic status
Black race (reflect low socioeconomic status)
Presence of antiphospholipid antibodies
Antiphospholipid syndrome
High overall disease activity
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AVN Femoral Head
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Causes of death
The major cause of death in the first few years of illness
is active disease (eg, CNS, renal or cardiovasculardisease) or infection due to immunosuppresion, while
late deaths are either caused by the illness (eg, end-
stage renal disease), treatment complications (including
infection and coronary disease)
Ward MM< Pyun E, Studenski S. Mortality risks associated with specific clinical manifestations of
SLE. Arch Intern Med 1996;157;1337
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Clinical Pearls
SLE is a systemic disease with the potential to affect any organsystem
The differential diagnosis ofa lupus flare mandates
consideration ofinfection, drug toxicities, or other etiologies
In the absence of data from randomized trials, use ofaggressivetreatment must be balanced against associated toxicity
SLE patients are at high riskof developing atherosclerotic
disease, osteoporosis, malignancy, diabetes mellitus and
hypertension (HTN); screening for and reduction of modifiablerisk factor are essential
Appropriate vaccinations are advisable
Antony Rosen. Mechanism of autoimmunity. Clinical Immunology Principles and Practice 3rd ed. 2008.
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Treating specific complication
The major complications of the disease must be
treated as separate problems,keeping in mind the specific aspect of SLE
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Treatment
Only three drugs are FDA-approved
Prednisone
Aspirin Hydrochloroquine
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Cytokines
Infections
Injuries
Tissue repair Blood clotting
Other aspects of healing
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Specific organ symptoms1
Joint pain and stiffness
Skin changes
Photosensitivity
Kidneys
Gastrointestinal tract
Pulmonary Pleurisy
Shortness of breath
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Treating specific complication
The major complications of the disease must be
treated as separate problems,keeping in mind the specific aspect of SLE
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Small Vessel
Vasculitis
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Frequency of symptoms of systemic lupus erythematosus1
Symptoms Percent at onset Percent at anytime
Fatigue 50 74-100
Fever 36 40-80+
Weight loss 21 44-60+
Arthritis or arthralgia 62-67 83-95
Skin 73 80-91
Butterfly rash Photosensitivity
Mucous membrane
lesion
Alopecia
Raynauds phenomenon
Purpura
Urticaria
28-3829
10-21
32
17-33
10
1
48-5441-60
27-52
18-71
22-71
15-34
4-8
Renal 16-38 15-34
Nephrosis 5 4-8
Von Feldt, JM, Postgrad Med 1995; 97:79
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Symptoms Percent at onset Percent at anytime
Gastrointestinal 18 38-44
Pulmonary 2-12 24-98
Pleurisy
Effusion
Pneumonia
17 30-45
24
29
Cardiac 15 20-46
Pericarditis
Murmurs
ECG changes
8 8-48
23
34-70
Lymphadenopathy 7-16 21-50
Splenomegaly 5 9-20
Hepatomegaly 2 7-25
Central nervous system 12-21 25-75
Functional
Psychosis
Convulsions
1
0,5
Most
5-52
2-20
Frequency of symptoms of systemic lupus erythematosus2
Von Feldt, JM, Postgrad Med 1995; 97:79
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Symptoms
Fatigue
Loss of appetite, nausea and
weight loss
Chest pain
Bruising
Menstrual irregularities
Thought and concentration
disturbances
Personality changes
Sleep disorders:
restless legs syndrome
sleep apnea
Dryness of the eyes and mouth
Brittle hair or hair loss
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b kli i
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Gambaran klinis LES
LES
SSP
20%
Hepotomepali/
Splenomegali
20%
Sal cerna
18%
Paru38%
Hematologi
50% Jantung
48%
Vaskulitis
Ginjal
50%
Limphadenopati12-50% Kelelahan90%
Panas lama
80-82%
BB turun
60%
Artritis/Artralgia
90%
Kulit
50-58%
/ i
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Faktor pencetus/eksaserbasi
LES
Obat :
Keguguran
KehamilanTindakan
pembedahan
Infeksi
Sinar UV(320-400 nm)
ProcainamidHidralazin
Metildopa
CPZ
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Lannys
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Laboratory aids in distinguishing pre-
eclampsia from lupus nephritis
LUPUS
NEPHRITIS
PRE-
ECLAMPSIA
urinalysis proteinuria and/or an activeurine sediment (red and white
cells and cellular casts)
Proteinuria only
C3, anti
dsDNA
Low C3, increased anti
ds DNA
High C3
Sign of SLE activities Thrombocytopenia,
elevated serum levels of
liver enzymes and uric
acid, and decreased
urinary excretion of
calcium
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The 1997 Revised Criteria forthe Classification of SLE
1.Malar rash
2.Discoid rash
3.Serositisa. Pericarditis
b. Pleuritis
4.Oral ulcer
5.Arthritis
6.Photosensitivity
(MD SOAP BRAIN)
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Steps in Pathogenesis of SLE
1. Genetic factors/immune dysfunction
2. Environmental/endogenous trigger
3. Inflammation
4. Development of Autoimmune
5. Accelerated of Antigen
6. Tissue Damage7. Clinical Disease
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Autoantibodies
Anti-ds DNA
Anti-Sm antibodies Anti-Ro (SSA) and Anti-La (SSB)
Antiphospholipid antibodies
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AUTOANTIBODIES PRECEDE
DISEASE BY YEARSSome Auto Ab
before Dx: 80%
ANA: 3 yrs (9)
Anti-Ro/La
Anti-DNA: 2 yrs (9)
Anti-PL: (7)
Anti-Sm: 1 yr (7)
Anti-RNP