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ERITEMATUS
Yuliasih
Sub departemen rematologi penyakit dalam
RSUD dr Soetomo Universitas Airlangga
Definisi
Lupus :
o penyakit autoimun
o akut dan kronik
o multisystem
o klinis ada beberapa bentuk
systemic (SLE),
discoid (DLE, scarring rash only),
drug-induced (DILE),
neonatal (NLE).
Systemic Lupus Erythematosus
General
autoimmune multisystem disease
prevalence 1 in 2,000
9 to 1; female to male (1 in 700)
peak age 15-25
immune complex deposition
photosensitive skin eruptions, serositis,
pneumonitis, myocarditis, nephritis, CNS
involvement
Angka kejadian
Genetik faktor
Lingkungan : infeksi ,
Sinar matahari, stress sel T& B autoreaktif
pembentukan autoantibodi
yang berlebihan
1. aktivasi komplemen,
2. sistem koagulasi,
3. sitokine
pembentukan imun komplek
Kerusakan organ
AUTOIMUNE ? antibodi gagal
mengenali sel tubuh sendiri
Gambaran Klasik :
wanita muda ,
demam ,
Tidak semua penderita
buterfly rash, memberi gambaran klinis jelas ,
kadang hanya 1 atau dua simtom
lymphadenopaty , berbulan bulan, baru
kemudian timbul gejala lain
nyeri sendi, Yang lebih jelas
alopecia,
efusi pleura,
edema +protein uria
Gejala non spesifik
Pada awalnya bisanya Hasil penelitian : penderita SLE
didahului dengan gejala yg dirawat inap di dr Soetomo
april sep 2003 (98 penderita)
yg non spesifik
odemam
omual Berat badan 67.3
turun
oMuntah
Cepat lelah 84.7
oNafsu makan turun
demam 53
oBerat badan turun
oCepat lelah
Manifestasi Sistem
Muskuloskeletal
Artralgia, Artritis
Myalgia, Myositis
Tenosinovitis
Jaccoud Arthritis
Osteonekrosis
SLE: Jacouds arthropathy
SLE can cause skin
problems
Manifesatasi sistem
mukokutaneus
Manifestasi yang sering dijumpai
Gejala klasik berbentuk malar rash
Gambaran kliniknya beragam
Dikelompokkan :
Kurataneus lupus akut:
sangat fotosensitive,
berlangsung beberapa/ hari minggu,
bila membaik menimbulkan bercak
hiperpigmentasi
Kutaneus lupus sub akut
ada dua bentuk
1. papulosqumosa
2.ruam anular eritema
/eritrema sentrifugum/psoriasis
Distribubusinya pada daerah yg terpapar
sinar matahari fotosensitive
Dihubungkan antibodi Ro
Ciri khasnya central area hiperpigmentasi
Bila sembuh tidak menimbulkan jaringan
parut
Acute Coetaneous Lupus Erythematosus
ACLE :
Localized
characterized by confluent symmetric
erythematic and edema centered over
the malar eminences The nasolabial
folds are typically spared. (malar rash ).
The forehead and V-area of the neck
can be similarly involved.
generalized
( photosensitive lupus dermatitis )
ACLE begins on the face as small, discrete
macular, or papular lesions, or both, that
later become confluent and hyperkeratosis
Typical DLE lesions occur most often on the face, scalp, ears, V-
area of the neck, and extensor aspects of the arms. Any area of
the face can be affected, including the eyebrows, eyelids, nose,
and lips.
Irreversible scarring alopecia resulting from permanent follicullar
destruction occurred in 34% of patients in one recent series.
SLE:
Interarticular
Dermatitis
PANNICULITIS
characterized by inflammatory lesions in the lower
dermis and subcutaneous tissue.
Vasculitis Sclerodactyly
Antiphospholipid syndrome Rheumatoid nodules
Raynaud phenomenon Erythema multiforme-
Alopecia like lesions
DLE lesions in the scalp
Telogen effluvium Cutaneous mucinosis
Lupus hair
Bullous lesions Pigmentary changes
Extension of liquefaction Miscellaneous
degeneration of acute Ro (SS-A) Acnetoderma
antibody-positive SLE lesions Mid-dermal elastolysis
Anti-type VII collagen antibodies Acanthosis nigricans
Bullae as the result of putative
immune complex vasculitis
Cutaneous Vasculitis
SCLERODACTYLY
Lupus Panikulitis/Profundus
Alopecia
1. Polyarthritis, :
metacarpophalangeal,
interphalangeal,
pergelangan tangan.
Non erosive
2. Constitutional symptoms :
malaise, fatigue, low grade fevers
(kadang high fever),
lymphadenopathy,
BB turun, nafsu makan turun
Mual, muntah
3. Mucocutaneous symptoms :
rash :khas malar rash ( bridge of the nose)
alopecia,
discoid rash, a
anular rash,
fotosensitive rash (shawl area),
extensor surfaces of the upper arms,
vasculitic lesions,
mucosal ulcers
hairline
the finger pulps and periungual areas.
4 Proteinuria
5 Hematologic abnormalities
(anemia, leukopenia, cytopenia )
6. Serositis:
Pleuropericardial pain is more frequent
Abdominal pain biasanya tidak terdiagnosis
secara radiologi maupuin endoskopi
7. Neuropsychiatric:
headache,
mood disorder, cognitive disorder,
psychosis, seizure,
stroke, meningitis,
movement disorder
.
GINJAL
o Keradangan pada ginjal menimbulkan
kebocoran (keluarnya protein).
1. Malar rash : Fixed erythema over malar areas, sparing nasolabial folds
2. Discoid rashErythematous : raised patches with keratotic scaling
and follicular plugging
3. PhotosensitivitySkin rash : after exposure to sunlight,
history or physical exam
4. Oral ulcersOral or nasopharyngeal, painless, by physical exam
5. Nonerosive arthritisTenderness, swelling,
effusion in 2 or more peripheral joints
6. Pleuritis or pericarditis Convincing history or physical exam or
ECG or other evidence
7. Renal disorder>0.5g protein/d or 3+ or cellular casts
8. Seizures, psychosisNot due to drugs, metabolic derangement, etc
9. Hematologic disorderHemolytic anemia or
leukopenia (<4000 twice) o
lymphopenia (<1500 twice) or trombocytopenia
10. Immunologic disorderAnti-dsDNA or anti-Sm or antiphospholipid antibodies
(anticardiolipin, lupus anticoagulant, or false positive test for syphilis)
11. Positive ANANot drug-induced
Systemic Lupus Erythematosus:
Diagnostic Criteria
diagnosis
The American College of Rheumatology
Classification Criteria for SLE ( 4 dari 11
kriteria terkelompokkan lupus )
1. Non Farmakologi
Stress, lelah, matahari
Farmakologi
1. NSAID
2. STEROID
3. Chloroquin
4Imunosupresan :
cyklopospamide,
azatioprine,
cyklosposrin
OBAT YANG DIGUNAKAN
Overlap diseases
rheumatoid arthritis, scleroderma,
dermatomyositis, Sjogren syndrome, or
systemic vasculitis.
SLE and pregnancy
SLE has been stable for more than 1 year.
Prednisone is no more than 10mg/d, and
cytotoxic drug has been stopped for more
than 6 moth.
SLE patients can plan to have a baby.