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SYSTEMIC LUPUS

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

> perbandingan 9:1, usia 15 - 45 tahun

kulit hitam > putih

Surve DI USA 1,500,000 penderita

trigger factors chronic inflammatory responses belum dikeyahui

SLE : hasil interkasi yang komplek antara genetik dan faktor


lingkungan

Faktor lingkungan : infeksi, antibiotics, ultraviolet light, extreme


stress, obat tertentu , hormones

Obat obat tertentu diduga meningkarkan aktivitas respon imun atau


menurunkan respon imun procainamide, hydrazaline,
Pathogenesis kegagalan memproses
imun respon

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

Akibatnya? timbulkan keradangan


didalam tubuh

Pada lupus keradangannya timbul bersifat


sistemik

Antibodi yang ditemukan: ANA, ds DNA,


anti Ro, anti La, & anti RNP.
Systemic Lupus Erythematosus
Mechanisms of autoantibody production in SLE and APS

Mechanisms in Rheumatology 2001


Mechanisms of renal damage by anti-DNA antibodies

Mechanisms in Rheumatology 2001


Gambaran klinis

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

- Generalized ACLE, a less common variety,


presents as a more widespread morbilliform
or exanthemata eruption

ACLE that can stimulate toxic epidermal


necrolysis

strong association between ACLE and


systemic LE activity,
ACLE :photosensitive , transient, lasting
only several days or weeks.
Post inflammatory pigmentary change is
most prominent in patients with LE with
darkly pigmented skin

ACLE lesions do not result in scarring.

Superficial ulceration of the oral or nasal mucosa is a


frequent accompaniment of ACLE
The posterior areas of the hard palate are most
commonly affected, however, the labial, gingival,
buccal, and lingual mucosa may also be involved. In
the early stages of such lesions,
the pathology is usually nonspecific;
SUBACUTE CUTANEOUS LUPUS ERYTHEMATOSUS

SCLE skin lesions including


symmetric erythema centrifugum,
disseminated DLE,
autoimmune annular erythema,
subacute disseminated in LE,
superficial disseminated LE,
psoriasiform LE, pityriasiform LE, and
maculopapular photosensitive LE

SCLE consists of non scarring papulosquamous or


annular skin lesions
LE-specific histopathology and occur in a
characteristic photo-distribution
a higher frequency of anti-Ro (SS-a) antibody
positivity
Bentuk ptiriasis Vesikulo anular
bulosa
papulosqumosa
SCLE lesions :
sun-exposed areas : upper back,
shoulders, extensor aspects of
the arms, V-area of the neck, and
less commonly, on the face

Infrequently, SCLE lesions present


initially with any appearance of erythema
multiforme
Lesions typically heal without
scarring
CHRONIC CUTANEOUS LUPUS ERYTHEMATOSUS

Classic Discoid Lupus Erythematosus

DLE lesion of this type begin as flat


or slightly elevated, well-
demarcated, red-purple macules or
papules with a scaly surface.
Early DLE lesions most commonly
evolve into larger, coin-shaped
(discoid) erythematous plaques
covered by a prominent, adherent
scale that extends into dilated hair
follicles
These discoid plaques can enlarge and
merge to form even larger, confluent,
disfiguring plaques

Involvement of hair follicles is a prominent


clinical feature of DLE lesions
Erythema and hyperpigmentation are present during the initial
phase of DLE lesions

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.

reversible, nonscarring alopecia that patients with SLE often


develop during periods of s stemic diasease activity.
DLE lesions that occur only on the
head or neck ( localized DLE.)

DLE lesions occuring both above and


below the neck are referred to as
generalized DLE.

Painful erosive palmar-plantar


DLE involvement can
predominate in some cases
producing significant disability
and presenting an especially
difficult management problem
DLE lesions can be precipitated
by sunlight exposure; however,
this occurs less frequently
than with ACLE and SCLE.
Dermatomyositis:
Gottrons Papules

SLE:
Interarticular
Dermatitis
PANNICULITIS
characterized by inflammatory lesions in the lower
dermis and subcutaneous tissue.

approximately 1% to 3% of patients with SLE have


this complication

Lupus panniculitis/profundus may occur in the absence


of systemic disease
The lesions present as deep, firm nodules, 1 to 3 cm in
diameter, often without overlying clinical change at
the surface of the skin
The head, proximal upper arms, chest, buttocks, and
thighs are the sites of predominant involvement
Table Nonspecific Cutaneous Manifestations of Systemic Lupus
Erythematosus (SLE)

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

Reportedly, vasculitic lesions occur in 20% to


70% of patients with SLE
The level and intensity of the inflammatory
insult of blood vessels in the skin determine
the morphologic features of cutaneous
vasculitis.
For example, vasculitis lesions involving
arterioles and veins high in the papillary
portion of the dermis produce erythema and
petechiae
Blood vessel involvement deep in the papillary dermis,
as well as in the reticular portion of the dermis, may
produce palpable and nonpalpable purpuric lesions or
urticaria-like lesions.
Intense vasculitic lesions in this area can result in
bullous formation or ulcerations
Splinter hemorrhages, cuticle nailfold injection
(periungual telangiectasia), and small infarcts can
also result from vasculitic lesions involving small
arterioles and venules in the hands
Nailfold erythema, telangiectasia, or both, have been
reported to occur in 10% to 15% of patients with
SLE.
erythematous, nontender lesions involving the thenar
and hyperthenar eminence (Janeway spots) can also
occur
Non specific manifestation

Urticaria vasculitis Plantar erythema Digital vasculitis

Periungual erythema Periungual erythem


Livido retikularis
Angioedema Angioedema
& ulcer vaskulitis

Digital vasculitis Atrophic of nail Nailfold capillary


RAYNAUDS PHENOMENON

Raynauds phenomenon is characterized by triphasic


color changes involving the fingers, toes, or both.
a vasospastic component characterized by blanching,
then by a purplish, cyanotic discoloration, followed by
a reperfusion erythema at rewarming associated
commonly with pain

The pathogenesis of Raynauds phenomenon is due to


severe vasospasm involving abnormal digital arteries
having intimal hyperplasia.
There is no correlation between the severity of the
Raynauds phenomenon and SLE disease activity
ALOPECIA
A third form of alopecia,
also transient, is
probably closely related
in causation to the
telogen effluvium, and is
termed lupus hair or
wooly hair

SCLERODACTYLY

Sclerodermatous changes are uncommon in SLE. However, in


recent years patients with U1RNP-positive lupus have been
detected to have sclerodermatous changes characterized
by sclerotic lesions and swollen puffy hands in addition to
Raynauds phenomenon
PIGMENTARY CHANGES

Postinflammatory hypo- and hyperpigmentation


at sites of previous cutaneous lesions is a
common finding in patients with LE
It should be noted that antimalarial therapy
has long been recognized as a cause of altered
pigmentation. This therapy can result in
grayness of the scalp hair, eyelashes,
eyebrows, and beard.
These pigmentary changes are reversible if
the antimalarial is stopped
hiperpigmentasi Oral ulcer
Manifestasi Sistem Mukokutaneus

3. Kutaneus Lupus Kronik

Discoid lesion Discoid lesion

Bentuk klasik : DLE


awalnya ruam berbentuk
bulat sebesar mata uang
koin,permukaan ada
kerak, sembuh
4. menimbulkan jaringan
parut,distribusi : telinga
leher, wajah , kulit
kepala
Arm And forearms
Violaceous eryhema ofent extends to
extensor aspect of deltoid area, upper
arms, forearms

Hand & wirst : periungual erytema,


cuticulre dystrophic apprearnce
Closed inspection with in light skinned
reveal dilated cappilary loops or
cuticular haemorrhage
Non pruritic hypperkeratotic eruption
accopanied by scaling, fisure,
hiperpigmentation mechanics hand
The finger especially at tip may
display the physical changes of
Raynauds phenomenon
Nailfold Capillary Exam

Normal Systemic Sclerosis

Juvenile Adult Dermatomyositis


Dermatomyositis
Digital vasculitis
Digital vaskulitis
Manifesatsi kulit lainnya
Non spesifik manifestation
Livido retikularis & Angioedema angioedema
ulcer vaskulitis

Lupus Panikulitis/Profundus
Alopecia

Lupus hair Reversible Non reversible


alopecia alopecia
Hasil penilitian : pasien yg dirawat inap di RSUD
dr soetomo : april sept 2006 (98 penderita)

Malar Rash 37.8 %


Discoid Rash 57.1 %
Maculopapulo Rash 29.6 %
Fotosensitif 28.6 %
Oral Ulcer 31.6 %
Manifestasi Non Spesifik 62 %
Alopecia 39.8 %
Raynauds Phenomenon 4.1 %
Manifestasi Paru
Pleuritis
Pnemonitis
Pulmonary haemorrhage
Emboli paru
Hipertensi pulmonal
Dysfungsi diafragma

Pleural efusi Pnemonitis Perdarahan paru Hipertensi pulmonal


Manifestasi
Jantung
Perikarditis
Myokarditis
Endokarditis
Penyakit koroner
Hipertensi
Gagal jantung
Kelainan konduksi
Manifestasi hematologi
Anemia : penyakit Anemia 21.4
kronik,AIHA Lekopenia 17.3
Leukopenia Lymfopenia 69.4
Lymphopenia
Trombopenia 45
Trombopenia
LED ,CRP LED
Lymfadenopathy < 25 8.2
splenomegali 25 50 15.3
51 - 75 6.1
Manifestasi Ginjal
Lupus nepritis
Manifestasi sistem gastrointestinal
Gambaran klinis :( gut Lupus)
nyeri perut,
mual/muntah,
oral ulcer, patofisiologi
disfagia, Vaskulitis gastrointestinal
gastritis, 50% penderita
diare,
obstipasi,
pseudo-obst intestinal.

Bisa merupakan Gejala / Ditemukan selama


perjalanan penyakit
sering dianggap sbg akut abdomen shg dilakukan
tindakan surgery/
BOF/LLD
ileus paralitik

Air fluid level


CT Scan
Abd:
dilatasi
gaster dan
dilatasi
traktus
Dilatasi intestinal
Dilatasi gaster intestinal
Disertai fecal material
Manifestasi sistem syaraf pusat
Berupa gejala neurologi dan psikiatri
Manifestasinya sangat luas
Gejala neurologi :
non spesifik : pusing, migrain
spesifik : central dan perifer

Nomenclature NPSLE ACR 1999 : 19


sindoma
Keluhan yang sering dijumpai

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).

o Menyebabkan tubuh kekurangan protein


tertentu (albumin) yang akhirnya
menimbulkan pembengkakan tubuh yang
biasanya dimulai dari muka, tungkai,
perut ( seluruh tubuh)

o Jika terlambat pengobatan, akan


menimbulkan kerusakan ginjal permanen.
OTAK & SISTEM SYARAF
Pusing
Sering lupa
Sulit berkonsentrasi
Gangguan penglihatan
Kejang
Stroke
Gangguan kepribadian / jiwa
8. laboratorium yang abnormal :
leukopenia,
lymphopenia ,
thrombocytopenia,
positive antinuclear antibody.

9 . Myelopathy, myocarditis, pulmonary hypertension,


acute lupus pneumonitis, pulmonary fibrosis,
lupus profundus, acute psychosis
jarang merupakan gejala awal SLE

Gejala-gejala tersebut bisa muncul bersamaan


atau muncul tunggal
sampai berbulan-bulan,
baru diikuti gejala lainnya.
Laboratorium

1. darah lengkap + LED ( hb,


leko,trombo,lymposit)
2. urine lengkap ( protein uria ,
hematuria, cast eritrosit, urobilin,
bilirubin )
3. kimia klinik : alb, transaminase, fs
ginjal
4. Esbach
5. serology : C3,C4, ANA, Ds DNA , anti
ro/la, antiphospolipid.
Systemic Lupus Erythematosus
specific labs -
native(Double
stranded) DNA, SM
antigen
lupus like reaction
LE cells
Kriteria ACR 1997

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 )

Kriteria ini bermanfaat untuk penelitian


bukan untuk diagnosis pasien secara individu

ACR Criteria tidak digunakan untuk


mengkonfirmasi atau mengeklude
diagnosis lupus

Lupus manifestasinya luas maka untuk


diagnosis harus tahu gambaran klinis lupus
Pengobatan

1. Non Farmakologi
Stress, lelah, matahari
Farmakologi
1. NSAID
2. STEROID
3. Chloroquin
4Imunosupresan :
cyklopospamide,
azatioprine,
cyklosposrin
OBAT YANG DIGUNAKAN

Anti nyeri & radang NSAID


Anti malaria: untuk mengontrol
kelelahan, nyeri sendi, & bercak dikulit
Cortikosteroid: hormon anti inflamasi
Cara kerja: secara cepat menekan
inflamasi. Digunakan dosis sekecil
mungkin sampai penyakit terkontrol.
Efek samping : DM, HT, infeksi Osteonecrosis,
Osteoporosis, & katarak.
Bahaya bila dilakukan penghentian yang mendadak
terutama setelah dosis tinggi.

Immuno supresive: Obat-obat yang digunakan untuk


menekan aktifitas penyakit lupus yang sangat
berat.
Misalnya: lupus yang mengenai otak, jantung, dan
ginjal.
Deferential diagnosis

mononucleosis, HIV, endocarditis,


streptococcal sequelae (rheumatic fever,
post-streptococcal glomerulonephritis)
Lymphomas, leukemias, and rarely
myxoma and

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.

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