SLE : Etiology, Pathogenesis,

Clinical Manifestations, and

Management
dr. Anwar Bet, Sp.PD
 Introduction
SLE
 Ferdinand Von Hebra (1848) : Lupus Wolf’s Bite describe malar
rash

 The prevalance : 36,7/100000, ratio women : men = 4 : 1

 More frequently in African American / Latin American

 SLE is chronic autoimun disease

 Clinically heterogenous

 Affects different organ’s system

 Characterized by the production of autoantibodies against self antigen that

form immune complex deposits
 Etiology
 Multifactorial disease

 Genetic/enviromental factors interact to modulate the final

phenotype
 Enviromental tigerring factors in SLE :
 Sunlight, Cigarette smoking, Infection

 Vaccine, Vitamin D deficiency, Exogenous oesterogen

 Conventional drugs, Biological agents, Pesticides, etc

 Pathogenesis
Hyperactive B cells Production of autoantibodies

Conjunction with impaired removal of apoptotic

cellular material Formation immune complexes

in microvasculature, the complexes inflamatory

reaction Tissue inflamation and damage SLE

 Signs and Symptoms
 Constitutional (eg. Fatigue, fever, arthalgia, weight
changes)
 Musculoskeletal (eg, arthalgia, arthropathy, myalgia, frank
arthritis, avascular necrosis)
 Dermatologic (eg, malar rash, photosensitivity, discoid
lupus)
 Renal (eg, acute or chronic renal failure, acute nephritic
disease)
 Neuropsychiatric (eg, seizure, psycosis)
 Pulmonary (eg, plurisy, pleural efusion, pneumonitis,
pulmonary hypertension, interstitial lung disease)
 Gastrointestinal ( eg, nausea, dyspepsia, abdominal
pain)
 Cardiac (eg, pericarditis, myocarditis)
 Hematologic ( eg, cytopenias such leukopenia,
lymphopenia, anemia, or thrombocytopenia)
 Laboratorium
Investigations in suspected systemic lupus erythematosus (SLE)
 Screening Laboratory test
o Erythrocyte sedimentation rate
o Blood count, differential blood count
o Creatinine
o Urinary status and sediment
o Antinuclear antibodies (ANA)( Hep-2 cell test with flourescence
pattern)
 Further laboratory test after positive screening
(particulary in case of positive ANA)
o Further differentiation of ANA (particulary anti-Sm,
-Ro/SSA,-La/SSB,U1RNP antibodies etc)
o Anti-dsDNA antibodies (ELISA, confirmation by
radioimmunoassay or immunolourence test with
Orithida lucilae)
o Complement C3, C4
o Antiphospolipid antibodies lupus anticoagulant
o Glomelular filtration rate 24-hour urine (if urine
protein positive),alternatively protein/creatinine ratio
in single urine sample, investigation for dysmorphic
erythrocytes in sediment
o Liver enzyme lactate dehydrogenase creatine kinase
in presence of muscular symptom
o Further laboratory test depending on clinical
symptom
o Screening for comorbidities
o Assassment of vaccination status
Organ-specific diagnostic as required
 Skin/oral mucous membrane
o Biopsy histology, immunoflourescence if indicated
 Joints
o Conventional X-ray
o Arthrosonography
o Magnetic resonance imaging (MRI)
 Muscle
o Creatine kinase
o Elecromyography
o MRI
o Muscle biopsy
 Kidney
o Sonography
o Renal Biopsy
 Lung/heart
o Chest X-ray
o Troracic high-resolition computed tomography
(HR-CT)
o Lung function test including diffusion capacity
o Bronchoalveolar lavage
o Transesophageal chocardigraphy
o Cardiac catheterization
o Cardiac MRI
o Myocardial scintigraphy
o Coronary angiongraphy
 Eye
o Funduscopy special investigation in patien on antimalarial
 Central and peripheral nervous system
o Electoenchephalography
o Primarily cranial MRI special MRI techniques if indicated
o Computed tomography
o Cerebrospinal fluid analysis
o Transcranial Doppler/angiography
o Neuropsyciatric examination
o Measurement of nerve coduction velocity
Criteria

Malar rash
Diagnosa
Definition
Fixed erythema, flat or raised, over the malar eminences, tending to spare the
nasolabial folds

Erythematous raised patches with adherent keratotic scaling and follicular

Discoid rash plugging;

atrophic scarring occurs in older lesions

Skin rash as a result of unusual reaction to sunlight, by patient history or

Photosensitivity physician

Observation

Oral ulcers Oral or nasopharyngeal ulceration, usually painless, observed by a physician

Non-erosive arthritis involving two or more peripheral joints, characterised by

Arthritis
tenderness, swelling or effusion
 a. Pleuritis:convincing history of pleuritic pain or rub heard by a physician
Or evidence of pleural effusion or
Serositis
b. Pericarditis: documented by ECG or rub or evidence of pericardial effusion

a. Persistent proteinuria >0.5 g per day or >3+ if quantitation is not performed or

Renal disorder
b. Cellularcasts : maybe red cell,haemoglobin,granulartubular,or mixed

a. Seizures:in the absence of offending drugs or known metabolic

 
Derangements (eg,uraemia, acidosis, or electrolyte imbalance) or
Neurological
 
disorder
b. Psychosis : in the absence of offending drugs or known metabolic derangements

(eg, uraemia, acidosis, or electrolyte imbalance)

a. Haemolytic anaemia with reticulocytosis,or

b. Leucopenia: <4000/mm3, or
Haematologic
c. Lymphopenia: <1500/mm3, or
disorder
d. Thrombocytopenia: <100 000/mm3 in the absence of offending drugs
 a. Anti-DNA:antibody to native DNA in abnormal titre ,or
b. Anti-Sm:presenceofantibodytoSmnuclearantigen,or
c. Positive finding of anti phospholipid anti bodies based on:
(1) an abnormalserum concentration of IgG or IgM anticardiolipin
antibodies,
Immunologic
(2) a positive test result for lupus anticoagulant using a standard method, or
disorder
(3) a false positive serologic test for syphilis known to be positive for at least
6
months and confirmed by Treponema pallidum immobilisation or
fluorescent
treponemal antibody absorption test

An abnormal titre of antinuclear antibody by immunofluorescence or an

equivalent assay
Antinuclear antibody
at any point in time and in the absence of drugs knownto be associated with

‘drug-induced lupus’ syndrome

 Penatalaksanaan
Secara garis besar penatalaksanaan SLE terdiri dari :

Edukasi dan Konseling :Penjelasan tentang penyakit lupus, perjalanan penyakit,

program pengobatan yamg direncanakan, komplikasi dan perlunya upaya
pencegahan termasuk paparan sinar matahari

Rehabilitasi : istirahat, terapi fisik, dll

Medikamentosa berdasarkan keterlibatan organ dan derajat aktifitas penyakit, yaitu :

SLE ringan : Parasetamol, oains, kortikosteroid topikal, klorokuin, kortikostroid oral

dosis rendah, dan tabir surya

SLE sedang : korrikosteroid dosis sedang-tinggi, imunosuprsan seperti azatioprin

dan mikofenolat mofetil

SLE berat : Kortikosteroid pulse dosis, siklofamid

 Kesimpulan
SLE penyakit autoimun sistemik dengan gejala klinis beragam/mengenai
berbagai organ dan sistem.

SLE diagnosa berdasarkan gejala klinis dan hasil pemeriksaan

laboratorium.

ACR merekomendasikan krteria untuk diagnosa SLE, ditegakkan jika

memenuhi minimal 4 dari 11 kriteria.

Terapi SLE berdasarkan berat ringannya penyakit dan organ yang terlibat.

IRA membuat rekomendasi untuk diagnosis dan pengelolaan SLE sebagai

panduan bagi seluruh lapisan dokter.
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