Interactive Lecture of Clinical Immune Disorder
Systemic Lupus Erythematosus
Tjokorda Raka Putra
Divisi of Reumathology
Dept of Internal Medicine, Medical Faculty
Udayana University/ IGNG NgurahHospital
Denpasar
2022
Refrences
1. Harrison’s Principle of Internal Medicine
2. Buku Ajar Ilmu Penyakit dalam PAPDI
3. Primer on Rheumatic Diseases, Arthritis
Foundation
The Goal
Able to diagnose and manage
SLE
Strategy : Must know
the anatomy and physiology of joints
the etiophatogenesis
the phatology of Ds
Anatomy of joint
Systemic Lupus
Erytemathosus
(SLE)
Selft Assesment
To make the diagnosis dan its
managements
•To explain the difinition
•To explain the Etiopathogenecis
•To inform the clinical symptoms and signs
•To plan the supporting data
•To explain the criteria of diagnosis
•To perform the general management
SLE is a chronic-
progressive systemic
and multisystem
disease, that
indentification and
characterization of an
abnormal
autoantobodies
•Epidemiology
–Sex : female-to female ratio 9 : 1,
–Age : at any age group
Usually in scond and fourth decade
–Ethnic distribution :
All race, Family distributions
Etiologic factors of SLE
•No clear
•Sugested:
–Genetic factor,
•HLA DR-2 or HLA DR-3
•Difficiency of complement (C2, C3, and C$)
–Enviroment
•Infection : Slow virus asn trigger
–Hormonal
Abnormality in metabolism of estrogen and
hiperprolaktemia.
–Other factors
Exposure of ultraviolet,drugs, stress, etc
The Pathogenese of SLEGenetik Faktor Penyebab Virus ?
T Helper Limfosit B
Antibodi terhadap DNA
Nukleoprotein
Histon
Nuclear Ribonucleoprotein
Lain-lain dari bahan inti sel
T Supressor
Komplek imun di
seluruh organ
Pathology of SLE
•No histologic feature is pathognomonic in SLE
•The general features :
–Fibrinoid necrosis of blood vessels and connective tissue
–The hematoxylin body (LE cell) phenomenon
•Skin
–Epidermal thikening , lequefactive degr. Of basal layer,
infiltration lymphocyte
•Synovium of joint
–Fibrinous villous synovitis
•Kidney
–Glomerulunephritis (membraneus, mesangial, [proliferative,
etc)
•CNS
–Multifocal cerebral microinfark
Clinical Manifestation of SLE
•A multisyatem disease
•Manifestation of extra articular more dominnat
•General manifestation : fever, fatique, anorexia
•Cutaneous
–Facial erythema (Butterfly eruption), Fotosensitive
chronic discoid lessiondiskoid with central
atropy, depigmentation, alopecia with or without
sicatric (lupus hair).
–Ulcer on skin or mucous membrane, purpura and
ecchymosis
–Raynaud’s phenomenome
–Echymosis and peringual eritheme ,Livido
retikularis, the form of slight vaskulitis
•Musculoskletal system
–Slight arthritis in small or large joint, asymetris
The joints : proximal interphalangeal
joint,knee,wrist, elbow, etacarpophalangeal joint,
feet.
–Inflamatory myositis
–Osteonecrosis
Cardiovaskuler:
•Atherosclerotic cardio-vascular ds
•Pericarditis with or without effusion
(serositis)
•Myocarditis
•Endokarditis verucosa.
•Peripheral vascular manivestation
•Vaskulitis on small arteries,
•capiller on the skin
•Lung:
–Pleuritis with or without effusion
–pneumonitis
–Pulmonary Hemorrhage -Hemoptisis
•Other organ:
–Non specific abdominal pain, steril
peritonitis (serositis), pancreatitis,
hepatomegalim, splenomegali.
–Lymphadenopathy
–Conyungtivitis, episcleritis, retinal vasculitis
Diagnostic Investigations
•Laboratory Tests
–Anemia, in aktive phase
–Coombs test positive (haemolitic anemia ).
–Leucopenia in active phase, limpofenia (e.c.
Antilymphocyte Ab).
–Thrombocytopenia
–ESR or CRP is elevated
–False positive reaction to VDRL (test for
syphilis)
–Hypergammaglobulinemia
–Urinalysis and Kidney fuction
•Autoantibody test.
–ANA (Antinuclear Antibody)antibody to
nuclear component.
–Anti-ds-DNA, spesific for LES ( 40-50% )
For diagnositic and aktivities of diseases
–LE cell
–Complement, decrease in active phase
•Imaging Studies
To support the clinnical assesment
–Chest X-ray
–Joint X-ray
–”Body Scan”
–Ensefalogram,
–etc.
Citeria of DIAGNOSE
•Criteria of ARA (American Rheumatism Association) revised in
1982.
1. Erytema on face (“Butterfly Rash”)
2. Discoid Lupus
3. Fotosensitivitas
4. Ulcer on mouth or nasopharing.
5. Non Erosive Arthritis
6. Kidney abnormality:Proteinuria > 0,5 gm/24 jam, Sylinderuria
7. Pleuritis atau Pericarditis
8. Psychosis, seizure
9. Haematologic abnomelity :Haemolitic Aenemia, Lecopenia,
Limphonemia, Trombocytopenia
10.Immunologic abn: LE cell positive, Ab Anti-DNA, Ab Anti-Sm,
11.False positive VDRL tes.
12.ANA test is positip
•Diagnose of SLE : 4 or more of that criterias.
PROGNOSE
•5 ysr : 90%.
•Depend on the abnormal of organs
(Kidney or CNS)
Management
•Individual response
•Depend on the severity of disease and organ
damage.
•Active form , depend on :
•Aktivitas penyakit ditentukan adanya :
–Pathologic figure of organ :
Nephritis, cardiopulmoner pathologic, Skin rash ,
serositis , hematologic abnormality
–Sign of systemic inflamation
fever, fatique and decreasing of BW
–Immunologic abnormality positive of
ANA , Ab Anti-DNA , decreasing of complement.
Management
•Education
–Prevent from exposure of sun light.
–The use of drug , be carefull-allergic.
–Plan the time of Gravid and contraseption pill (use
the non hormonal contraception).
–Live stile and psycologis
–Prevent the infektion
–Diet, ?
–Monitor and controle regularly
•Phyisical activity and excercise.
Medicine
•NSAIDs
–Indications for fever, joint pain, maucle pain.
–Aspirin 500mg, po 3 x/d or
other NSAIDs
•Cortikosteroid : the most important.
•Antimalaria
•Imunosupresant
Indicationns of cortikosteroiddrug
•Dermal efflorecense steroid topical
•Atritis, that fails with NSAIDs, prednison 10-20 mg perhari
•Serositis (al. perikarditis) Prednison 20mg 3x/d.
•Fulminant pnemonitis : high dose cortikossteroid
•Haemolitic Aenemia Prednison 40-60mg/d.
•Immune Thrombocytopenia = haemolitic aenemia.
•Vasculitis
–Small vessel (hand): small dose of prednison (20mg/d).
–Medium or Large vessel : prednison 60mg/d
•Cerebrall lupus Prednison 20 mg 3 x/d.
•Peripheral nerve abn. : prednison 20 mg 3 x/d
•Lupus Nefritis)
–prednison 60 mg/d