Penyakit Autoimmune

Dr. E. Henny Herningtyas, MSi. PhD

Clinical Pathologi Department,
Faculty of Medicine, UGM Yogyakarta
henny_herningtyas@yahoo.com
Yogyakarta, 25 April 2012

4/25/2012

Kursus imunologi Dasar ke-11

c
c

Mackay IR, 2000, BMJ 321: 93-96

Central tolerance: role of AIRE

Thymus

AIRE (+)

AIRE (-)

Negative
selection

AIRE = autoimmune regulator, turn on many peripheral gene on thymus

= self Ag-expressed
thymus medullary cell

= self-reactive- T cell

= non-self Ag-expressed
thymus medullary cell

= non-reactive T-cell

= apoptotic T-cell

Peripheral Tolerance

Regulatory tolerance by T regulatory

Autoreactive T cell

Nave T cell

T regulatory

Autoreactive T cell
Thymus

Periphery

Cytokine release

Clonal deletion B-cell in germinal center

Nave B cell

B cell clones
self antigen

Autoimmune diseases and mechanism

Requirement for autoimmunity

Genetic factors

Infection and environmental

exposure

Breaking tolerance

Autoimmunity

HLA typing and risk of disease

Faktor genetik DM tipe 1

HLA DR3/4 increase susceptibility of

disease in type 1 DM
Autoimmunity is more
common in female

Shared HLA in sibling

increase susceptibility of
disease in type 1 DM

Single gene and autoimmunity

Infection induced autoimmunity

Breaking of antigen segregation

Sympathetic
opthalmia

Mechanism of autoimmune

Cluster of
autoimmune
diseases

Prevalence of autoimmune diseases

Rheumatoid arthritis: 1% of general population,

but female excess
SLE: 0.12% of general population, but female
excess
Myasthenia gravis: 0.0% of general population,
but female excess
Multiple sclerosis: 0.1% of general population but
female excess
Primary Sjogren Syndrome: 0.6-3% of adult
women
Thyroid disease: >3% of adult women
Type 1 diabetes: 0.1% of children
Mackay IR, 2000, BMJ 321: 93-96

RA prevalence globally
Country
C.Spain
Minnesota
UK
N.Spain
Sweden
France
Tudela Spain
N.Greece

Total
1.6%
1.07%
0.81%
0.7%
0.51%
0.5%
0.37%
0.35%

Country
Cordoba
Oslo
Greece
N. Italy
France
Indonesia
Yugoslavia
Nigeria

Total
0.34%
0.33%
0.32%
0.32%
0.3%
0.25%
0.19%
0%

Prevalence in developing country is much lower than develop country

Darmawan, 1993, British Journal of Rheumatology, 32: 537-540

Systemic Lupus Erythematosus (SLE)

Autoantibody-mediated inflamation can lead to release of autoantigens from

damaged tissue and promotes further activation o f autoreactive B cells

Chronic stage

Systemic Lupus Erythematosus (SLE)

Epitope spreading: B cells specific for various component of a complex
antigen are stimulated by a single specific autoreactive T cell

Rheumatoid Arthritis (RA)

Myasthenia gravis

Autoimmune Haemolytic Anemia (AIHA)

AIHA cases in Yogyakarta

Perempuan

14

Warm type

12

Cold type

Laki-laki
35.53%

Mix type

10
8

64.47%

6
4
2
0
1997

1998

1999

2000

2001

2002

Year

Mean age of AIHA cases in Yogyakarta

50

Mean

40

Patient age

Case number

AIHA cases in Yogyakarta

Male : female ratio = 1: 1.8

30
20
10
0
1997

1998

1999

2000

2001

2002

Year

Herningtyas EH, 2003, Research reports (unpublished data)

Take home message:

Autoimmunity is a breaking of selftolerance condition, influenced by genetic
factor and environmental factor
Various mechanisms on autoimmune
disease involving immune system at the
level of T cell, B-cell and autoAb
formation
Autoimmune disease prevalence vary in
different type of disease and different
countries

Terima kasih atas perhatian yang

anda berikan