CURRICULUM VITAE

Nama : dr. AGUS JOKO SUSANTO, SpPD, K-AI, FINASIM

Tempat/tanggal lahir : Wonogiri, 6 Desember 1973
Agama : Islam
Istri : dr. Dyah Rohmania Agustiana
Alamat kantor : Divisi Alergi Imunologi Klinik-KSM Ilmu Penyakit Dalam
RSUD dr. Moewardi/FK UNS Surakarta
Alamat rumah : Jl. Garuda 43 RT 1/RW 2 Triyagan, Mojolaban, Sukoharjo
Email : agusjoko.susanto4@gmail.com

RIWAYAT PENDIDIKAN

1. Dokter umum Tahun 1999 FK UNS Surakarta

2. Dokter Spesialis Penyakit Dalam Tahun 2010 FK UNS Surakarta
3. Dokter Spesialis Konsultan Tahun 2017 FK UI Jakarta
Alergi Imunologi Klinik

RIWAYAT PEKERJAAN

Tahun Nama Instansi Jabatan

 Riwayat Pekerjaan
Tahun Nama Instansi Jabatan

Tahun 1999-2000 RS Keluarga Ibu, Tangerang Dokter Umum

Tahun 2000-2003 Puskesmas Pagerbarang, Tegal Kepala Puskesmas
Tahun 2003-2005 RSI Muhammadiyah Dokter Umum
Jatibarang, Brebes
Tahun 2003-2005 Bapel JPKM Kabupaten Tegal Kepala Bapel JPKM
Tahun 2010-2012 Bagian IPD RSUP dr. Sardjito/ Staf Bagian IPD
FK UGM Yogyakarta
Tahun 2013-Sekarang SMF IPD RSUD dr. Moewardi/ Ketua Divisi Alergi
FK UNS Surakarta Imunologi Klinik
Tahun 2013-Sekarang Tim Pelaksana Uji Coba Kurikulum Anggota
Pendidikan Klinis FK UNS
Tahun 2014-2015 Program Studi Profesi Dokter FK UNS Sekretaris Prodi
Tahun 2014-2017 Tim Perawatan Paliatif RS Moewardi Anggota
Tahun 2017-2018 Tim Verifikator Internal RS Moewardi Anggota
Tahun 2018-sekarang Tim Akreditasi RS Moewardi Ketua Pokja ARK
Tahun 2018-sekarang RS Dr Moewardi Gedung Flamboyan Kepala Instalasi

Keanggotaan Mulai Tahun

EAACI Alergi Imunologi Eropa 2017-sekarang

PERALMUNI Indonesia 2015-sekarang
PERALMUNI Cabang Surakarta 2015-sekarang
FINASIM 2014-sekarang
PAPDI Cabang Surakarta 2010-sekarang
IDI Cabang Sukoharjo 2008-sekarang
 Immunodeficiency :
Primary and Secondary

AGUS JOKO SUSANTO

Allergy and Clinical Immunology Division

Internal Department-Moewardi Hospital/Sebelas Maret University
 IMMUNE SYSTEM

AGUS JOKO S, 2018

 Schematic representation of
the human immune system IMMUNE
Ann Nutr Metab 2007;51:301-323 SYSTEM

INNATE ADAPTIVE
IMMUNITY IMMUNITY

Cell
Other non- Complement Humoral
Phagocytosis mediated
specific factors system immunity
immunity

Epithelial barrier
Neutrophilic T lymphocytes
and digestive
granulocyte
functions
Antigen-
NK cells, acute Eosinophilic presenting
phase proteins granulocyte

AGUS JOKO S, 2018

Monocyte/
Enzymes,
Macrophage,
transferrin, etc
Dendritic cells
 TNF-, IFN- Lymphokine mediated cytotoxicity
IFN-
TGF-
T IL-2 Complement
Helper 1 B cell IgG2a mediated
cell IFN- cytotoxicity
IFN- IFN-, TNF-
FCR
Macrophage
Antibody dependent
Cell mediated cytotoxicity
Phagocytosis
Naïve Intracellular killing
CD4+
T Cell IFN-, TNF-
DTH
IL-4
IL-10 IL-4
Eosinophil Mediator Release
IL-10 progenitor
Eosinophil

IL-5
T B cell
Helper 2 IgM, IgG1 Antibody
IL-4 cell IL-4

Stimulatory IgE
IL-3,IL-4
Mast Mast
Inhibitory IL-10 cell
Mediator Release
cell

Differentiation Cross regulation Effector Function AGUS JOKO S, 2018

IMMUNODEFICIENCY

AGUS JOKO S, 2019

 Immunodeficiency :

- Primary
- Secondary
Primary Immunodeficiency
(PID)
Primary Immunodeficiency

• Primary immunodeficiency diseases (PIDs) are a group of

disorders caused by basic defects in immune function of
the cells and proteins of the immune system.

• There are more than 350 types of PIDs. Some are relatively
common, while others are quite rare. Some affect a single
cell or protein of the immune system and others may affect
two or more components of the immune system.
 Classification

The International Union of

Immunological Societies
(IUIS)
 Primary Immunodeficiency
I. Immunodeficiencies affecting cellular and humoral immunity
II. Mixed Immunodeficiencies with related syndrome
III. Predominantly antibody deficiencies
a. Hypogammglobulinemia
b. Other Antibody deficiencies
IV. Diseasea of immune dysregulation
a. Hemophagocytic Lymphohistiocytosis HLH and EBV susceptibility
b. Sd with Autoimmunity and Others
V. Congenital defects of phagocyte number, function, or both.
a. Neutropenia
b. Functional defects
VI. Defects in intrinsic and innate immunity
a. Bacterial and Parasitic infections
b. MSMD and Viral infection
VII. Auto-inflammatory disorders
VIII. Complement deficiencies
 Secondary
Immunodeficiency
 Secondary Immunodeficiency

1. Surgical manipulation (Lymphoid organs,

Bursa Fabricius / Thymus)

2. Ionizing Radiation

Radiation damages DNA

3. Chemical & biologic Immunosuppressive agents

AGUS JOKO S, 2018

 4. Corticosteroids ( Immuno Suppressive )

a. Decreasing the binding of immune

complexes to Fc and C3b receptors of phagocytic cells.
b. Depressing bacterial activity, antigen –
processing activity and IL1 production by macrophage
c. Depressing the release various lymphokines
5. Malignancies
6. Infections
a. Measles and other certain other viral diseases
b. HIV
c. TBC
AGUS JOKO S, 2018
 7. Malnutrition
Decreased Food Intake

Lethargy and Apathy

Decreased Motor Activity

Further Decreased Food Intake

AGUS JOKO S, 2018

 8. Chemical

Anesthesia
Opiate
Alcohol

AGUS JOKO S, 2018

Therapy PID
• For antibody deficiencies, the options range from a) monitoring
(asymptomatic SIgAD) b) prophylactic antibiotics (symptomatic
SIgAD) to c) immunoglobulin replacement therapy
(Agammaglobulinemia, CVID, and SAD).

• Immunosuppressants (for example: hydroxychloroquine, infliximab,

and a combination of azathioprine and Rituximab) are used for
treatment of granulomatous lymphocytic interstitial lung disease
(GLILD) associated with CVID. High mortality was observed in the
clinical trials of CVID patients who were treated with hemopoetic
stem cell transplantation (HSCT); though benefit was noted in the
surviving patients suggesting a need for further studies.
• For severe combined immunodeficiency, the definitive treatment
options include enzyme replacement (e.g. PEG-ADA), gene therapy,
and HSCT.
• Gene therapy with retroviral vectors was previously attempted but
was reported to increase the risk for development of leukemia and
myelodysplasia. Recent studies have shown some promise for gene
therapy using lentivirus vectors.
• HSCT outcomes depend on several factors like the age at the time of
HSCT, active infections, type of donor (matched/ mismatched,
related/ unrelated, bone marrow/ cord blood), and conditioning
regimen.
• Phagocytic defects are managed with prophylactic antimicrobials and
interferon gamma infusions. Some disorders with neutropenia can be
treated with granulocyte- colony stimulating factor (G-CSF).

• Some well-defined syndromes such as Wiskott Aldrich Syndrome

(WAS), hyper IgM syndrome caused by CD40 and CD40L defects,
DOCK8 defect, IPEX, HLH, XLP, and, CGD can also be treated with
HSCT. Gene therapy has been attempted for X-linked SCID, ADA
deficiency, WAS, and CGD.
• Chromosome 22q11.2 deletion syndrome (OMIM# 188400) and CHARGE
Syndrome (CHD7 (OMIM# 608892), SEMA3E (OMIM# 214800)) with
profound T cell immunodeficiency have shown to be treated with thymic
transplantation in a clinical trial.

• Auto inflammatory disorders can be treated depending on the

etiopathogenesis.

• Corticosteroids can be used for some periodic fevers syndromes.

• IL1 receptor antagonists such as anakinra can be used in inflammasome
associated periodic fevers.
• HSCT has been used for IL-10 defects causing early onset inflammatory
bowel disease.
 Immunoglobulin
Replacement Therapy
• Immunoglobulin (Ig), also known as gamma globulin or
immune globulin, refers to the component of blood plasma that
contains immunoglobulins or antibodies.

• These antibodies are used in the body to neutralize bacteria

and viruses. They are large, Y-shaped proteins produced by
specialized lymphocytes called plasma cells.

• Only the IgG is purified from the plasma to produce therapeutic

Ig products, so Ig used for treatment contains 95- 98% pure
IgG with small amounts of other plasma proteins including
some IgA.
• Human immunoglobulin was originally used as
antibody replacement therapy in primary and
secondary antibody deficiencies.

• Ig replacement therapy is generally administered

either intravenously (abbreviated IVIG), or
subcutaneously (abbreviated SCIG).
• Ig replacement therapy is typically dosed based on the
patient’s weight. Many factors, however, are considered
when the medication is prescribed. Typically, a starting
dose is between 400-600mg/kg/month.

• Doses are adjusted to clinical effect, with the

expectation of minimizing the frequency and severity of
recurrent infections while minimizing side effects of the
medication.
Side effects IVIG to report include but
are not limited to :
• Headache
• Body aches
• Fever/chills
• Diarrhea
• Muscle cramps
• Nausea and vomiting
• Symptoms of infection
Prognosis
- Etiology
- Infection and antibiotic
- BMT
- HSCT
 REAKSI HIPERSENSITIFITAS (ASID)
• Reaksi tipe I : A nafilaksis dengan Antibodi Ig E
• Reaksi tipe II : S itolitik atau sitotoksik dengan
Antibodi Ig M/Ig G
• Reaksi tipe III : Reaksi I mun komplek dengan
Antibodi Ig M /Ig G
• Reaksi tipe IV : Cell-mediated immunity atau
D elayed type (reaksi tipe lambat)
AGUS JOKO S, 2019
TYPE I REACTION
AGUS JOKO S, 2019
Pathophysiology of an allergic reaction
DUST MITE

HISTAMINE
 Antigen
IgE
Allergic Inflamation Respons Fc-
CLINICAL EFFECTS

Acute phase Late phase

- Bronchus contraction asthma Infiltration of eosinophil and neutrophil

- Perifer vasodilatation
- Capillary permeability increase

Mucous secretion increase Eritema, fluid transudation

relative Hipovolemik
Cell Infiltration of mononucleosis
(macrophage, fibroblast)
Urtikaria, Pruritus
Tissue damage
Shock
 Genetik
Lingkungan
Alergen APC Th2 Th1

APC
Genetik
Lingkungan
Th2 Th1
IL-4
IL-13 IL-3 IL-4
IL-5 IL-4
IL-4 IL-13
Sel B IL-13
IL-9
IL-13

Sel mast Basofil

Eosinofil Sel B
IgE Sirkulasi (mukosa/
jaringan)

Uji IgE Reaksi Sirkulasi IgE

spesifik jaringan
serum

Derajat Uji IgE

Paparan pertama keparahan spesifik
serum
Paparan kedua dan selanjutnya
Agus Joko S. Acta Medica; 2017
TYPE II REACTION
AGUS JOKO S, 2019
TYPE III REACTION
AGUS JOKO S, 2019
TYPE IV REACTION
AGUS JOKO S, 2019
AUTOIMMUNE
DISEASES
 I. CLASSICAL SYSTEMATIC AUTOIMMUNE DISEASES
- Systemic Lupus Erythematosus - Mixed Connective Tissue Disease
- Antiphospholipid Syndrome - Relapsing Polychondrisis
- Rheumatoid Arthritis - Raynaud Phenomenon
- Macrophage Activation Syndrome in - Drug-Induced Autoimmunity
- Juvenile Idiopathic Arthritis - The Multiple Autoimmune Syndrome
- Adult Still Disease

- Systemic Sclerosis

- Sjogren Syndrome

AGUS JOKO S, 2019

 II. SYSTEMIC VASCULITIS
- Giant Cell Arteritis
- Polymyalgia Rheumatica
- Cogan Syndrome
- Henoch-Shoenlein Purpura
- Cryoglobulinemic Syndrome
- Hypersensitivity Angitis
- Takayasu Arteritis
- Polyarteritis Nodosa
- Microscopic Polyangitis
AGUS JOKO S, 2019
- Wagener Granulomatosis
- Churg-Strauss Syndrome
- Buerger Disease
- Behcet Disease
- Susac Syndrome
- Goodpasture Disease
- Kawasaki Disease
 III. IDIOPATHIC INFLAMMATORY MYOPATHIES

- Polymyositis

- Dermatomyositis

- Clinically Amyopathic Dermatomyositis

- Sporadic Inclusion Body Myositis

- Antisynthetase Syndrome

- Granulomatous Myositis

- Eosinophilic Myositis

- Eosinophilic Fasciitis

AGUS JOKO S, 2019

 IV. OTHER IMMUNE-MEDIATED SYSTEMIC DISEASES

- Sarcoidosis

- Spondyloarthropathies

- Periodic Fever Syndrome

- Fibromyalgia Syndrome

AGUS JOKO S, 2019

 V. ENDOCRINE AND REPRODUCTIVE AUTOIMMUNE DISEASE

- Hashimoto Thyroiditis - Autoimmune Parathyroid Disease

- Atrophic Thyroiditis - Autoimmune Polyendocrine Syndrome

- Subacute Thyroiditis - Endometriosis

- Graves Disease - Autoimune Ovarian Failure

- Postpartum Thyroiditis - Autoimmune Orchitis

- Autoimmune Diabetes Mellitus

- Autoimmune Addison Disease or

- Autoimmune Adrenalitis

- Autoimmune Hypophysis
AGUS JOKO S, 2019
 VI. HEPATOBILIARY AND PANCREATIC AUTOIMMUNE DISEASE

- Autoimmune Hepatitis

- Primary Biliary Cirrhosis

- Primary Sclerosing Chalangitis

- Extrahepatic Manifestations in Patient with Cirrhosis Hep C Virus Infx

- Autoimmune Pancreatitis

AGUS JOKO S, 2019

 VII. GASTROINSTESTINAL AUTOIMMUNE DISEASES

- Autoimmune Gastritis

- Ulcerative Colitis

- Inflammatory Bowel Disease

- Celiac Disease

AGUS JOKO S, 2019

 VIII. CUTANEOUS AUTOIMMUNE DISEASES

- Cutaneous Lupus Erythematosus

- Pemphigus and Bullous Pemphigoid

- Vitiligo

- Psoriatic Arthropathy

AGUS JOKO S, 2019

 IX. CARDIOVASCULAR AND PULMONARY AUTOIMMUNE DISEASES

- Autoimmune Dilated Cardiomyopathy

- Rheumatic Fever

- Accelerated Atherosclerosis in Autoimmune Diseases

- Idiopathic Interstitial Pneumonitis

- Pulmonary Arterial Hypertension

AGUS JOKO S, 2019

 X. NEUROLOGICAL AUTOIMMUNE DISEASES
- Multiple Sclerosis

- Myasthenia Gravis

- Lambert-Eaton Myasthenic Syndrome

- Amyotropic Lateral Sclerosis

- Paraneoplastic Neurological Syndrome

- Guillain-Barre and Other Immune-Mediated Neuropathies

- Neuromyelitis Optica (Devic Syndrome)

- Central Nervous System Vasculitis

- Autoimmune Epilepsy
AGUS JOKO S, 2019 - Autoimmune Chorea and Autoimmune Sensorineural Hearing Loss
 XI. OCULAR AUTOIMMUNE DISEASES

- Autoimmune Retinopathies

- Autoimmune Uveitis

- Vogt-Koyanagi-Harada Disease

- Orbital Myositis

AGUS JOKO S, 2019

 XII. RENAL AUTOIMMUNE DISEASES

- IgA Nephropathy

- Membranous Nephropathy

- Minimal Change Nephropathy

- Focal and Segmental Glomerulosclerosis

- Membranoproliferative or Mesangiocapillary Glomerulonephritis

AGUS JOKO S, 2019

 XIII. HEMATOLOGIC AUTOIMMUNE DISEASES
- Autoimmune Hemolytic Anemia

- Pernicious Anemia

- Idiopathic Aplastic Anemia

- Acquired Adult Pure Red Cell Aplasia

- Myelodysplastic Syndrome

- Autoimmune Lymphopenia

- Autoimmune Neutropenia

- Autoimmune Thrombocytopenic Purpura

- Trombotic Thrombocytopenic Purpura

AGUS JOKO S, 2019 - Heparin-Induced Thrombocytopenia and Autoimmune Coagulopathies
THANK YOU