Spektrum klinis penyakit reumatik

autoimun

Gede Kambayana
Autoimun ???
 Hashimoto Thyroiditis
Organ specific Primary myxedema
Thyrotoxicosis
Pernicious Anemia
Autoimmune atrophic gastritis
Addison’s disease
Premature menopause (certain cases)
Male infertility (certain cases)
Myasthenia gravis
IDDM
Goodpasture’s syndrome
Pemphigus vulgaris The Spectrum of
Pemphigoid
Sympathetic ophtalmia
Phacogenic uveitis
Autoimmune
(Multiple sclerosis)
AIHA disease
ITP
Idiopathic leucopenia
Primary billiaris cirhosis
Spondiloatropaty
Ulcerative colitis
Sjogren syndrome
Rheumatoid Arthritis
Scleroderma
Vaskulitis
Poly/dermatomyositis
Kelly et al, Text book of
Non-organ specific Discoid lupus erythematosus Rheumatology, 2005
Systemic lupus erythematosus (SLE)
Fase-fase perkembangan autoimun patogenik
 The Impact of Chronic Systemic Inflammation

Cardiovascular Diseases: Diabetic (complication) Chronic Inflammatory

Atherosclerosis, CVD, Heart Cardiomyiopathy, Atherosclerosis, Disease:
Failure, Cardiomyopathy CKD, Retinopathy, Sepsis, IBD, COPD, RA, Psoriasis,
Neuropathy Pancreatitis

Metabolic Disorder
Cancer: MS system
Complication: Lung, kidney, gastric, colon, Osteoporosis, OA,
Fatty Liver, Heart Disease, T2DM, pancreatic, lymphoma Muscular dystrophy
CKD, Sleep Apnea
 Pemeriksaan Lab :
• DL, LED  leukopeni, anemia,
trombositopeni, lympopeni
• UL  protein uria, cash
• CRP
• C3,4 complement
• ANA IF, ANA profile
 PREDICTIVE AUTOANTIBODIES
DISEASE AUTOANTIBODIES
Type 1 DM Anti-GAD, IA-2, IAA
Rheumatoid arthritis RF (anti IgG), ACPAs/Anti CCP Ab
Systemic Lupus Erythematosus Anti-PL, Anti-Ro, Anti-La, Anti-Sm, Anti-nuclear,
Ribonucleoprotein, Anti-dsDNA, Anti-Histone, Anti HS,
Anti nucleosome, Anti Ribosomal p protein
SjÖgren disease Anti-Ro, Anti-La
Anti phospholipid syndrome Lupus anti-coagulant, Anti cardiolipin
Primary Biliary Cirrhosis Anti-gp120, Anti-PDC
Autoimmune hepatitis ANA
Crohn’s disease ASCA
Ulcerative colitis pANCA
Autoimmune Addison’s disease ACA (adrenal cortec Ab / anti 21 hydroxylase)
Autoimmune Thyroid disease Anti-TG, Anti-TPO
Pemphigus Anti-desmoglein-1

Harel M, Shoenfeld Y. Ann NY Sci Acad. 2006;1069:322-45; Bizzaro N. Autoimmun Rev. 2007; 6:325–33;
BizzaroN,TozzoliR,ShoenfeldY. Arthritis Rheum. 2007; 56:1736–44.; ScofieldRH..Lancet.2004;363:1544–6.
Systemic Lupus
Erythematosus
Non-scarring
alopecia Renal Hematological Acne form

Penyakit seribu wajah (SLE)

Nerves Dry eyes Liver Arthritis
THE SLE CRITERIA FOR DIAGNOSIS FROM TIME
TO TIME
ARA ARA ACR SLICC

57 ITEMS 30 ITEMS Add APS 600 SLE

14 ITEMS 11 ITEMS patients

4 OF 14 4 OF 11 4 OF 11 4 of 11 clinical items
ITEMS ITEMS ITEMS and 6 immunological
items
Sn 90% Sn 78% Sn 75% Sn 98%
Sp 98% Sp 87% Sp 95% Sp 91%

1971 1982 1997 2012

 Kapan curiga LUPUS??
• Wanita muda
• Gejaya konstitusional : mudah lelah, febris,
atritis, rambut rontok, sariawan berulang

Curiga :LUPUS
 SLICC 2012
SYSTEMIC LUPUS INTERNATIONAL COLLABORATING CLINICS

Clinical Criteria Imunologic Criteria

1. Acute cutaneous lupus
2. Chronic cutaneous lupus
3. Oral or nasal ulcer 1. ANA
4. Non-scarring allopecia 2. Anti-DNA
5. Arthritis 3. Anti-Sm
6. Serositis 4. Antiphospholipid Ab
7. Renal 5. Low complment (C3, C4, CH50)
8. Neurologic 6. Direct Coomb’s test (do not count in
9. Hemolytic anemia the presence of hemolytic anemia)
10. Leukopenia
11. Thrombocytopenia (<100,000/mm3)

Requirements:  4 criteria (at least 1 clinical and 1 laboratory criteria) OR biopsy proven
lupus nephritis with positive ANA or anti-DNA
Vaskulitis
 Fig 1.
Normal artery

Fig 2.
with inflammation
Diagnosis HSP
( Hanoch schonlein Purpura)
Penyakit Kawasaki
Wegener’s granulomatosis
Scleroderma
 Scleroderma

ACR

American Osteopathic College of Dermatology, Grand

Rounds
 Systemik Sclerosis
• CREST Syndrome
• Calcinosis
• Raynaud’s
• Esophageal
Dysmotility
• Sclerodactyly
• Telangiectasisa
ACR/EULAR CRITERIA
 Scleroderma Autoantibodies
Antigen ANA Frequency Clinical Organs Involved
Pattern Associations
Scl-70 Speckled 10-40 dcSSC Lung fibrosis
(topoisomerase 1)
RNA Polymerase III Speck/Nuc 4-25 dcSSC Renal,
Pulmonary HTN
Centromere Centromere 15-40 lcSSc, CREST Pulmonary HTN
Esophageal
U1-RNP Speckled 5-35 lcSSC, MCTD Muscle
U3 RNP (fibrillarin) Nucleolar 1-5 dcSSC, poor prognosis Muscle
Pulmonary HTN
PM-SCL Nucleolar 3-6 Overlap, mixed Muscle
Th/To Nucleolar 1-7 lcSSc Pulmonary HTN,
Lung fibrosis,
Small bowel
Anti U11/U12 Nucleolar 1-5 lcSSc & dcSSC Lung Fibrosis
Anti-Ku 1-3 Overlap Ssc Muscle, Joint,
SLE overlap
Adapted from: Nihtyanova SI, Denton CP. Nat Rev Rheumatol 2010; 6:112
RHEUMATOID ARTHRITIS
Sendi yang
sering
terserang
Keradangan sendi
(Artritis reumatoid)
THE 1987 REVISED CRITERIA FOR THE CLASSIFICATION OF
RHEUMATOID ARTHRITIS

CRITERIA OF RA DIAGNOSIS
1.Stiffening in the morning  1 jam
2. Arthritis  3 sendi
3. Arthritis joint of the hands
4. Artritis symetric
5. Rhuematoid Nodule
6. Rheumatoid Factor positif
7. Typical appearance of imaging test
Artritis Rematoid:  4 criteria above;
criteria 1-4  6 weeks
American College of Rheumatology
(ACR; formerly the American Rheumatism Association)
Perjalanan AR
SJOGREN’S SYNDROME
Gambar 2.Maurizio cutalo 9 th EULAR Postgraduate course in
Rhematology.Warsow-Poland 23-27 0ktober 2006
Schirmer test
･This is probably the only test available in the ED to strongly support
or refute suspicion of Sjogren syndrome.
･A test strip of number 41 Whatman filter paper is placed near the
lower conjunctival sac to measure tear formation.
･Healthy persons
wet 15 mm or more after 5 minutes. A positive test occurs when less
than 5 mm is wet after 5 minutes.
Frequency of extraglandular manifestations
In Primary Sjogren syndrome
Gambar 3..Maurizio cutalo 9 th EULAR Postgraduate course in
Rhematology.Warsow-Poland 23-27 0ktober 2006
DIAGNOSIS
 The family of Seronegative
Spondyloarthropathies (SSA)

AS=Ankylosing Spondylitis
IBD=Inflammatory Bowel Disease
ReA=Reactive Arthritis
(RD = Reiter’s Syndrome)
PsA=Psoriatic Arthritis
USpA=Undifferentiated SpA

Px/ Autoimun : kumpulan Px dg manifestasi berbeda dg bbrp

karakteristik yg sama (atritis perifer, uveitis dan sacroiliitis)
Deteksi dan Diagnosis Dini
Nyeri pinggang inflamasi ≥3
bulan (spondiloartritis aksial),
maupun artritis perifer yang
asimetris, dan/atau yang
predominan di ekstremitas
bawah (spondiloartritis perifer),
pada laki-laki < 45 tahun harus
dicurigai sebagai
spondiloartropati
 Atritis pd SpA
• Atritis asimetris
(Unilateral)
• Predominan
extremitas bawah
• Entesitis ditumit
• daktilitis
Gambran Ro Sacroilitis
The Assessment of SpondyloArthritis international Society
(ASAS), 2010

Pada pasien nyeri pinggang ≥ 3 bulan

Pada pasien dengan manifestasi perifer
(dengan/tanpa manifestasi perifer) dengan
saja
onset usia pasien < 45 tahun

Sakroiliitis pada HLA-B27 PLUS

pencitraaan PLUS ≥1 ≥2 gambaran SpA Artritis atau entesitis atau daktilitis
gambaran SpA yang lain PLUS

Gambaran SpA yang dimaksud: ≥1 gambaran SpA

• Nyeri pinggang inflamasi • Uveitis
• Artritis • Psoriasis
• Entesitis (tumit) • Penyakit Crohn/Colitis Ulseratif
• Uveitis • Infeksi yang mendahului
• Daktilitis • HLA-B27
• Sakroiliitis pada pencitraan
• Psoriasis
atau
• Penyakit Crohn/Colitis Ulseratif ≥2 gambaran SpA yang lain :
• Respon baik dengan OAINS • Artritis
• Riwayat keluarga dengan SpA • Entesitis
• HLA-B27 • Daktilitis
• Peningkatan kadar C-Reactive Protein (CRP) • Riwayat nyeri pinggang inflamasi
• Riwayat keluarga dengan SpA
Ankilosing Spondilitis

• SpA dengan prevalensi

tertinggi
• Tanda Awal : nyeri
pinggang inflamasi
(tumpul, berlahan,
menjalar kebokong)
• Keterbatasan gerak
lumbal
• Keterbatasan rotasi
cervical
Ankilosing Spondilitis

• Gambaran radiologis :
- sacroilitis
- bamboo spine

• Lab : marker inflamasi tdk

spesifik (tergantung aktivitas
penyakit)
• HLA B27 (px/ keturunan china)
 Dx/ Ankilosing Spondilitis, New York 1984
Kriteria Klinis
• Nyeri pinggang 3 bln, yg membaik dg aktifitas, & tdk membaik dg
istirahat
• Keterbatasan gerak vertebra lumbalis pada arah sagital dan frontal
• Pe↓ ekspansi rongga dada, jika dibandingkan umur dan jenis
kelamin yg sesuai

Kriteria Radiologis
Sakroiliitis bilateral grade 2 - 4 atau sakroiliitis unilateral grade 3 – 4

Ankilosing Spondilitis definitif

Didapatkan kriteria sakroiliitis ditambah dg salah satu kriteria klinis

Ket: Sakroilitis pada pencitraan adalah sakroilitis dengan grade 2-4 (unilateral) atau 3-4
(bilateral) pada radiografi berdasarkan kriteria modifikasi New York
(diadaptasi dari Sieper J, dkk. Ann Rheum Dis 2009;68:784–8)
 Artritis Psoriatik
• Atritis : >> poliatritis
• Keterlibatan
inflamasi spinal :
jarang
• Didahului gambaran
Psoriasis
• Distropi kuku
psoriatik
• Gambaran dactylitis
 Diagnosis Artritis Psoriatik
Atritis/nyeri pinggang/entesis inflamasi, ditambah 3 dari 5
hal berikut :

1. Bukti adanya Psoriasis

2. Distrofi kuku psoriasis
3. Faktor reumatoid negatif
4. Daktilitis
5. Gambaran radiologis spesifik

Sensitifitas 91,4% dan spesifisitas 98,7%

(diadaptasi dari Taylor W, dkk. Arthritis Rheum 2006;54(8):2665-73)

 Artritis Reaktif

Belum ada kriteria diagnosis

Diagnosis di buat berdasarkan

kumpulan gejala glinis, Lab, radiologis

1. Kriteria : terpenuhi kriteria SpA (ASAS 2010)

2. Dewasa muda
3. HLA B27 : 50 % positif
 Dx/ Atritis Reaktif
• Klinis : Oligoatritis dominan extremitas bawah Asimetris, kadang
disertai nyeri pinggang inflamasi, Uveitis
• Riwayat infeksi 1 – 4 minggu sebelumnya
• Spektrum kuman yang telah diketahui antara lain Chlamydia
trachomatis, Yersinia Enterocolitica, Yersinia
pseudotuberculosis, Campylobacter jejuni, Shigella fexneri,
dan Salmonella enteric
• Lab : LED dan CRP yg me ↑, analisa cairan sendi tanda infamasi,
namun hasil ini tidak spesifk.
• Gamb Radiologis yg sering : Sakroiliitis, periostitis, sindesmoft
non-marginal, erosi sendi & penyempitan celah sendi.
Klinis Enteropathic artritis

Manifestasi ekstraartikuler :
• Clubbing finger
• Uveitis
• Eritema nodosum
• Pioderma gangrenosum

Lab & Radiologi tdk spesifik

 Diagnosis Artritis Enteropati

Penegakan dx/ diawali dari diagnosis Infammatory

bowel disease (IBD) dg endoskopi dan biopsi, adanya
fakta penyakit Crohn atau kolitis ulseratif

• Tipe 1 : poliatritis  perjalanan akut, membaik

dalam beberapa minggu
• Tipe 2 : oligoatritis (MTP, lutut, tumit)  kronik
dan sering persisten/menetap

Keterlibatan tulang belakang : 10 -20%

Work up SS :
Wegener’s granulomatosis
• Systemic vascultis of medium and small arteries (e.g. venules and
arterioles).
• Produces granulomatosus inflammation of the respiratory tracts and
necrotizing, pauci immune glomerulonephritis.
• Most common cause of saddle nose defromity in USA (nose flattened
due to destruction of nasal septum by granulomatous inflammation).
• Almost all patients with WG has c-ANCA, but not vise versa.
• Current treatment of choice is cyclophosphamide.
• At least 2 out of 4 criteria yields sensitivity and specificity of 88 and 92%.
• nasal or oral inflammation ( oral ulcers or purulent / bloody nasal
discharge, may be painful )
• abnormal CXR showing nodules, infiltrates, cavities
• microscopic hematuria or RBC casts
• vessel biopsy shows granulomatous inflammation