Sjögren Syndrome and Spondyloarthritisccftp.scu.edu.cn/Download/20180816210908159.pdf ·...
Transcript of Sjögren Syndrome and Spondyloarthritisccftp.scu.edu.cn/Download/20180816210908159.pdf ·...
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1
Sjögren Syndrome
and
Spondyloarthritis
Hui Lin
Department of Rheumatology
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Sjögren Syndrome
2
sicca syndrome
autoimmune exocrinopathy
autoimmune epithelitis
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Sjögren Syndrome
3
hypofunction of the salivary and lacrimal glands
multisystem autoimmune disease
Prevalence rate 0.1%~4.6%
Male/female 1/9
Peak ages 40~50 years old
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Sjögren Syndrome
4
dry
mouth
dry
eyes
Rampant
tooth
parotid gland
enlargement
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Sjögren Syndrome
6
systemic chronic inflammatory disorder
lymphocytic infiltrates in exocrine organs
autoantibodies
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Sjögren Syndrome
7
CD3+
T cells
CD21+
B cells
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Sjögren Syndrome
8
homogeneous pattern speckled pattern
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Pathogenesis of SS
9
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Pathogenesis of SS
10
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Clinical features
11
Keratoconjunctivitis Sicca
Xerostomia
Involvement of other exocrine glands
Extraglandular manifestations
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Extraglandular Manifestations
12
Skin
Polyarthalgia
Airways and lung disease
Renal disease
Gastrointestinal disease
Neurologic abnormalities
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Skin
13
Dry skin
Eyelid dermatitis
Angular cheilitis
Annular erythema
Purpura
Urticarial vasculitis
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Airways and lung disease Xerotrachea
Xerobronchitis
Nonspecific interstitial pneumonitis (NSIP)
Lymphocytic interstitial peumonitis(LIP)
Usual interstitial pneumonitis (UIP)
Bronchiolitis
Lymphoma
14
NSIP UIPLIP
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15
Lymphocytic interstitial pneumonitis (LIP) in a 44-year-old woman
with primary Sjögren’s syndrome
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Renal disease
16
Tubular interstitial nephritis
Peritubular lymphocytic infiltrate and fibrosis
Type I renal tubular acidosis (RTA)
Hypokalemia, hyponatremia, hypocalcemia, periodic paralysis
Glomerulonephritis
Proteinuria
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Gastrointestinal disease
17
Dysphagia
Heartburn
Esophageal dysfunction
Chronic atrophic gastritis
Liver enzyme abnormalities
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Neurologic abnormalities
18
Central nervous system involvement
• Depression
• Cognitive disturbances
• Hemiparesis
• Movement disorders
• Cerebellar syndromes
• Transverse myelitis
• Neuromyelitis optica
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Neurologic abnormalities
19
Peripheral nervous system involvement
• motor neuropathy
• sensory neuropathy
• cranial neuropathies
• autonomic neuropathies
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Lymphoma
20
Non-Hodgkin’s lymphoma (NHL)
Mucosa-associated lymphoid tissue (MALT) lymphoma
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21
Clinical photograph and photomicrograph of a 48-year-old man with
Sjögren syndrome with a large left parotid mass. On biopsy, B-cell
lymphoma of mucosa-associated lymphoid tissue (MALT) type was
identified. Microscopic section of parotid biopsy, stained with
immunoperoxidase for kappa light chains (brown-stained cells), showed
monoclonal population of B cells, confirming the diagnosis.
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Classification criteria for SS in 2002(AECG)
22
I. Ocular symptoms –
1. Dry eyes for more than 3 months
2. a recurrent sensation of sand or gravel in the eyes
3. use of tear substitutes more than 3 times daily
II. Oral symptoms –
1. Feeling of dry mouth for more than 3 months
2. recurrently swollen salivary glands
3. frequent use of liquids to aid swallowing dry food
III. Ocular signs –
1. Schirmer test performed without anesthesia (≤ 5 mm in 5 min)
2. positive cornea dye staining results (≥4 according to van Bijsterveld’s scoring )
IV. Positive minor salivary gland biopsy findings: at least 1 focus(which contain more
than 50 lymphocytes) /4 mm 2 in labial salivary gland biopsy samples
V. Oral signs –
1. Unstimulated whole salivary flow (≤1.5 mL in 15 minutes)
2. abnormal parotid sialography findings
3. Abnormal salivary scintigraphy findings
VI. Autoantibodies: Antibodies to Ro(SSA) or La(SSB) antigens, or both
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Classification criteria for SS in 2002(AECG)
23
For Primary SS
In patients without any potentially associated disease, primary SS may be defined as
follows:
a. The presence of any 4 of the 6 items is indicative of primary SS, as long as either
item IV (Histopathology) or VI (Serology) is positive.
b. Any 3 of the 4 objective criteria items (i.e., items III, IV, V, and VI) are present.
For Secondary SS
In patients with a potentially associated disease (for instance, another well-defined
connective tissue disease), the presence of item I or item II plus any 2 from among items
III, IV, and V may be considered as indicative of secondary SS.
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Classification criteria for SS in 2002(AECG)
24
Exclusion criteria include any of the following
Past head and neck radiation treatment
Hepatitis C virus infection
Acquired immunodeficiency syndrome (AIDS)
Pre-existing lymphoma
Sarcoidosis
Graft-versus-host disease
Use of anticholinergic drugs
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ACR classification criteria for SS in 2012
25
I. Positive serum anti-SSA (Ro) and/or anti-
SSB (La) or [positive RF and ANA ≥1:320]
II. OSS ≥ 3 (using lissamine green and
fluorescein) to diagnose KCS
III. Presence of FLS in a LSG biopsy with a FS
≥ 1 focus/4mm 2
at least 2 out of 3 of the following objective tests
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treatment
26
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27
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ACRACR classification criteria in 2002Referencesssification criteria in 2002
28
1. Kelley’s Textbook of Rheumatology. Ninth edition.
2. Shiboski SC, Shiboski CH, Criswell L, et al. American College of
Rheumatology classification criteria for Sjögren's syndrome: a data-driven,
expert consensus approach in the Sjögren's International Collaborative
Clinical Alliance cohort. Arthritis Care Res (Hoboken). 2012;64(4):475-87.
3. Ramos-Casals M, Brito-Zerón P, Sisó-Almirall A, Bosch X. Primary
Sjögren's syndrome. BMJ. 2012;344:e3821.
4. Ramos-Casals M, Brito-Zerón P, Sisó-Almirall A, et al. Topical and systemic
medications for the treatment of primary Sjögren's syndrome. Nat Rev
Rheumatol 2012;8(7):399-411.
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Spondyloarthritis
29
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30
AS
ReA
IBDA
PsA
USpA
Uveitis
JCA
JSPA
Peripheral arthritis: predominantly of
lower limb,asymmetric
Radiographic sacroiliitis
Enthesitis
Absence of rheumatoid factor
Extra-articular features: anterior
uveitis
Significant familial aggregation
Association with HLA-B27
Spondyloarthritision criteria in 2002
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31
Ankylosing spondylitis ria in 2002
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32
Axial spondyloarthritis
Peripheral arthritis
Spinal ankylosis
Prevalence rate in China 0.25%
The incidence of male more
common
Peak ages 20~30 years old
Associations with HLA-B27
Ankylosing spondylitis criteria in 2002
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Pathogenesis
33
Three different structures of
HLA-B27 and how they might
induce the processes of arthritis
systemic chronic inflammatory
disorder
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34
Low back pain and stiffness
different from mechanical back pain
• onset of complaints before age 45
• duration of symptoms more than 3 months (chronic pain)
• improvement with exercises
• no improvement of back pain with rest
• improvement with use of nonsteroidal agents
Tenderness-enthesitis
Arthritis-lower limb, asymmetrical
Clinical manifestations-Skeletal Manifestations
criteria in 2002
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35
Acute anterior uveitis or iridocyclitis
Ascending aortitis, aortic valve incompetence, conduction abnormalities
Fibrosis of the upper lobes of the lungs
IgA nephropathy, Amyloidosis
Osteoporosis
Clinical manifestations-Extraskeletal Manifestations
criteria in 2002
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36
Spinal mobility-the schober test
Chest expansion
Enthesitis
Sacroiliitis
Posture-occiput to wall distance
Physical findings
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37
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38
Elevated ESR
Elevated CRP
HLA-B27 positive
RF negative
Laboratory tests
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39
Imaging studies
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40
Imaging studies
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41
Imaging studies
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Criteria for AS
42
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Criteria for AS
43
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44
Rheumatoid arthritis
Lubar intervertebral disc protrusion
Spinal trauma, infection, tumor
Diffuse Idiopathic Skeletal Hyperostosis(DISH)
Differential Diagnosis
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45
Treatment
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Other Spondyloarthritis
46
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50
ASAS classification criteria for AxialSpondyloarthritis (SpA)
Patients with Back Pain ≥ 3 Months and Age at onset < 45 years
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ASAS classification criteria forPeripheral Spondyloarthritis
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ACRACR classification criteria in 2002Referencesssification criteria in 2002
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1. Kelley’s Textbook of Rheumatology. Ninth edition.
2. Rudwaleit M et al. Ann Rheum Dis. 2009;68:777-783.
3. van Tubergen A. Nat Rev Rheumatol. 2015; 11:110-118.
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Thanks!