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Sjögren Syndrome

and

Spondyloarthritis

Hui Lin

Department of Rheumatology

Sjögren Syndrome

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sicca syndrome

autoimmune exocrinopathy

autoimmune epithelitis

Sjögren Syndrome

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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

Sjögren Syndrome

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dry

mouth

dry

eyes

Rampant

tooth

parotid gland

enlargement

Sjögren Syndrome

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systemic chronic inflammatory disorder

lymphocytic infiltrates in exocrine organs

autoantibodies

Sjögren Syndrome

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CD3+

T cells

CD21+

B cells

Sjögren Syndrome

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homogeneous pattern speckled pattern

Pathogenesis of SS

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Pathogenesis of SS

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Clinical features

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Keratoconjunctivitis Sicca

Xerostomia

Involvement of other exocrine glands

Extraglandular manifestations

Extraglandular Manifestations

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Skin

Polyarthalgia

Airways and lung disease

Renal disease

Gastrointestinal disease

Neurologic abnormalities

Skin

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Dry skin

Eyelid dermatitis

Angular cheilitis

Annular erythema

Purpura

Urticarial vasculitis

Airways and lung disease Xerotrachea

Xerobronchitis

Nonspecific interstitial pneumonitis (NSIP)

Lymphocytic interstitial peumonitis(LIP)

Usual interstitial pneumonitis (UIP)

Bronchiolitis

Lymphoma

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NSIP UIPLIP

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Lymphocytic interstitial pneumonitis (LIP) in a 44-year-old woman

with primary Sjögren’s syndrome

Renal disease

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Tubular interstitial nephritis

Peritubular lymphocytic infiltrate and fibrosis

Type I renal tubular acidosis (RTA)

Hypokalemia, hyponatremia, hypocalcemia, periodic paralysis

Glomerulonephritis

Proteinuria

Gastrointestinal disease

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Dysphagia

Heartburn

Esophageal dysfunction

Chronic atrophic gastritis

Liver enzyme abnormalities

Neurologic abnormalities

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Central nervous system involvement

• Depression

• Cognitive disturbances

• Hemiparesis

• Movement disorders

• Cerebellar syndromes

• Transverse myelitis

• Neuromyelitis optica

Neurologic abnormalities

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Peripheral nervous system involvement

• motor neuropathy

• sensory neuropathy

• cranial neuropathies

• autonomic neuropathies

Lymphoma

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Non-Hodgkin’s lymphoma (NHL)

Mucosa-associated lymphoid tissue (MALT) lymphoma

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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.

Classification criteria for SS in 2002(AECG)

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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

Classification criteria for SS in 2002(AECG)

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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.

Classification criteria for SS in 2002(AECG)

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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

ACR classification criteria for SS in 2012

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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

treatment

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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. 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.

Spondyloarthritis

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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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Ankylosing spondylitis ria in 2002

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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

Pathogenesis

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Three different structures of

HLA-B27 and how they might

induce the processes of arthritis

systemic chronic inflammatory

disorder

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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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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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Spinal mobility-the schober test

Chest expansion

Enthesitis

Sacroiliitis

Posture-occiput to wall distance

Physical findings

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Elevated ESR

Elevated CRP

HLA-B27 positive

RF negative

Laboratory tests

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Imaging studies

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Imaging studies

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Imaging studies

Criteria for AS

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Criteria for AS

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Rheumatoid arthritis

Lubar intervertebral disc protrusion

Spinal trauma, infection, tumor

Diffuse Idiopathic Skeletal Hyperostosis(DISH)

Differential Diagnosis

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Treatment

Other Spondyloarthritis

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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

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!