SJGREN SYNDROME (2).pptx

7/29/2019 SJ GREN SYNDROME (2).pptx

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DR. FAMUREWA B.A.


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OUTLINE INTRODUCTION

EPIDEMIOLOGY PATHOPHYSIOLOGY

CLINICAL MANIFESTATION

DIFFERENTIAL DIAGNOSES

INVESTIGATIONS DIAGNOSIS

TREATMENT

COMPLICATIONS

CONCLUSION


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SJOGREN SYNDROMEINTRODUCTION

Sjogren (Shrgren) syndrome(SS) is a chronicautoimmune dsease that primarily affects the

lacrimal & salivary glands; and may have

multisystem extraglandular manifestations.

First described in 1933 by Dr. Henrik Sjogren

He introduced Keratoconjunctivitis Sicca(KCS) as a

component of the disease


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SJOGREN SYNDROME

SS is a triad of:

dry eyes (Keratoconjunctivitis Sicca),

dry mouth (xerostomia) &

arthritis.

Synonyms- Sjogren dx, Gougerot- Sjogrensyndrome.

2 forms- 1 &2 .


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SJOGREN SYNDROME

1 SS

is defined as autoimmune exocrinopathypredominantly characterised by lymphocytic

infiltration of the lacrimal & salivary glands resulting

in dry eyes & dry mouth in the absence ofassociated connective tissue disease.

Synonyms - Sicca syndrome


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SJOGREN SYNDROME2 SS

can be defined as dry eyes & dry mouth coexistingwith a CT disease.

Rheumatoid arthritis,

Systemic Lupus Erythematosus(SLE),

Polymyosits,

Scleroderma,

1 biliary cirrhosis (in descending order of frequency).


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SJOGREN SYNDROME

EPIDEMIOLOGY

Prevalence in US is about 2-10 million

UK about 1-3% of the population

No known geographic & race incidence

Age- middleage(4th-5th decade)

Sex- F>M(10:1)

It is commoner in peri & postmenopausal women.


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SJOGREN SYNDROMEPATHOPHYSIOLOGY

IMMUNOPATHOLOGY- 3 steps.

ENVIRONMENTAL TRIGGERS

PATHOLOGY


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SJOGREN SYNDROME

HISTOPATHOLOGY


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SJOGREN SYNDROMECLINICAL MANIFESTATION

Symptoms of ocular dryness:

itching, grittiness, foreign body or sandy sensation, eye

fatigue

Oral symptoms:

difficulty with speaking, eating(dry foods) & swallowing,

oral soreness, teeth decay, loss or altered taste, frequent

sipping of water.


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SJOGREN SYNDROME

CLINICAL MANIFESTATION

Oral signs:

Lipstick & tongue blade signs,

swollen salivary glands,

lack of salivary pooling@ floor of d mouth,

frothiness with lack of saliva from salivary gland ducts,

sticking of mucosa to dental mirror,

Characteristic tongue sign.


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SJOGREN SYNDROME*Dimunition of SL salivary pool

*Cheilosis,

*loss of glistening of the tongue and mucous membranes.

*tongue depressor may adhere/stick to mucosal surfaces

Lobulated,erythematous with total/partial depapillation.


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SJOGREN SYNDROME

CLINICAL MANIFESTATION contd

Other exocrine glands involvement:

Dry skin(Xerosis)-bruising, bleeding &purpura

Dry vagina- dyspareunia, pruritus,irritation

Dry throat & trachea(xerotrachea)

Dry nasal passages- hyposmia


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SJOGREN SYNDROME

EXTRAGLANDULAR MANIFESTATIONS:

Hashimoto thyroiditis/Graves dx Tubular interstitial nephritis/Renal tubular acidosis

1 biliary cirrhosis

Peripheral neuropathies Vasculitis- Purpura

NHL( glandular/extraglandular)

Atrophic gastritis

Interstitial lung dx

Raynaud phenomenom


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SJOGREN SYNDROMEDIFFERENTIAL DIAGNOSIS

Viral infection- HCV, HIV,HTLV-1, EBV Sarcoidosis

Glandular deposits in: Amyloidosis,

Haemochromatosis, & Lipoproteinaemia. Salivary gland lymphoma

Graft versus host dx

Chronic alcoholism/ uncontrolled DM

Anorexia nervosa/ Bulimia

Benign lymphoepithelial lesion(Mikuliczs dx )

S OG S O


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SJOGREN SYNDROMEINVESTIGATIONS

1. OCULAR:

Schirmer test

Rose bengal dye/ Lissamine green dye


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SJOGREN SYNDROME INVESTIGATIONS

INVESTIGATIONS contd

2. ORAL:

Sialometry

Sialography- snowstorm/ punctate sialectasis Scintigraphy

Labial salivary gland(LSG)biopsy-focal lymphocytic

sialadenitis


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SJOGREN SYNDROME

INVESTIGATIONS

SEROLOGY:

ESR

Serum autoantibodies- SS-A, SS-B

Rheumatoid factor


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AMERICAN- EUROPEAN DIAGNOSTIC CRITERIA(2002) FOR

SJOGREN SYNDROME

1. Ocular symptoms (at least one of the following):

daily, persistent, troublesome dry eyes for more than 3

months,

recurrent sensation of sand or gravel in eyes,

use of tear substitutes more than three time per day

2. Oral symptoms (at least one of the following symptoms):

daily feeling of dry mouth for more than three months, recurrent or persistently swollen salivary glands as

adult

need to drink liquids frequently to aid in swallowing dry

food.

AMERICAN EUROPEAN DIAGNOSTIC CRITERIA(2002)


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AMERICAN- EUROPEAN DIAGNOSTIC CRITERIA(2002)

FOR SJOGREN SYNDROME- CONTD

3. Ocular signs (at least one positive):

Schirmer test,

Rose Bengal test or other ocular dye test

4. Histopathology (positive biopsy of a salivary

gland):Focus score 1. Focus score= no of foci in

4mm sq area.

AMERICAN EUROPEAN DIAGNOSTIC CRITERIA(2002)


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AMERICAN- EUROPEAN DIAGNOSTIC CRITERIA(2002)

FOR SJOGREN SYNDROME- CONTD

5. Salivary gland involvement (positive results from at

least one of the following tests):

unstimulated whole salivary flow collection (< 1.5ml in

15 minutes);

parotid sialography showing diffuse sialectasia;

Salivary scintigraphy showing delayed uptake, reduced

concentration and delayed excretion of tracer

6. Presence of Anti-SSA and Anti-SSB


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SJOGREN SYNDROME

DIAGNOSIS

RULES OF DIAGNOSIS:

1 SS = any 4 of the 6 criteria is present as long as either

histopathology or serology is +ve

2 SS = presence of associated connective tissue dx witheither ocular or oral symptoms + any 2 of criteria ,

, & .

SJOGREN SYNDROME


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SJOGREN SYNDROME

TREATMENT

Goal= Symptomatic & prevention of long-termcomplications.

1. Rx of dry eyes:

Avoid conditions that worsen dry eyes

Tear supplements- artificial saliva e.g TearsAgain ,HypoTears

Secretagogues- Pilocapine(5mg tds),

- Cevimeline(30mg tds).

Cyclosporine 0.05 ophthalmic emulsion

Punctal occlusion


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SJOGREN SYNDROME

TREATMENT- contd2. Rx of dry mouth:

Frequent sipping of water

Chewing of sugar-free gum e.g Xylitol Secretagogues- Pilocarpine, Cevimeline

Saliva substitutes-artificial saliva e.g Orthana,

Oralbalance


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SJOGREN SYNDROME TREATMENT

TREATMENT- contd

Dental caries prevention: pt counselling on diet,OH

& fluoride supplement

Treatment of existing carious lesions

Prompt diagnosis & rx of oral candidiasis

Extraglandular manifestations- referral to medicalspecialists.


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SJOGREN SYNDROMECOMPLICATIONS

1. ORAL:

Oral Candidiasis

Dental caries,

ascending sialadenitis

major silivary gland enlargement- NHL


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SJOGREN SYNDROME

COMPLICATIONS

2. OCULAR:

Corneal abrasion & ulceration

Blepharitis Conjunctivitis

3.Malignant transformation- Lymphoma


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SJOGREN SYNDROME

CONCLUSION

SS requires multidisciplinary approach in its mgt to

improve pts QOL & long-term follow-up to prevent

complications.


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SJOGREN SYNDROME

SORRY FOR LISTENING TO A DRY SEMINAR THIS

MORNING.

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SJGREN SYNDROME (2).pptx

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