SJOGREN SYNDROME DR. FAMUREWA B.A.

OUTLINE
INTRODUCTION EPIDEMIOLOGY PATHOPHYSIOLOGY CLINICAL MANIFESTATION DIFFERENTIAL DIAGNOSES INVESTIGATIONS DIAGNOSIS TREATMENT COMPLICATIONS CONCLUSION

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INTRODUCTION Sjogren (Shrgren) syndrome(SS) is a chronic autoimmune 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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SS is a triad of:
dry eyes (Keratoconjunctivitis Sicca), dry mouth (xerostomia) & arthritis.

Synonyms- Sjogren dx, Gougerot- Sjogren syndrome. 2 forms- 1 &2 .

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1 SS is defined as autoimmune exocrinopathy predominantly characterised by lymphocytic infiltration of the lacrimal & salivary glands resulting in dry eyes & dry mouth in the absence of associated connective tissue disease. Synonyms - Sicca syndrome

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2 SS can be defined as dry eyes & dry mouth coexisting with a CT disease.
Rheumatoid arthritis, Systemic Lupus Erythematosus(SLE), Polymyosits, Scleroderma, 1 biliary cirrhosis (in descending order of frequency).

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EPIDEMIOLOGY Prevalence in US is about 2-10 million UK about 1-3% of the population No known geographic & race incidence Age- middle age(4th-5th decade) Sex- F>M(10:1) It is commoner in peri & postmenopausal women.

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PATHOPHYSIOLOGY IMMUNOPATHOLOGY- 3 steps. ENVIRONMENTAL TRIGGERS PATHOLOGY

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HISTOPATHOLOGY

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CLINICAL 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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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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*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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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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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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DIFFERENTIAL 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 )

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INVESTIGATIONS 1. OCULAR: Schirmer test Rose bengal dye/ Lissamine green dye

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INVESTIGATIONS contd 2. ORAL: Sialometry Sialography- snowstorm/ punctate sialectasis Scintigraphy Labial salivary gland(LSG)biopsy-focal lymphocytic sialadenitis

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INVESTIGATIONS

SEROLOGY: ESR Serum autoantibodies- SS-A, SS-B Rheumatoid factor

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) 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) 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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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 with either ocular or oral symptoms + any 2 of criteria , ,& .

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TREATMENT Goal= Symptomatic & prevention of long-term complications. 1. Rx of dry eyes: Avoid conditions that worsen dry eyes Tear supplements- artificial saliva e.g Tears Again ,HypoTears Secretagogues- Pilocapine(5mg tds), - Cevimeline(30mg tds). Cyclosporine 0.05 ophthalmic emulsion Punctal occlusion

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TREATMENT- contd 2. 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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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 medical specialists.

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COMPLICATIONS 1. ORAL: Oral Candidiasis Dental caries, ascending sialadenitis major silivary gland enlargement- NHL

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COMPLICATIONS 2. OCULAR: Corneal abrasion & ulceration Blepharitis Conjunctivitis 3.Malignant transformation- Lymphoma

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CONCLUSION SS requires multidisciplinary approach in its mgt to improve pts QOL & long-term follow-up to prevent complications.

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SORRY FOR LISTENING TO A DRY SEMINAR THIS MORNING.