Seminar G Sjorgens Syndrome Flashcards Preview

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Flashcards in Seminar G Sjorgens Syndrome Deck (24):
1

Definition

Chronic autoimmune disease characterised by lymphocytic infiltrates and destruction of epithelial exocrine glands.

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Principal features

Keratoconjunctivitis (dry eyes)
Xerostomia (Dry mouth)

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Primary syndrome

Occurs alone

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Secondary Sjogrens

In association with other autoimmune rheumatic diseases such as RA, SLE and scleroderma

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Epi

More common in 9:1

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Autoimmune post viral infections

Cytomegalovirus (CMV) and EBV have been found in patients with Sjorgens

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Initial Presentation

Dry gritty eyes
Difficulty in swallowing dry foods
Problems with their teeth or dentures

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Progression of the disease organ symptoms

Constitutional symptoms
MSK aches and pains
Raynauds

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Constitutional

Fever, malaise

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Glandular

Xerostomia, dental caries
Parotid gland enlargement
Keratoconjunctivitis sicca, corneal ulceration
Dry vagina, dyspareunia

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MSK

Arthralgia, arthritis
Myalgia

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Skin

Raynaud’s phenomenon
Palpable purpura (Cutaneous vasculitis)

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Kidney

Interstial nephritis
Renal tubular acidosis and renal stones

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Lung

Interstial lung disease

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Lymphoreticular

Splenomegaly
Lymphadenopathy
Lymphoma

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Schirmer’s tests →

Strip of paper is placed beneath the lower eyelid and left for five minutes – wetness of paper <5mm is strongly suggestive of decreased tear secretion.

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Rose Bengal Stain

Placed on the conjunctival sac, damaged by corneal epithelium takes up the strain which is then easily visible under slit lamp examination.

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Sialography

Measuring the anatomy of the major salivary gland ducts using radiographic contrast material –can be used to assist diagnosis

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Gold standard is

Salivary gland biopsy, will show lymphocytic infiltrates

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Laboratory features include

Anaemia of chronic disease
Raised ESR (not usually raised CRP)
Raised Gammaglobulins
Anti-RO (or SSA)
Anti-La (SSB)

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Dry Eyes treatment

Artificial tears (prevents corneal scarring)

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Xerostomia treatment

• Drugs with antocholinergic side-effects avoided (tricyclic antidepressants)
• Sugar free lozangers
• Adequate oral hygience and dental followup
• Piocarpine is sometimes used, but this is hampered by systemic adverse effects

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Glucocorticoids

For Major Organ involvement Rare only for severe extraglandular disease such as renal involvement and vasculitis

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Risk of Malignancy →

Patient with Sjogrens Syndrome 40x relative risk of developing lymphoid malignancy vs. age sex and race matched controls.
• B cell origin
Treat

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