Seminar G Sjorgens Syndrome Flashcards
Definition
Chronic autoimmune disease characterised by lymphocytic infiltrates and destruction of epithelial exocrine glands.
Principal features
Keratoconjunctivitis (dry eyes)
Xerostomia (Dry mouth)
Primary syndrome
Occurs alone
Secondary Sjogrens
In association with other autoimmune rheumatic diseases such as RA, SLE and scleroderma
Epi
More common in 9:1
Autoimmune post viral infections
Cytomegalovirus (CMV) and EBV have been found in patients with Sjorgens
Initial Presentation
Dry gritty eyes
Difficulty in swallowing dry foods
Problems with their teeth or dentures
Progression of the disease organ symptoms
Constitutional symptoms
MSK aches and pains
Raynauds
Constitutional
Fever, malaise
Glandular
Xerostomia, dental caries
Parotid gland enlargement
Keratoconjunctivitis sicca, corneal ulceration
Dry vagina, dyspareunia
MSK
Arthralgia, arthritis
Myalgia
Skin
Raynaud’s phenomenon Palpable purpura (Cutaneous vasculitis)
Kidney
Interstial nephritis
Renal tubular acidosis and renal stones
Lung
Interstial lung disease
Lymphoreticular
Splenomegaly
Lymphadenopathy
Lymphoma
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.
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.
Sialography
Measuring the anatomy of the major salivary gland ducts using radiographic contrast material –can be used to assist diagnosis
Gold standard is
Salivary gland biopsy, will show lymphocytic infiltrates
Laboratory features include
Anaemia of chronic disease Raised ESR (not usually raised CRP) Raised Gammaglobulins Anti-RO (or SSA) Anti-La (SSB)
Dry Eyes treatment
Artificial tears (prevents corneal scarring)
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
Glucocorticoids
For Major Organ involvement Rare only for severe extraglandular disease such as renal involvement and vasculitis
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
