Sjogrens Syndrome

Introduction

• Sjogrens syndrome is an autoimmune disease primarily affecting the exocrine glands, especially the lacrimal and salivary glands (sicca symptoms).
• characterized by
  • chronic arthritis
  • dry eyes
  • dry mouth
• Sjogrens occurs in a primary form (pSS) and secondary (sSS) form which is caused by another disease, most commonly rheumatoid arthritis.
• M:F = 1:9
• More common in middle-aged women
• Genetic component (pSS associated with HLADR2 and DR3).
• have an increased risk of developing B-cell non-Hodgkin’s lymphoma
  • Risk factors for lymphoma include
    • persistent gland swelling
    • enlarging lymph nodes

Presentation

Symptoms can be divided into exocrine and extra-exocrine disease.

EXOCRINE = Lacrimal and salivary glands are commonly affected.

Eyes

• SS produces reduced quality and quantity of tears. Symptoms are generally worse in the evening. Patient may complain of dry, gritty eyes. Photophobia may be present. Complications of dry eyes include corneal damage and blepharitis.
• Examination may show reduced Schirmer’s test.

Oral

• Patients may complain of oral dryness, difficulty swallowing dry food, changes in tastes, and inability to talk for a long time.
• Dental caries may be present, and atrophy of tongue papillae may be present. 50% of patients get parotid swellings.

Other Exocrine

• Vaginal dryness: irritation and dyspareunia.
• Dryness of upper airway: hoarse voice, recurrent infections.

EXTRA-EXOCRINE = SS can affect the patient systemically. Common extra-exocrine involvement are:

• Fatigue: common; can be a major complaint.
•  Cutaneous: dry skin, Raynaulds
• Joints: most SS suffered will have arthritis at some point
• Lungs: can cause ILD
• GIT: deranged LFTs; chronically elevated amylase; irritable bowel syndrome
• Kidneys: interstitial nephritis; glomerulonephritis
• Nervous system: autonomic dysfunction; ‘brain fog’
• Lymphoma: SS have 44x the risk of developing lymphoma

History and Physical

• Common complaints include dry eyes and mouth, foreign body sensation in eyes.
• Physical signs may include lack of tears, dry mouth, enlarged glands.
• Extraglandular manifestations like polyarthritis and neuropathy are common.

Tests for SS

• Assessment of oral and ocular dryness
• Schirmer’s test:
  • put filter paper on lateral third of lower eyelid. Measure the extent of wetting in 5 minutes. >15mm is normal; <5mm is abnormal.
• Sialography may show silaectasia.
• Parotid biopsy can confirm diagnosis SS.
• Anti-Ro and Anti-La have fairly high specificity for SS.
• ESR/CRP: may be elevated
• UEC, LFT: may be deranged
• Hypergammaglobinaemia is common
• HCV serology: hepC can cause sicca symptoms

DDx

• Many medical conditions (eg DM) and medications (eg anticholinergics) can cause dry mouth.
• Unilateral parotid gland enlargement: neoplasm, infection, stones
• Bilateral parotid gland enlargement: infection (eg mumps), sarcoidosis, liver disease, alcoholism
• Sarcoidosis, Rosacea, Mumps, Dehydration, Mouth breathing, Lymphoma, Advanced age, Parkinson’s disease, Scleroderma, Rheumatoid arthritis, AIDS, and Lupus

Treatment

Symptoms vary widely in intensity.

For dry eyes/Eye symptoms

• Lubricating eye drops
• Avoid contact lens. Be vigilant of infections.
• Puncta occluding surgery: decreases drainage of tears.

For dry mouth/Xerostomia

• Increased water intake
• chewing gum
• saliva substitutes
• Suck on lozenges
• Particular attention to dental care and oral hygiene.
• ? role of cholinergics (eg pilocarpine – not available in Australia).

Collaboration among rheumatologists, ophthalmologists, dentists, and primary care physicians is essential for optimal management.

Referral to specialist is warranted in the following:

• Extra-glandular disease (particularly skin / lung / liver / joint involvement).
• Florid salivary gland enlargement
• Lymphadenothy
• Cryoglobuminaemia
• Hypocomplementaemia
• Need for systemic immunosuppression.