Sjogren's Syndrome

History Taking

Chief Complaint:

• Dry eyes

• Dry mouth

• Joint pain and stiffness

History of Presenting Complaint:

• Chronic sensation of eye grittiness, burning or stinging

• Difficulty in swallowing food, especially dry ones

• Fatigue and feeling unwell persistently

• Increase in dental cavities

• Swollen salivary glands (parotid/submandibular)

• Rashes, dry skin, or sensitivity to the sun

• Persistent dry cough

System Review:

• Musculoskeletal: Symmetrical arthralgia or arthritis, especially small joints

• Gastrointestinal: Reflux, dyspepsia due to decreased gastric secretion

• Respiratory: Interstitial lung disease, persistent dry cough

• Neurological: Peripheral neuropathy, cognitive dysfunction

Past Medical History:

• Previous diagnosis of rheumatoid arthritis or other autoimmune disease

• Recurrent parotitis

• History of Raynaud's phenomenon

Drug History:

• Use of cholinergic drugs such as Pilocarpine to alleviate dryness symptoms

• Use of corticosteroids or immunosuppressive drugs for associated autoimmune conditions

• Over-the-counter use of artificial tears, eye drops, or saliva substitutes

Family History:

• Relatives with Sjogren's syndrome or other autoimmune disorders (e.g., systemic lupus erythematosus, rheumatoid arthritis)

Personal History:

• Smoking and alcohol history - both can exacerbate dryness symptoms

• Daily routines impacted by symptoms e.g., needing to drink water frequently, difficulty wearing contact lenses

Social History:

• Occupation: Jobs that require prolonged visual focus can exacerbate eye symptoms

• Living situation: Assess for support network as chronic illnesses can lead to depression/anxiety

OBG History:

• Gynaecological history of recurrent vaginal dryness or recurrent thrush

• Obstetric history of neonatal lupus or congenital heart block in a child

Physical Examination

General Examination:

• Fatigued appearance

• Dry skin

• Bilateral parotid gland enlargement

Hands and Arms:

• Joint swelling or deformity indicating rheumatoid arthritis

Eye Examination:

• Schirmer's test: Reduced tear production

• Ophthalmological examination: Conjunctival redness, corneal ulcers or abrasions due to dryness

Mouth Examination:

• Dry oral mucosa

• Dental caries

• Parotid gland enlargement

• Candidiasis due to the dry mouth

• Angular cheilitis

Neck Examination:

• Enlarged submandibular glands

Cardiac Examination:

• May be normal, or may indicate pericarditis in the case of associated autoimmune conditions

Respiratory Examination:

• Evidence of interstitial lung disease (bilateral fine crackles at lung bases)

Abdominal Examination:

• Typically normal in Sjogren's syndrome unless associated with other autoimmune disorders

Neurological Examination:

• Signs of peripheral neuropathy (decreased sensation, weakness, diminished reflexes)

Investigations

Laboratory Investigations:

• Complete blood count: Anemia or leukopenia in case of systemic involvement

• ESR and CRP: Elevated in case of systemic inflammation

• Autoantibodies: Anti-Ro (SSA) and Anti-La (SSB) antibodies are characteristic, antinuclear antibodies (ANA) can also be present

• Rheumatoid factor: Frequently positive in Sjogren's syndrome

• Immunoglobulins: Elevated IgG levels

Imaging Studies:

• Salivary gland ultrasonography: To identify changes in gland architecture

• Chest X-ray or HRCT chest: To identify interstitial lung disease if respiratory symptoms are present

• Sialography: May show sialectasis (dilation of the salivary ducts)

• MRI of salivary glands: Can show characteristic changes in Sjogren's

Invasive Tests:

• Lip biopsy: To detect lymphocytic infiltration in minor salivary glands, considered a gold standard for diagnosis

• Parotid gland biopsy: If enlargement is present and malignancy is suspected

Other Tests:

• Schirmer's test: To measure tear production, decreased in Sjogren's

• Rose Bengal staining: To assess ocular surface damage

• Salivary flow rate measurement: Reduced in Sjogren's syndrome

• Ophthalmological examination: Dry eye assessment

• Oral examination by dentist: Assess for tooth decay and oral health

• Pulmonary function test: If interstitial lung disease is suspected

• Neurological tests: EMG/NCS if peripheral neuropathy is suspected

Management

General Management:

• Patient education: Explain the chronic nature of the disease and importance of regular follow-up

• Symptom management: Encourage frequent sips of water, use of humidifiers, good oral hygiene

• Regular dental check-ups: To prevent dental caries due to dry mouth

• Avoid triggers: Such as dry environments or medications that can cause dryness

• Regular eye check-ups: To prevent complications from dry eyes

• Mental health support: As chronic diseases can lead to depression and anxiety

Medical Management:

• Artificial tears: For dry eyes

• Pilocarpine or cevimeline: To stimulate salivary production

• Hydroxychloroquine: For arthralgia and fatigue

• Topical or systemic corticosteroids, immunosuppressive agents: For systemic manifestations

• Regular dental care with high fluoride toothpaste and mouthwashes: To prevent caries

• Vaginal moisturizers or lubricants: For vaginal dryness

Surgical Management:

• Punctal plugs: To reduce tear drainage and keep the eyes moist

• Salivary duct dilation or stenting: For recurrent salivary gland infections or obstruction

• In severe refractory cases, parotid duct ligation or rerouting of salivary ducts to the oropharynx

Other Management:

• Regular physiotherapy: To help with joint symptoms

• Occupational therapy: To help adapt to dry eyes and mouth in daily activities

• Dietary adjustments: Eating moist foods, avoiding spicy or acidic foods that can irritate the mouth

• Smoking cessation: As smoking can exacerbate symptoms

• Support groups and counseling: For coping strategies and emotional support