SJOGREN’S SYNDROME

• Sjogren’s syndrome is a chronic autoimmune exocrinopathy characterised by lymphocytic infiltration of lacrimal, salivary and other exocrine glands. Sjogren’s syndrome derives its name from Henrik Sjogren who introduced the term Kerato conjunctivitis sicca for dryness of eyes noted in patients
• It can occur in any age group but generally presents in fourth to sixth decade mainly in women. Sjogren’s syndrome may exist as primary or secondary when it occurs in association with other systemic auto immune diseases like RA, SLE, polymyositis etc.
• In sjogren’s the immune system mainly attacks lacrimal and salivary glands, patients typically present with xerophthalmia (dry eyes) and xerostomia (dry mouth), the combination of which is also known as sicca syndrome. The disease may also involve the skin, joints, internal organs, and nervous system

Primary Sjogren syndrome

• Idiopathic (association with HLA-DR)

Secondary Sjogren syndrome

• Autoimmune connective tissue diseases
• Rheumatoid arthritis
• Systemic lupus erythematosus
• Systemic sclerosis
• Polymyositis
• Primary biliary cirrhosis

Glandular symptoms

Occular : Chronic lacrimal gland inflammation leads to decreased aqueous tear secretion producing symptoms of

• Dry eye
• Foreign body sensation
• Photophobia
• Redness
• Thick mucous strands and encrusted eyelids on awakening in the morning
• Corneal abrasion , ulcer, infection

Oral: Inflammation of salivary glands leads to decreased production of saliva producing

• Difficult in eating without the help of liquids
• Parched feeling in mouth & throat
• Altered taste in mouth
• Dental caries
• Oral candidiasis
• parotitis

Systemic features

• General: fatigue & Arthralgias , Myalgia
• Nasal dryness : Chronic rhinitis
• Vaginal dryness: Dyspareunia
• Vasculitis : purpuric lesions on lower limb on prolonged standing
• Skin : Raynaud’s phenomenon, Xerosis
• Pulmonary : Dry cough, recurrent bronchitis, interstitial lung disease
• Gastro intestinal : Dryness of Pharynx & Oesophagus, Gastro oesophageal reflex
• Cardiovascular : Pericarditis, Pulmonary HTN
• Nervous system : Myelopathy, optic neuropathy, seizures, paraesthesia
• Reanal : Interstitial nephritis

Blood 

• ↑ ESR, normocytic anemia, Leukopenia, Eosinophilia, hypergammaglobulinemia
• Anti-Ro/SSA antibody and anti-La/SSB antibody – positive in 70% of cases
• Antinuclear antibodies (ANA) - positive in up to 95% of cases
• Rheumatoid factor (RF) - positive in up to 60% of cases

Urine analysis

• proteinuria, red cell casts

Eye examination 

Schirmer's test

• shows decreased tear production . < 5 mm of moisture on the filter paper after 5 minutes are indicative of Sjogren syndrome 

USG 

• parotid gland shows Honeycomb or cloud-like structure of the glandular parenchyma

Biopsy 

• Labial salivary gland

• Treat the underlying disease in Secondary Sjogren syndrome
• Adequate ophthalmic care to prevent corneal ulceration and its sequelae
• Adequate hydration and foods that stimulate salivary flow