Sjogren syndrome

What it is

Sjogren syndrome is idiopathic in its primary form. The secondary form is associated with rheumatoid arthritis and with primary biliary cholangitis, and secondary Sjogren's syndrome is also called sicca syndrome. It is far more common in women than in men. Most patients present in the post menopausal years, and multi-system involvement is common.

How it presents

The cardinal symptoms are the sicca symptoms, dry mouth and dry eyes, and there is grittiness in the eyes. Diffuse swelling of the parotid gland occurs, and it comes with no evidence of facial nerve palsy. Glandular features:

• Xerophthalmia and keratoconjunctivitis sicca.
• Parotid swelling.
• Xerostomia with difficulty swallowing, and caries.
• Vaginal dryness.

Systemic features:

• Fatigue, difficulty concentrating, CNS involvement.
• Interstitial pneumonia.
• Arthralgias and nonerosive polyarthritis.
• Nephritis and cholangitis.
• Secondary Raynaud phenomenon.
• Small vessel vasculitis with purpura.
• Peripheral neuropathy.

How it is diagnosed

ANA and rheumatoid factor are checked, together with Ro (SS-A) and La (SS-B), which are the anti-Ro/SSA and anti-La/SSB antibodies. The Schirmer test measures tear production and assesses dry eye; it is commonly used to diagnose Sjogren's syndrome and other conditions causing decreased tear production. Salivary gland biopsy is the most accurate. Two parotid mimics have to be excluded. Bilateral parotid swelling with bilateral facial nerve palsies that improves with steroid treatment is sarcoidosis, which occurs bilaterally in 70% of cases and in which facial nerve involvement is recognised. Painful bilateral parotid enlargement in a young adult with pancreatitis, orchitis, reduced hearing or meningoencephalitis points to mumps.