Sjögren syndrome

Last revised by Liz Silverstone on 9 Dec 2023

Sjögren syndrome, or Sjögren disease, is an autoimmune condition of the exocrine glands that produce tears and saliva.

Sjögren syndrome is the second most common autoimmune disorder after rheumatoid arthritis. There is a recognized female predilection with F:M ratio of ~9:1. Patients typically present around the 4th to 5th decades.

Approximately 40% of cases occur in isolation. Known associations include:

Clinically, the cardinal features are glandular:

Additionally, there may be multiorgan extra-glandular involvement, which can have a wide variety of clinical features.

It is a chronic autoimmune disorder involving mainly the salivary and lacrimal glands and is associated with hyperactivity of the B-lymphocytes and with autoantibody and immune complex production. 

  • early stage: the gland can be normal or become enlarged and hyperechoic 5 

  • late-stage: may characteristically show a multicystic or reticular pattern within an atrophic gland 5

Parotid gland involvement may give a salt and pepper appearance or a honeycomb appearance. A change in the size of the lacrimal glands associated with accelerated fat deposition may also be seen 3.

In the setting of the extraglandular complication of sensory neuronopathy (ganglionopathy), there may be T2 hyperintensity affecting the dorsal columns, which can be longitudinally extensive 18,19.

One of the complications of this syndrome is the development of non-Hodgkin B-cell lymphoma, typically mucosa-associated lymphoid tissue (MALT) lymphoma, marginal zone lymphoma, and diffuse large B-cell lymphoma 17.

This condition is named after Swedish ophthalmologist Henrik Sjögren (1899-1986), although it was first described by W B Hadden and J W Hutchinson in 1871 13.

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