Sjögren Syndrome/Sjogren Syndrome

Page Contents

1 WHAT IS IT?
2 WHAT CAUSES IT?
3 WHY IS IT A PROBLEM?
4 WHAT MAKES US SUSPECT IT?
5 HOW DO WE CONFIRM A DIAGNOSIS?
6 HOW DO WE TREAT IT?
7 HOW WELL DO THE PATIENTS DO?
8 WAS THERE A WAY TO PREVENT IT?
9 WHAT ELSE ARE WE WORRIED ABOUT?
10 OTHER HY FACTS?
11 FURTHER READING

WHAT IS IT?

Sjögren syndrome is an autoimmune disorder that is characterized by the destruction of the exocrine glands (in particular the lacrimal and salivary glands).

Sjögren syndomre is typically characterized by the auto-immune destruction of the salivary and lacrimal glands (source)

WHAT CAUSES IT?

Lymphocytic infiltration into the exocrine glands is ultimately to blame for mediating this auto-immune process (this is a type IV hypersensitivity reaction).

WHY IS IT A PROBLEM?

As these exocrine glands are destroyed, the ability to produce both tears and saliva will become compromised. This can lead to corneal damage and the formation of dental cavities (dental carries) due to the loss of these secretions. Similarly, this immune process can also target the joints leading to arthritis.

Whats more, chronic inflammation in the parotid gland can lead to lymphoma.

WHAT MAKES US SUSPECT IT?

Risk factors

General: female sex, 40-60 year old age, HLA-DR3

Other autoimmune conditions: Primary biliary cirrhosis, rheumatoid arthritis, dermatomyositis, polymyositis

Initial Presentation

Common Chief Complaints:

Dry mouth (decreased saliva production)
Dry eyes (decreased tear production)

Associated Symptoms:

Joint pain (arthritis)
Dysphagia can be secondary to the dry mouth

Physical Exam Findings

HEENT exam can show:

Bilateral parotid enlargement
Dry mucous membranes (eyes and mouth)
Tooth decay

This patient with Sjögren syndrome has very obvious bilateral parotid gland swelling (source)

Joint inspection can reveal inflammatory arthritis

HOW DO WE CONFIRM A DIAGNOSIS?

Ultrasound of the neck can help confirm that it is the parotid gland that is swollen.

Confirmation of parotid gland swelling (via ultrasound) in a patient with Sjögren syndrome (source)

Serology can help aid in the diagnosis. The presence of the following auto-antibodies can raise one’s suspicions for this condition.

Anti-SSA/anti-Ro
Anti-SSB/anti-La
Rheumatoid factor.

Biopsy of the salivary gland (minor salivary glands of the lip are accessible) that demonstrates lymphocytic infiltrate further supports the diagnosis of Sjögren syndrome.

Lip salivary bland biopsy in a patient with Sjögren syndrome (above) shows the presence of immune cell infiltration (source)

Diagnostic criteria: can be found here.

HOW DO WE TREAT IT?

**No known cure for this condition, treatment goal is symptom relief and the prevention of complications**

To treat the patient’s ocular dryness: one can use artificial tears

To treat oral dryness: saliva substitutes can be used.

Organ specific disease should have treatments tailored to it.

HOW WELL DO THE PATIENTS DO?

This will depend on a few factors. The disease itself is not deadly, however the lymphoma that can be associated with it can be fatal for some patients.

WAS THERE A WAY TO PREVENT IT?

N/A

WHAT ELSE ARE WE WORRIED ABOUT?

Dental caries/decay is a complication.

Keratoconjunctivitis can be caused by the loss of tear production.

MALT lymphoma is more common in patients who have this condition. This will present as a unilateral parotid mass later on in the disease.

OTHER HY FACTS?

The parasympathetic system is generally more dominant in controlling alive production.

The Schemer test assesses tear production by measuring the amount of wetness on a piece of filter paper in the lower eyelid for 5 minutes, and can be used to help screen for this condition. Affected patients will have lower amounts of tears produced. This seems to be a bit of an outdated test but may still pop up on standardized exams.