About Sjögrens Syndrome (SS)

What is  Sjögren’s Syndrome (aka Sicca) (pronounced Show-grens)?

Dry mouth and dry eyes - sounds like no big deal...However, it can cause damage to your corneas, increased dental cavities, pain, difficulty eating....just to name a few of the life altering issues.  It can also lead to lymphoma. It is thus very sad that so few doctors know much at all about it and it generally takes 7-10 years for diagnosis.

Sjögren’s syndrome often accompanies other autoimmune disorders, such as rheumatoid arthritis and lupus. In Sjögren’s syndrome, the mucous membranes and moisture-secreting glands of your eyes and mouth are usually affected first — resulting in decreased production of tears and saliva.
Although you can develop Sjögren’s syndrome at any age, most people are older than 40 at the time of diagnosis. The condition is much more common in women. Treatment focuses on relieving symptoms, which often subside with time.

Sjögren’s syndrome is a chronic inflammatory disorder of probable autoimmune nature characterized by infiltration of the exocrine glands, particularly the salivary and lacrimal glands, by lymphocytes and plasma cells. The classic signs of the Sjögren’s syndrome, therefore, includes enlargement of the parotid glands with mucosal dryness manifest by dry mouth (xerostomia) and dry eyes (xerophthalmia).

White blood cells infiltrate the glands that secrete fluids, such as the salivary glands in the mouth and the tear glands in the eyes. The white blood cells injure the glands, resulting in a dry mouth and dry eyes—the hallmark symptoms of this syndrome.

The parotid glands, which produce saliva and are located behind the cheeks and in front of the ears, may also become enlarged and Sjogren's syndrome can destroy the salivary glands.  Damage to the trachea and lung tissues can lead to pneumonia and other respiratory infections. Nasal dryness may cause frequent nosebleeds. The sac around the heart can become infected, called pericarditis. Vaginal dryness is another common symptom. Sjogren's syndrome can also affect the liver, spleen, pancreas, kidneys, and lymph nodes. Because Sjogren's syndrome is an inflammatory disease, many patients will also develop mild symptoms of arthritis. 

Sjögren’s syndrome exists in both a primary and secondary form. In the absence of other autoimmune disorders, it is classified as primary Sjögren’s syndrome. When occurring with a well-defined connective tissue disease, usually rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis or polymyositis, it is known as secondary.

The disease affects predominantly middle-aged women, in the peri- or post-menopausal period, in a female-to-male ratio of 9:1. However, it can be seen in both sexes and all ages. The prevalence is still unknown. Nevertheless, it is considered to be quite common since in addition to the primary syndrome, 30% of patients with rheumatoid arthritis, systemic lupus erythematosus and systemic sclerosis suffer secondary Sjögren’s syndrome. Besides, 2 to 5 % of people aged 60 and above have primary Sjögren’s syndrome.

Sjogren's syndrome can destroy the tear glands, causing damage to the cornea, itching, burning, redness, and increased sensitivity to light. Thick secretions often collect in the inside corners of the eyes or foreign body (gritty, sandy) sensation and accumulation of thick, ropy secretions along the inner canthus of the eyes. With time conjunctival injection, reduced visual acuity and increased photosensitivity develop. These symptoms, which occur because of the decreased and altered tear production, result in the destruction of corneal and bulbar conjunctival epithelium defined as keratoconjunctivitis sicca.

Drying of the mouth, xerostomia, is frequent but variable in severity. Patients usually complain of difficulty in eating dry food (like crackers, bread), inability to speak continuously, oral soreness, changes in tasting and smelling and fissures of the tongue and lips (angular queilitis).

Many patients, such as myself also suffer from hyper-sensitivity to spices - experiencing burning of the tongue and lips that can last long after the food is tasted.

An increase in dental caries is due to decreased saliva volume and the relative loss of its antibacterial factors. Physical examination shows a dry, erythematous, sticky oral mucosa.

Involvement of other exocrine glands occur less frequently. A decrease in the mucous gland secretions of the upper and lower respiratory tree results in dry nose, throat and trachea.

I, and others, experience sinus infections as a result of the drying of the mucus.

Mucosal gland involvement of gastrointestinal tract can be associated with dysphagia, atrophic gastritis, esophageal mucosal atrophy, constipation and subclinical pancreatic insufficiency. Vulva and vaginal dryness can result in dyspareunia.

I also have dry skin and hair from SS.

Extraglandular (systemic) features can complicate the course of the Sjögren’s syndrome and are seen in one third of the patients. These manifestations are more common in patients with primary rather than secondary Sjögren’s syndrome. The spectrum of extraglandular involvement varies. Most patients complain of easy fatigability, low-grade fever, myalgias and arthralgias.

Malignant or pseudomalignant lymphoproliferation may be a prominent part of the illness, especially in primary Sjögren’s syndrome. Most lymphomas derive from B cell lineage and may develop into monoclonal gammopathies (as Waldenstron’s macroglobulinemia). Pseudolymphoma or frank lymphoma should always be suspected when persistent major salivary gland enlargement, lymphadenopathy or lung nodules are noted.

Diagnosis and Differential Diagnosis

Diagnosis of Sjögren’s syndrome is based on the presence of two of the three following manifestations: keratoconjunctivitis sicca, xerostomia and an associated connective tissue or lymphoproliferative disorder. Salivary or lacrimal gland enlargement may or may not be present.

Minor salivary gland biopsy serves as the cornerstone for the diagnosis. Schirmer’s tear test is used for evaluation of tear secretion by the lacrimal glands. The test is performed with strips of filtered paper slipped beneath the inferior lid of an unanesthetized eye. After 5 minutes the wetting length of the paper is measured. Wetting of less than 5 mm is a strong indication for diminished secretion. Keratoconjunctivitis sicca, the sequelae of decreased tear production, is diagnosed with slit-lamp examination after using Rose Bengal staining of the corneal epithelium. Rose Bengal is a dye which stains the devitalized epithelium of both the cornea and conjunctiva.

The ophthalmologic and oral manifestations of Sjögren’s syndrome are generally not progressive. Patients with primary Sjögren’s syndrome are at an increased risk of developing lymphoproliferative disorders, including non-Hodgkin lymphoma. Patients with splenomegaly, bilateral parotid enlargement, and a history of radiation treatment are at especially high risk.

Sjögren’s syndrome remains fundamentally an incurable disease, since no therapeutic modality has been identified that may alter the course of the disease. Hence, the treatment of dry eyes is largely symptomatic and includes artificial tears and lubricant ointments. Occasionally, patients may require surgical punctual occlusions to block tear drainage. Managing the oral component of Sjögren’s syndrome requires the use of saliva substitutes, stimulation of salivary flow from functioning acinar tissue and aggressively fighting dental caries through both prevention and treatment.

Patients with severe extraglandular manifestations are usually treated with systemic corticosteroids and immunosuppressive drugs such as cyclophosphamide.

Sjögren’s also causes gastro-intestinal issues such as heartburn or GERD and constipation.

What Causes Sjögren’s syndrome?

While the exact cause of Sjögren’s syndrome is not known, there is growing scientific support for genetic (inherited) factors. The illness is sometimes found in other family members. It is also found more commonly in families that have members with other autoimmune illnesses, such as systemic lupus erythematosus, autoimmune thyroid disease, juvenile diabetes, etc. About 90% of patients with Sjögren’s syndrome are female.

Tests for Sjögren’s Syndrome

Since there are 3 different tear layers produced to make the right consistency of the tears, these tests, performed by an eye doctor, help determine where the dry eye problem may be coming from, and what treatment may be needed.
  • Schirmer 1 test - a test of eyelid wetness
  • Schirmer 2 test - a test of nasal wetness
  • Eye stain dye tests 
  • Tear film break-up time (<10 seconds)
  • Tear protein levels (lactorerrin and lysozyme
  • Positive Schrimer’s test.  In this test, a small piece of filter paper is placed under your lower eyelid to measure your tear production.
  • Slit-lamp test. An ophthalmologist, a physician specializing in the treatment of eye disorders, may also examine the surface of your eyes with a magnifying device called a slit lamp. He or she may place a drop of liquid containing a dye in your eye to make any damage to your cornea easier to see. 
  • Tear Osmolarity
  • Rose Bengal staining of eye (cornea or conjunctiva)
  • Lissamine green 
  • Evaluation of debris in tear film
  • Conjunctival impression cytoloty
  • Presence of corneal filaments
  • Mouth exam
  • Salivary gland biopsy of the lip
  • SS-A antibody blood test - also called SS-Ro
  • SS-B antibodies - also called SS-La
  • Antinuclear antibodies (ANAs) blood test (positive in most cases)
  • Antithyroid antibody blood test
  • Immunoglobulins blood test
  • Gamma globulins blood test
  • Cryoglobulin test
  • Chest x ray
  • Urinalysis
  • Spit test. In this test, you spit into a test tube every minute for 15 minutes. The total amount of saliva collected is then measured to determine the severity of your dry mouth.
  • Urine sample. Your doctor may want you to provide a urine sample that can be analyzed in the laboratory to determine whether Sjogren's syndrome has affected your kidneys.
  • Rheumatoid factor (RF) (may be positive)
  • Antibodies specific to Sjögren’s syndrome: Anti-SS-A (also called Ro) and Anti-SS-B (also called La)- (frequently positive)
  • HLA-DR positive glandular cells
  • Sialogram detects dye that's injected into your parotid glands, located behind your jaw and in front of your ears. This procedure shows how much saliva flows into your mouth.
  • Salivary scintigraphy. This nuclear medicine test involves the injection of a radioactive isotope, which is tracked to measure your salivary gland function.
  •  Low red blood count (anemia) is common