Home Sjogren's Syndrome And Lupus
Stuart S. Kassan, M.D., F.A.C.P.
Clinical Professor of Medicine,
University of Colorado Health Sciences Center

1. Mouth

2. Eyes

3. Extraglandular

Introduction

Sjogren's syndrome is a chronic autoimmune disorder in which the glands that produce tears and saliva do not function correctly.

1. The disease may lead to eye and mucosal dryness.

2. The disease primarily affects females in their 40s and 50s.

3. Sjogren's syndrome is a relatively common disease, although often under-diagnosed.

Description of Sjogren's Syndrome

Sjogren's syndrome can occur:

1. alone OR

2. in association with other autoimmune diseases, most commonly systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA).

Several studies have shown various differences between people with Sjogren's syndrome alone and those with Sjogren's syndrome and another connective tissue disease. Because of these differences:

Sjogren's syndrome is termed primary Sjogren's syndrome when it occurs by itself.

When associated with another connective tissue disease, such as SLE or RA, it is termed secondary Sjogren's syndrome.

The diagnostic criteria for Sjogren's syndrome include:

1. dry mouth

2. poor salivary (saliva producing) gland production

3. dry eyes

4. often the presence of antinuclear antibodies

5. the presence of a positive rheumatoid factor.

Because the disease is mild in many people, the first signs of mucosal dryness may be present for years before the disease becomes clearly evident.

Symptoms of Sjogren's Syndrome

Mouth

The symptom most associated with Sjogren's syndrome is oral (mouth) dryness, known as xerostomia.

Other oral symptoms that may be present include:

- burning of the tongue

- cracking of the tongue

- increased dental caries (cavities)

- trouble swallowing

- difficulty speaking without the use of continued lubrication.

Fluid intake is often necessary both with and between meals because of the decrease in salivary gland secretion.

The parotid glands (major salivary glands located behind the jaw) may be enlarged and painful swelling may also be noted.

- Enlargement of parotid or other major salivary glands occurs in two-thirds of those with primary Sjogren's syndrome

- Enlargement of the parotid and other glands is uncommon in those with secondary Sjogren's syndrome.

Eyes

Eye involvement is the other major manifestation of Sjogren's syndrome.

People often complain of a sandy or gritty feeling in their eyes, especially in the morning.

Other ocular symptoms that may be present include:

- decreased tearing

- redness

- itching

- photosensitivity, due to the loss of the lining cells of the conjunctiva, a condition called keratoconjunctivitis sicca.

Glands

- A decrease in mucous gland secretion of the upper and lower respiratory tract will be indicated by a chronic dry cough.

- A decrease in mucous gland secretions of the gastrointestinal tract may be associated with esophageal (throat) dryness, as well as trouble swallowing and irritation of the stomach.

- Vaginal dryness may cause irritation and pain during intercourse.

Extraglandular

Symptoms that show up outside the glands are seen in one-third of people with primary Sjogren's syndrome, but rarely in those with secondary syndrome.

These extraglandular symptoms may include:
1. joint pain
2. muscle pain
3. low-grade fevers
4. increased fatigue.

Such symptoms and signs may be associated with:

- lung involvement
- kidney involvement
- vasculitis (inflammation of blood vessels).
- lymphomas (tumors of the lymph glands), which develop in 6% of people with Sjogren's syndrome who have systemic disease.

Laboratory Abnormalities in Sjogren's Syndrome

Autoantibodies are common in Sjogren's, with
- 80% testing positive for antinuclear antibodies (ANA).
- Rheumatoid factor is present in 75% to 95% of those with Sjogren's.
- Elevated proteins will be seen on a serum protein electrophoresis test in 80% with Sjogren's.

Other nonspecific laboratory abnormalities that are commonly noted in Sjogren's:
- elevated erythrocyte sedimentation rates (a sign of inflammation)
- mild anemia
- low albumin levels.

Certain genes may also be found more frequently in people with primary Sjogren's. These "histocompatibility antigens" may include HLA-B8 and HLA-DR3.

Diagnostic Tests And Procedures in Sjogren's Syndrome

Several diagnostic tests are commonly used in people suspected of having Sjogren's syndrome:

In the Schirmer's test, a piece of filter paper is placed in the corner of the eye to measure the degree of wetting after five minutes.

The Rose-Bengal staining test determines whether there is inflammation of the cornea.

Salivary gland flow rates help to determine whether there is decreased saliva production.

Salivary gland biopsy of the lip or parotid gland may help to establish the diagnosis.

Associations of Sjogren's Syndrome With Lupus And Other Disorders

The coexistence of Sjogren's syndrome with SLE was first described in 1959. Since that report, numerous studies have compared and contrasted the two diseases. Many of the clinical and serological (serum-related) features of Sjogren's syndrome and SLE make the precise diagnosis difficult because there are similarities between the two diseases.

- It is not uncommon to find features of secondary Sjogren's in people with SLE.

- Those with primary Sjogren's syndrome and those with SLE have other similar disease symptoms, including:

1. arthritis
2. skin rash
3. kidney disease.
4. increased levels of antinuclear antibodies are seen in both diseases.

- In some cases, an increased association of certain genes is found in individuals with both primary Sjogren's syndrome and SLE.

- Sjogren's syndrome may also be confused with disease processes, such as:
1. viral infections
2. drug side effects (dryness from various antihypertensive agents, antidepressant agents or antihistamine agents, etc.).

- Other disease processes may also cause salivary gland enlargement:
1. viral infections
2. sarcoidosis
3. diabetes.

Treatment for Sjogren's Syndrome

Treatment for Sjogren's syndrome attempts to relieve the effects of chronic dryness of the mouth and eyes by increasing the lubrication and moisturization of the affected tissues:

1. Artificial tears in various forms help replace decreased tear production. However, artificial tears containing preservatives may cause irritation.

2. Plugging the lacrimal (tear) ducts with temporary or longer lasting collagen plugs, can be very effective in increasing moisture of the conjunctiva and cornea. Various other experimental agents are being developed.

3. Local oral agents have been used for continuing lubrication, but with limited success.

4. Systemic agents have also been used, with varied success, including:
- 5 mg three times a day of pilocarpine (Salagen), or bromhexine

Hydroxychloroquine (brand name: Plaquenil), an antimalarial, has been used for several reasons:

- the fatigue and joint pain associated with primary Sjogren's syndrome

- to decrease levels of some of the autoantibodies and sedimentation rates.

Systemic corticosteroids and/or immunosuppressive agents (cytotoxic drugs) have been used for various extraglandular symptoms of Sjogren's syndrome, such as:

- vasculitis
- lung involvement
- kidney involvement.

However, cytotoxic agents should be used with great care as they may increase the risk of lymphoma.

Prognosis of Sjogren's Syndrome

- The overall disease course of Sjogren's syndrome can vary from very mild to fairly significant.

- Individuals with secondary Sjogren's syndrome seem to have milder disease, as compared to those with primary Sjogren's syndrome.

- As previously noted, people with primary Sjogren's syndrome seem to have a greater chance of extraglandular involvement.

- Early diagnosis and early treatment are extremely important in trying to prevent damage to major organs.

- Learning as much as possible about the disease process will enable individuals to be aware of problems and symptoms.

- Routine follow-up with the physician is equally important.