May 16, 2012
   
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Dry Another Day

 
Dr. Triantafyllopoulou is Senior Resident, Department of Medicine, Montefiore Medical Center and Albert Einstein College of Medicine, New York. Dr. Triantafyllopoulou reports no commerical conflicts of interest.


Have you noticed that your mouth is often dry? Or that your eyes feel like they have sand in them?(1) If so, you may be suffering from an autoimmune disorder that affects more people than rheumatoid arthritis and that often goes undetected.

The disorder, Sjogren's syndrome (SS), occurs in 1-3% of people,(2) yet SS remains undiagnosed more than half of the time. Women are affected much more often than men (ratio of 9:1). Though the syndrome can appear at any age, Sjogren's occurs primarily among women in their thirties or forties.

It is important to establish a diagnosis of SS for a number of reasons because treatment can improve an unsuspecting patient's quality of life. And, for a small group of patients with the disorder, who may be at an increased risk for the lymphoma, a malignant cancer, early diagnosis is especially important.


Primary and Secondary SS
Doctors have classified SS into two types: primary (pSS) when it occurs alone and secondary (sSS) when it is accompanied by another autoimmune disorder such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) or scleroderma.

Signs and Symptoms of SS
As shown in Table 1, manifestations can be thought of as:
  • Glandular, which result from destruction of glands by an invasion of lymphocytes (white blood cells)
  • Systemic, which are characterized by lymphocyte invasion of the skin, lungs, kidneys or peripheral nervous system.

Table 1.
Manifestations of Sjogren's Syndrome.
Glandular Manifestations
  1. Xerostomia (dry mouth)
  2. Parotid gland enlargement
  3. Xerophthalmia (dry eyes)
  4. Xerotrachea (dry trachea)
  5. Dyspareunia (painful sexual intercourse)
Extraglandular Systemic Manifestations
  1. Fatigue
  2. Raynaud's phenomenon, easy bruising (purpura), recurrent hives (urticaria)
  3. Arthralgias/arthritis
  4. Lung involvement
  5. Difficulty swallowing food
  6. Inflammation of the kidneys (interstitial nephritis)
  7. Peripheral neuropathy
  8. Lymphoma
Glandular Manifestations
Xerostomia(from the Greek words xeros=dry and stoma=mouth, to describe destruction of salivary glands resulting in dry mouth): along with dry eyes (xerophthalmia), the presence of dry mouth helps the doctor make the diagnosis (see Table 2). Patients with xerostomia may complain of dry mouth, frequent need to drink fluids, or may simply complain of an unpleasant taste, difficulty eating dry food, soreness of the mouth and throat, or difficulty using dentures.

On exam, the affected patient has little or no saliva in the floor of their mouth; in advanced disease, the surface of the tongue becomes red and lobulated, with partial or complete depapillation.

Normally, the salivary glands produce 1-1.5 L of saliva every day, which help fight bacteria, so that, in its absence, patients with SS are prone to develop dental carries and increased rate of oral bacterial infections with Streptococcus mutans and Lactobacillus. Furthermore, SS is the most common cause of acute bacterial infection of the salivary glands (sialadenitis). Acute bacterial sialadenitis produces a tender swelling of the salivary gland and, sometimes, other lymph node enlargement, fever and malaise.

Dry mouth, however, is not specific for SS and can be found in a number of other conditions as listed in Table 2.

Table 2.
Differential Diagnosis (D/D) of Sjogren's Syndrome.

D/D of dry mouth D/D of parotid gland enlargement D/D of dry eyes
  • SS
  • sarcoidosis
  • amyloidosis
  • diabetes mellitus
  • dehydration
  • medications (anticholinergics, antihypertensives, antidepressants)
  • irradiation
  • viral infection
  • congenital (salivary gland malformation)
  • psychogenic
Bilateral
  • SS
  • sarcoidosis
  • diabetes mellitus
  • cirrhosis of the liver
  • viral infections (e.g., EBV, CMV, HCV, HIV)
  • other (acromegaly or hypogonadism, hyperlipoproteinemias, chronic pancreatitis)
Unilateral
  • SS
  • Salivary gland neoplasm
  • bacterial infection
  • chronic sialadenitis
  • sialolithiasis
  • SS
  • sarcoidosis
  • amyloidosis
  • chronic blepharitis or conjunctivitis
  • pemphigoid
  • Stevens Johnson syndrome
  • toxicity from drugs or burns
  • other (hypovitaminosis A, corneal anesthesia, eyelid scarring, blink abnormality, trauma)
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