What is Sjogren’s Syndrome?

Sjögren’s Syndrome (pronounced “show grins”) …is a chronic, inflammatory, multi-system, auto-immune disorder that is progressive. This means those who have Sjögren’s have it for the rest of their lives and it may affect many parts of their bodies.  Auto-immune refers to a process whereby the cells that are usually directed to fighting infections are actually attacking our own cells, creating inflammation and damage to tissues, in particular the mucous membranes (moisture-producing tissues/glands). This damage is slowly progressive (gets worse with time).

Sjögren’s predominately affects woman (90% of people with Sjögrens are women: 10% men).

The two main symptoms of Sjögrens syndrome are:                            
• Dry eye (keratoconjuctivitis sicca) and.
• Dry mouth (xerostomia)

However, people who have Sjogren’s Syndrome usually have a varied mixture of many other symptoms, which may include:
• Extreme fatigue
• Muscle pain
• Arthritis (joint pain, swelling, sometimes deformity)
• Dry/itchy skin
• Dry nose with crusting and infection
• Dry vagina, recurrent thrush
• Gastroesophageal reflux (heartburn, indigestion)
• Difficulty swallowing, alteration of taste
• Painful mouth with ulcers, recurrent oral candida
• Rampant tooth decay
• Hoarseness, cough
• Swollen salivary glands – The 3 main glands being:
   o The Parotid glands, which sit in front of the lower part of each ear (they are the same glands that swell during mumps)
   o The Submandibular glands, which sit on the jaw bone (mandible) in the lower mid section of each cheek
   o The Sublingual glands, which are found under the tongue.
There are a few much smaller salivary glands throughout the mouth, particularly in the tissues close to the lip, which is the area sometimes biopsied for diagnosis of Sjogren’s Syndrome

Rarely more serious complications may occur, including:
• Vasculitis (inflammation of the blood vessels), which can lead to rashes and ulcers on the skin.  Vasculitis can also cause injury to muscles, nerves and any organ of the bodies organs. When the blood vessels supplying these organs and tissues become inflamed the blood supply is decreased and damage results to the tissues causing symptoms relating to these organs/tissues.
In some cases, vasculitis causes inflammation of blood vessels in the brain, causing difficulty with thinking and concentration, confusion, memory problems,   stroke-like symptoms, headache and, very rarely, fits and coma.  However this is very rare and most people with Sjogren’s who experience headaches, poor concentration and memory problems will have other more common reasons for these such as stress, depression, poor sleep/fatigue or menopause.

• Neuritis (nerve inflammation caused by lymphocytes attacking and infiltrating nerves)
 Numbness, weakness, and painful neuropathy (nerve pains) may result from this injury to the nerves.  The muscles supplied by the inflamed nerves also become damaged as they rely on the nerve supply to keep them healthy and functioning.  This may result in paralysis and wasting of the muscles which are supplied by the inflamed nerves.

   o Involvement of internal organs.  The abnormal lymphocytes may attack and infiltrate the internal organs causing problems with any one of the organs including:
   o Lungs. Breathing problems may develop (you may feel like you can’t get enough air) Infiltration of the lung tissues makes the lungs stiffer and this reduces lung volume and makes it harder to take a full breath of air. Cough and tendency to infections may be noticed from dryness of the mucous membranes lining the tubes to the lungs.
   o Heart. Pericarditis (Inflammation of the sac which holds the heart) This complication is more commonly found  in people who have Rheumatoid Arthritis, Systemic Sclerosis (Scleroderma) or Systemic Lupus Erythematosis (S.L.E.) often called Lupus.  Many people with these conditions also have Sjogrens Syndrome.  Pericarditis causes shortness of breath and chest pain which is often worse when changing position such as leaning forward or with when taking a deep breath. It is diagnosed with an ECG and often an echocardiogram to look for fluid accumulation in the space between the heart and the pericardial sac.  If fluid develops this is called a pericardial effusion .  This is a very serious complication as the heart is squeezed and unable to fill and pump blood properly causing fluid to build up in the lungs (pulmonary oedema).  Extreme shortness of breath and fatigue results. This condition is likely to become fatal if not diagnosed and treated promptly and appropriately.
   o Liver…Autoimmune Hepatitits is inflammation of the liver caused by immune cells (lymphocytes) attacking the liver.  This causes raised levels of liver enzymes noticed on blood tests. Symptoms may include a tender liver (the liver sits under your right rib), jaundice (yellow pigmentation of the skin and whites of the eyes), and nausea/vomiting.
   o Gallbladder
   o Kidneys
   o Gastrointestinal tract (including pancreas)…build up of acid from lack of the neutralising and protective effects of saliva causes burning indigestion and tendency to develop gastrointestinal inflammation and ulceration.  Infiltration by lymphocytes can also cause inflammatory bowel disease.  Symptoms of this include blood and mucous in the bowel motions and colicky abdominal pains, and weight loss.  Some infections can give these symptoms also so faecal specimens need to be taken.  Usually this condition is investigated with a colonoscopy and biopsy of the bowel. This is usually done under sedation and is painless.
   o Bladder. Interstitial Cystitis- causes bladder pain inparticular pain when passing urine and a feeling of needing to pass urine often.  These symptoms are similar to the symptoms of a urinary infection so this needs to be checked for however in interstitial cystitis there is no infection- the inflammation causing the irritation of the bladder is caused by lymphocytes infiltrating and attacking the tissues of the bladder.

•  Lymphoma (a tumour of the lymph nodes) is more frequently found in those with Sjogren’s Syndrome than in the general population.  It is important to take notice of any lymph nodes which are persistently enlarged or getting larger and get these checked by your doctor.  These nodes may not be tender but lymphoma is a serious complication sometimes seen in people with Sjogren’s.  It may develop in the salivary glands also.  Any swelling of the salivary glands should also be checked by your doctor.

•  Pregnancy complications – Congenital heart block may develop in the foetus during pregnancy in woman who have Sjogren’s Syndrome auto antibodies. Anti SS-A and Anti SS–B are able to cross the placenta, damaging heart tissues, causing the baby to have a very slow heart beat. In severe cases, the child may require a pacemaker. Many of these mothers do not have symptoms of Sjogren’s; of those who do and become pregnant, only as few as 1-2 % will develop this rare complication. Pregnancy should be monitored from 18 weeks, as treatment with steroids will improve the outcome.

There are two forms of Sjogren’s Syndrome:
Sjogren’s is often classified as Primary or Secondary Sjogren’s.

Primary Sjogren’s Syndrome is defined as dry mouth and eye (and other mucous membranes including the vagina) not associated with another underlying auto-immune disorder.

Secondary Sjogren’s is when dry eye and dry mouth is associated with an underlying autoimmune connective tissue disorder, such as rheumatoid arthritis, systemic lupus erythematosus (SLE), or scleroderma.

Sjogren’s Syndrome is named after a Swedish ophthalmologist Henrik Sjogren, who, in the early 1900s, recognised a group of people with the combination of dry eyes and dry mouth. He called the syndrome “keratoconjunctivitis sicca”. The term Sicca Syndrome is still used to describe this combination of dryness of the mouth and eyes, no matter what the cause. There are many other causes of dry eyes and mouth.

Many Sjogren’s symptoms, as in most auto-immune diseases, will flare up at times while at others appear to settle down (go into remission). Stress is well recognised as a trigger to a “flare up”. However the destruction of the mucous secreting glands in Sjogren’s is slowly progressive: it results in increasing loss of function of the moisture-producing glands, causing increasing severity of the dry symptoms of Sjogren’s over the years.

In Sjogren’s Syndrome, T lymphocytes infiltrate into the mucous membranes of the exocrine glands of the body, causing progressive damage and loss of function of these glands.

Sjogren’s Syndrome is thought to be associated with genetic factors, which may be triggered by some viruses and stress.  The exact cause is not yet known.  There is much research being done into the cause and the treatment of Sjogren’s.  Some research has suggested there is an association with exposure to some environmental toxins/chemicals and autoimmune diseases.

Sjogren’s is not strongly inherited. It is not common for children of a parent with Sjogren’s, to develop Sjogren’s.  However people with Sjogren often have another associated autoimmune disease and have members of their extended family who are diagnosed with some form of autoimmune disease.

Some of these autoimmune diseases are:
o •         S.L.E.(systemic lupus erythematosus or Lupus)
o •         Rheumatoid arthritis
o •         Autoimmune thyroid disease
o •         Primary biliary cirrhosis.. autoimmune complexes cause inflammation and progressive destruction of the small bile ducts within the liver.  Symptoms: Skin itch, fatigue, dry mouth and eyes, and jaundice.
o •         Scleroderma (systemic sclerosis ), or associated CREST Syndrome …(Calcinosis -calcium deposits in the skin, usually in fingers and around joints, Raynaud phenomenon -where hands and feet when cold go white/purple when cold and then red as they warm up, Esophageal dysmotility and reflux oesophagitis sometimes with strictures -causes indigestion; difficulty swallowing and sometimes food sticking , Sclerodactyly/Scleroderma -thickening and shrinking/contracture of the skin especially of fingers and around mouth , and Telangiectasia-small blood spots on the skin with spidery appearance-on face hands and in gastrointestinal system)
o •         Polymyositis (PMR) …chronic muscle inflammation with associated muscle weakness
o •         Mixed connective tissue disease…a combination of  S.L.E., scleroderma, and polymyositis
o           Ankolysing Spondylitis..  a destructive arthritis of the spine, which can also affect kidneys, heart, lungs, bowels and eyes.  Affects men predominantly.