Tuesday, December 2, 2008 - 5:09PM EST

Introduction to Sjogren's Syndrome

Signs and Symptoms of Sjogren's Syndrome

  • Leukopenia - the number of white blood cells (leukocytes) in the bloodstream is lower than normal
  • Autoimmune cytopenia (reduced number of cells circulating in the blood) - this is usually a mild condition but can develop into a serious condition
  • Hyper/hypoglobulinemia (elevated/reduced levels of globulins in the blood)
Lymphoproliferative Disease

Lymphoma develops in approximately 5% of patients diagnosed with Sjogren's syndrome. It has been estimated that patients with primary and secondary Sjogren's syndrome have an approximately 44-fold increased risk for developing lymphoma as compared with healthy, age-matched controls. The most common form of lymphoma associated with Sjogren's syndrome is low or intermediate grade B-cell lymphoma that originates in mucosa-associated lymphoid tissue. Lymphoma occurs more frequently in patients with primary Sjogren's syndrome than secondary Sjogren's syndrome.

It is thought that the chronic stimulation and proliferation of monoclonal B cells in Sjogren's syndrome is related to the transition that takes place from an autoimmune status to the development of non-Hodgkin's lymphoma. Most lymphomas associated with Sjogren's syndrome are characterized as:

  • Low or intermediate-grade malignancy potential
  • Localized in extranodal spaces (spaces around the outside of the lymph nodes).

Clinical or laboratory evidence of an emerging lymphoma may include:

  • Persistent enlargement of parotid glands
  • Persistent enlargement of the spleen (splenomegaly)
  • Persistent enlargement of lymph nodes (lymphadenopathy)
  • Type II mixed monoclonal cryoglobulinemia
  • Low levels of complement factor C4 in the bloodstream
  • Vasculitis

Other lymph-related conditions that may develop with Sjogren's syndrome include:

  • Lymphopenia - a reduction in the number of circulating lymphocytes in the bloodstream
  • Waldenstrom's macroglobulinemia - this is a cancer of the B-lymphocytes (a type of white blood cell) which causes overproduction of monoclonal macroglobulins (IgM antibody)
  • Lymphadenopathy - an enlargement of the lymph glands
Cardiac Symptoms

Cardiac conditions which may be associated with Sjogren's syndrome include:

  • Pericarditis - inflammation of the sac surrounding the heart
  • Pulmonary hypertension - high blood pressure in the arteries that supply the lungs
  • Abnormal blood pressure related responses such as orthostatic hypotension (sudden drop in blood pressure when changing from sitting or lying to standing)
Hepatobiliary Symptoms

Some patients may also develop hepatitis C or a condition called primary biliary cirrhosis - a liver disease that slowly destroys the bile ducts and leads to the buildup of bile in the liver and eventual cirrhosis (hardening) of the liver. Typically, however, liver involvement in patients with Sjogren's syndrome is rare (up to 5% of Sjogren's syndrome patients) and when is does occur, is usually asymptomatic and subclinical.

Psychiatric Symptoms

The most common psychiatric conditions associated with Sjogren's syndrome are:

  • Depression
  • Anxiety

The high incidence of these conditions has led some researchers to believe that they may be part of Sjogren's syndrome rather than a reaction to the stress. Depression and anxiety also commonly precede the diagnosis of systemic lupus erythematosus as well as other autoimmune conditions. Other symptoms that may develop include subtle changes in cognitive function, memory, and concentration.

Thyroid Symptoms
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