Tuesday, December 2, 2008 - 3:50PM EST

Introduction to Sjogren's Syndrome

Signs and Symptoms of Sjogren's Syndrome

  • Vitiligo - white patches on skin due to loss of pigmentation

Cutaneous vasculitis (as well as other forms of vasculitis - see below) is considered to be a significant prognostic indicator with the development of lymphoma and mortality. A large 2004 study of 558 Sjogren's syndrome patients diagnosed with cutaneous involvement reported that 58% of the patients had cutaneous vasculitis and showed a higher incidence of:

  • Peripheral neuropathy - damage to the nerves that supply the arms and legs characterized by burning, tingling, numbness and pain
  • Raynaud's phenomenon - a circulatory disorder caused by insufficient blood supply to the hands and feet
  • Renal (kidney) involvement
  • Presence of autoimmune markers such as antinuclear antibodies (ANA) and rheumatoid factor
  • B-cell lymphoma

Other skin problems that may be experienced by patients with Sjogren's syndrome include:

  • Dry skin - affects up to 55% of patients with Sjogren's syndrome (xerosis)
  • Itchy skin
  • Burning skin - may be experienced in up to 20% of patients with Sjogren's syndrome
  • Skin rashes - may be experienced in up to 10% of patients with Sjogren's syndrome
  • Raynaud's phenomenon - may appear before the development of symptoms of dry mouth and dry eyes by many years. It is estimated to occur in up to 30% of patients with Sjogren's syndrome and is usually of minor clinical significance.
Vasculitis

Vasculitis refers to inflammation of the blood vessels and is estimated to occur in up to 10% of patients with Sjogren's syndrome. It typically involves small and medium blood vessels and ranges from benign (most common) to life threatening (rare). Risk factors for development of vasculitis include:

  • Parotid scintigraphy - Grades III-IV (a diagnostic technique based on the detection of radiation emitted by radioactive substances injected into the body; also called radionuclide scanning). This is considered as an independent prognostic factor of development of vasculitis.
  • Low C3 and/or C4 levels in the blood (hypocomplementemia)
  • Cryoglobulinemia (low levels of immunoglobulins that congeal in cold temperature)
  • Anti Ro/La antibodies

Life-threatening vasculitis, however, is rare and appears to be related with the presence of cryoglobulinemia (elevated levels of cryoglobulins in the blood plasma).

Pulmonary Symptoms

The most common pulmonary (lung) symptom of Sjogren's syndrome is a dry cough due to xerotrachea (dry, scratchy trachea). Pulmonary involvement ranges from 9-75% of patients with Sjogren's syndrome depending upon the diagnostic techniques that are used but is rarely clinically significant. Evidence of pulmonary changes appears on up to 50% of lung scans by some estimates, but they usually remain subclinical. Progression is very slow and typically does not develop into clinically significant pulmonary disease. In one study examining the incidence of pulmonary involvement in patients with Sjogren's syndrome, approximately 87% of patients had some degree abnormal pulmonary function.

Recent studies show that pulmonary involvement is typically at the bronchial or bronchiolar level but interstitial disease (damage to lung tissue) can occur. Other pulmonary complications which may occur include:

  • Tracheobronchia sicca - dryness of the tracheal pathways leading to continual dry cough
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