Introduction to Sjogren's Syndrome
Extraglandular Involvement in Sjogren's Disease
Dry mouth and eyes may occur with or without systemic involvement of other organ systems including:
Cutaneous symptoms - Skin involvement is the most common systemic manifestation of Sjogren's syndrome. Symptoms may include:
- rash
- itching
- purpura (the purple color of skin after blood has "leaked" under it)
- vasculitis (inflammation of the blood vessels)
Musculoskeletal symptoms such as painful joints
- Pulmonary symptoms such as a dry cough
- Hematological symptoms such as cytopenia (reduction in the number of cells circulating in the blood)
- Lymphoproliferative disorders such as lymphoma (cancer that starts in the cells of the immune system)
Sjogren's syndrome is difficult to diagnose since it is comprised of a wide range of symptoms that may not appear concurrently and, as a result, often are treated as individual conditions rather than as a total syndrome. Alternatively, diagnosis may be missed since the individual symptoms of Sjogren's syndrome mimic symptoms related to many other medical conditions. Some studies indicate that many patients with Sjogren's syndrome may suffer for an average of 10 years before they are correctly diagnosed.
While Sjogren's syndrome is a chronic, progressive condition, the progression for most patients is very slow. Sjogren's syndrome is more benign than other autoimmune diseases and typically is not associated with rapid deterioration of symptoms or dramatic changes in condition. Sjogren's syndrome is considered to be more a condition of morbidity rather than mortality. The most serious aspect of Sjogren's syndrome, however, is the increased risk of developing non-Hodgkin's B-cell lymphoma which is approximately 44 times greater than the risk of the general population.
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