In Primary Sjogren's Syndrome (pSS) patients, concurrent anemia, lymphocytopenia, or hypocomplementemia are strong indicators that the outcome will be poor. The patients may progress to other manifestations such as lymphoma.

In a study carried out at the Vall D'Hebron University Hospital (Barcelona, Spain), 244 patients with pSS were followed for 20 years. Those with the abnormal hematological manifestations showed a progression towards such diseases as non-Hodgkin's lymphoma and other malignancies (including lung, colon, breast, and gynecologic neoplasms).

An excess mortality due to lymphoproliferative malignancies was found in patients with pSS. The existence of pulmonary (lung fibrosis, brochiectasis) and hepatic (biliary cirrhosis, autoimmune hepatitis) involvement were also shown to be independent risk factors related to mortality.

Primary Sjogren's Syndrome is often characterized by changes in exocrine function. However, this study has determined the profile of some of the non-exocrine signs of the disease, including the pulmonary, hematologic, and hepatic manifestations. The results of the study were presented recently at the Annual Congress of the European League Against Rheumatism in Rome, Italy (EULAR 2010).

Primary Sjogren's Syndrome is an autoimmune disease in which immune cells attack and destroy moisture-producing glands. In patients with pSS, the immune system targets moisture-producing glands and causes dryness in the mouth and eyes. Other parts of the body can be affected as well, resulting in a wide range of possible symptoms.

Sjögren's syndrome affects one to four million people in the United States, most of whom are more than 40 years old at the time of diagnosis, and women are nine times more likely to have the disease than men.

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Vall D'Hebron University Hospital