For healthcare professionals diagnosing primary Sjögren's Syndrome (pSS, an autoimmune disorder in which immune cells attack and destroy moisture-producing glands), the incidence of blood based deficiencies is the strongest predictor of a poor outcome in patients, according to the results of a study presented at EULAR 2010, the Annual Congress of the European League Against Rheumatism in Rome, Italy.
The study also showed that liver and lung involvement and non-Hodgkin lymphoma (NHL) development were also related to an increased mortality in pSS patients.
Results of a Spanish study have shown that patients who present with concurrent anemia (a reduced number of red blood cells), lymphocytopenia (a reduced number of white blood cells), or hypocomplementemia (where components of blood are lacking or reduced) were the most likely to develop poor outcomes, such as lymphoma. 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.
"Whilst pSS is often characterised by changes in exocrine function, the results of our study have determined the profile of some of the non-exocrine signs of the disease, including the pulmonary, haematological and hepatic manifestations," said Professor Roser Solans-Laqué, Internal Medicine, Vall D'Hebron University Hospital, Barcelona, Spain. "We hope that the results of our study will enable a better understanding of the factors that impact prognosis with the aim of monitoring for and managing these appropriately in the future."
Two hundred and forty-four patients (females n=235 males n=9) with pSS (primary SS occurs as a disorder on its own, with no known association with another connective tissue disease or rheumatic condition) registered at the Vall D'Hebron University Hospital, Barcelona were included in this single centre study. Mean age was 58 years, and clinical follow up ranged from nine months to 20 years (mean 8.6 years). Statistical analyses including multiple logistic regression were undertaken to determine associations between disease manifestations and patient outcome. At 20 year follow up, 11 patients (4.5%) had developed non-Hodgkins lymphoma, 22 (9%) patients had developed other malignancies (including lung, colon, breast and gynaecological neoplasms), and 18 (7.4%) of patients had died. Only an excess mortality due to lymphoproliferative malignancies was found in patients with pSS.
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