BIRMINGHAM, England -- Patients with primary Sjögren's syndrome are prone to develop serious complications over the long term, despite the presumption that this is a relatively benign autoimmune disease, a 25-year review found.
For instance, fully half developed an additional autoimmune disease such as cutaneous lupus, and one in every 10 was diagnosed with non-Hodgkin's lymphoma, according to Esha Abrol, BSc, of University College London.
Action Points
- Note that this study was published as an abstract and presented at a conference. These data and conclusions should be considered to be preliminary until published in a peer-reviewed journal.
- Note that in this cohort of patients with Sjögren's syndrome, the incidence of lupus and non-Hodgkin's lymphoma seems to be increased.
- Be aware that those with glandular disease or vasculitis were more likely to develop non-Hodgkin's lymphoma than those without those complications.
"Primary Sjögren's syndrome is a common inflammatory autoimmune disease characterized by lymphocytic infiltration of the exocrine glands, resulting in dry eyes and dry mouth," Abrol said at the annual meeting of the British Society for Rheumatology here.
The pathogenesis is uncertain, but some authors have suggested links with viruses, environmental factors, and vitamin D deficiency.
And while an association with B-cell lymphoma has been well described -- it's 24 times more common in Sjögren's patients than in the general population -- many other complications such as vasculitis that can compromise quality and length of life have been less recognized, she said.
"Previous studies of the long-term outcomes of primary Sjögren's syndrome have been limited in that studies with large sample sizes tended to have short follow-up, while those with longer follow-up focused on specific complications such as fatigue without looking at the bigger picture," Abrol said.
She therefore reviewed the experience at her center's Sjögren's syndrome clinic, where 152 patients have been followed since 1986.
More than 90% were women, 87% were white, and average age at diagnosis was 54.
Three-quarters of the patients were seropositive for anti-nuclear antibodies, more than half were positive for rheumatoid factor, and a third were positive for anti-Ro/La antigens.
The overall types of complications were glandular in 20.3% and extraglandular in 71%.
Specific autoimmune complications included thyroid disease in 15.8%, vasculitis in 10.5%, gastrointestinal diseases such as celiac disease and autoimmune hepatitis in 10.5, and dermatologic disorders in 10.5%.
One patient had four additional autoimmune conditions along with primary Sjögren's, Abrol said.
Hematologic abnormalities included hypergammaglobulinemia in 36.8% and monoclonal gammopathy in just under 10%.
Nervous system involvement and peripheral neuropathy were present in one-quarter of patients.
With mean follow-up of 12.4 years, 18.5% had died, at an average age of 72.
"This was 10 years earlier than the average life expectancy of a woman in the U.K.," she noted.
The most common cause was malignancy, in 11 patients, two of which were non-Hodgkin's lymphoma. Four other patients also had been diagnosed with non-Hodgkin's lymphoma, but died from other causes.
"When we looked at patients with and without non-Hodgkin's lymphoma, we found a 10.5-fold increased risk in those who had vasculitis and a 3.4-fold increase in those with glandular complications," Abrol said.
"We have carefully observed these patients over 25 years, and the main message of this study was that patients with primary Sjögren's syndrome have a substantial burden of disease over their lifetime, and careful surveillance is clearly warranted," she concluded.
Disclosures
The authors had no conflicts of interest.
Primary Source
British Society for Rheumatology
Source Reference: Abrol E, et al "A retrospective study of long-term outcome in 152 patients with primary Sjögren's syndrome: 25 year experience" BSR 2013; Abstract O4.