BIRMINGHAM, ENGLAND – Patients with Sjögren’s syndrome had a diverse and high rate of long-term complications over the course of a 25-year review of cases seen at a single center.
Between 1986 and 2011, 152 patients with primary Sjögren’s syndrome were treated at University College London (UCL), England. More than half of the patients developed serological abnormalities (51.3%), followed by additional autoimmune diseases in 49.3% and systemic malignancy in 28.3%.
“We found that the main complications were extraglandular,” Ms. Esha Abrol, a fifth-year medical student at UCL, reported at the British Society for Rheumatology annual conference. Extraglandular manifestations occurred in 71% of patients, and 20% of patients experiencing local or glandular complications, such as parotid swelling.
These data highlight that primary Sjögren’s syndrome is perhaps not as benign as people might think, Ms. Abrol suggested, and that an increase in the risk of non-Hodgkin’s lymphoma (NHL) is not the only problem to watch out for.
The majority of the study population was female (91.4%) and white (86.8%); three-quarters (75.7%) had antinuclear antibodies, 55.9% had anti-Ro/SS-A antibodies, 35.5% anti-La/SS-B antibodies, 54.6% were rheumatoid factor positive, and 36.8% had hypergammaglobulinemia. The mean age at diagnosis was 54.4 years.
At the time of the case review, at a mean of 12.4 years’ follow-up, 55.9% of patients were still alive; 26.6% had been lost to follow-up, which was mainly from old age, and 18.5% had died. The mean age at death was 72 years.
“One of the most common extraglandular manifestations was additional autoimmune diseases,” Ms. Abrol reported. These included autoimmune thyroid disease (for example, Hashimoto’s thyroiditis and Graves’ disease) in 15.8% of patients. Other autoimmune diseases included those affecting the blood vessels (vasculitis occurred in 10.5% of patients), skin (10.5%), gastrointestinal system (10.5%), lungs (7.2%), and kidneys (6.5%).
Ms. Abrol observed that patients who developed autoimmune diseases were more likely to be female than male, have anti-Ro/SS-A or Anti-La/SS-B antibodies, and hypergammaglobulinemia. Nineteen percent of patients had more than one autoimmune disease in addition to Sjögren’s, with one patient having four additional autoimmune diseases.
As expected, the most common type of malignancy seen was NHL, which occurred in approximately one in ten patients (10.5%). A range of other malignancies was seen, including ovarian and cervical (3.3%), bladder (2.6%), skin (2.6%), lung (2.0%), and oropharyngeal (2.0%) cancers, to name a few.
The risk for malignancy was higher in patients older than 50 years, with an odds ratio (OR) of 9.6 (P = .03). Patients who developed cancer were also more likely to be negative for Anti-RNP antibodies (OR, 4.9; P = .06).
The risk for NHL was increased in patients who had vasculitis (OR, 10.5; P less than .001) and in those with glandular manifestations (OR, 3.4; P = .041).
There were other numerous complications, including serologic alterations (51.3%), principally hypergammaglobulinemia, Raynaud’s syndrome (30.9%), and arthritis (19.7%). Peripheral and central nervous system complications were also seen in 15.8% and 9.2% of patients.
These data highlight that primary Sjögren’s syndrome carries a higher disease burden than previously suggested, Ms. Abrol concluded, noting that they can be used to inform patients on what may happen to them long-term.
Ms. Abrol had no conflicts of interest.