Aggressive Treatment, Monitoring Key in Early-onset Sjögren’s, Study Suggests

People with early-onset primary Sjögren’s syndrome may need more intensive glucocorticoid and immunosuppressant therapy, closer follow-up, and regular monitoring than is customary, due to greater immune system activation and a less favorable prognosis than patients with later-onset disease, a study suggests.

The study, “Patients with early-onset primary Sjögren’s syndrome have distinctive clinical manifestations and circulating lymphocyte profiles,” was published in Rheumatology.

As with many autoimmune disorders, Sjögren’s syndrome manifests differently in each person, with symptoms often presenting at various points of development, rather than all at once or in the same sequence. This has led scientists to study subsets of people with the disorder — such as different age groups — for evidence of distinct clinical patterns.

Although Sjögren’s typically appears in women after age 40, it can be diagnosed earlier and in men. Early-onset disease occurs in an estimated 11.4% to 19% of all patients, and few studies have explored the relationship between age of onset and both clinical and laboratory findings.

To better understand whether such a relationship exists and to characterize it, researchers with Heibei General Hospital and Peking Union Medical College Hospital, both in China, examined data gathered on 333 Sjögren’s syndrome patients (310 females and 23 males).

A total of 36 patients (including 35 women) met the criteria for early-onset pSS, with an average age at diagnosis of 28.97 years and a mean disease duration of two years. In contrast, people in the later-onset group were diagnosed at a mean age of 56.95, and had the disease for five years.

Overall, individuals diagnosed at younger ages showed more immune system activation, more severe disease activity and symptoms, and unfavorable prognoses.

Compared with later-onset cases, those with early-onset involved higher levels of the immune system activity markers and greater amounts of SS-A and SS-B, the autoantibodies most commonly associated with Sjögren’s syndrome.

The blood, kidneys, skin, and mucus membranes of people in this group were also involved significantly more often than those of the later-onset group.

People with early-onset disease also had significantly lower levels of certain immune cells — helper T-cells and NK cells — which  correlated with higher disease activity, as measured with the European League Against Rheumatism Sjögren’s Syndrome Disease Activity Index (ESSDAI).

Immunosuppressant therapy differed little between the two patient groups, but those with early-onset disease tended to receive corticosteroids (prednisone/methylprednisolone) more often, and more of them were prescribed mycophenolate mofetil.

Yet, while both groups experienced a significant reduction in their disease activity scores over about 30 months of follow-up, early-onset individuals still recorded higher ESSDAI scores at their last follow-up than did later-onset patients.

Furthermore, more early-onset patients developed systemic lupus erythematosus, although the difference was not deemed statistically significant.

Four later-onset patients died, with one death due to disease complications.

Because of the distinct characteristics of early-onset cases, featuring greater cellular immune system activation, more severe clinical symptoms, and a worse prognosis, the investigators concluded that this patient subset requires “more intensive treatment with glucocorticoids and/or immunosuppressants and stricter monitoring.”