Original articleElderly onset of primary Sjögren's syndrome: Clinical manifestations, serological features and oral/ocular diagnostic tests. Comparison with adult and young onset of the disease in a cohort of 336 Italian patients
Introduction
Sjögren's syndrome (SS) is a chronic autoimmune exocrinopathy characterized by dryness of eyes (keratoconjunctivitis sicca) and mouth (xerostomia) as well as recurrent parotid gland swelling. The spectrum of SS extends from an organ-specific autoimmune disorder (autoimmune exocrinopathy) to a systemic process that may involve the musculoskeletal, pulmonary, gastrointestinal, hematologic, vascular, dermatologic, renal, or nervous systems [1], [2], [3]. SS can occur alone (primary) or in association with another autoimmune rheumatic disease (secondary). Primary SS (pSS) affects 0.3–5% of the population, the proportion being determined by the age group studied and by the diagnostic criteria used [4], [5]. The disease can occur in patients of all ages, but mainly affects women in the forth and fifth decade of life, with a female:male ratio of 9:1 or even more [4].
Several investigators have reported that age at onset has a modifying effect on disease expression of some autoimmune diseases, such as systemic lupus erythematosus (SLE) [6], [7] and rheumatoid arthritis (RA) [8]. In SLE, certain clinical features usually associated with severity, such as nephropathy, are less common in patients with an older onset [7] and serological abnormalities have also been reported to be different in elderly patients [7], [9], [10]. Also in pSS, juvenile (< 16 years) and young (< 35 years > 16) onsets of the disease have been associated with distinctive clinical or immunological characteristics [11], [12]. To our knowledge, only few studies have been published so far on the clinical and immunological features of pSS in the elderly [13], [14].
To better determine whether any relation exists between disease manifestations and age at onset, we analyzed, in a series of 336 unselected patients, clinical manifestations and immunoserological findings of patients with an elderly age at onset (age ≥ 65 years) in a retrospective designed study.
Section snippets
Methods
Three hundred and twenty-two female and 14 male outpatients with pSS consecutively attending our Rheumatologic Unit between May 1990 and December 2007 were studied retrospectively. The mean age and disease duration (time from onset of symptoms at the time of the diagnosis) were (M ± SD): 47.8 ± 14.5 years, range: 17–84 years and 4.3 ± 3.0 years, range: 1–22 years, respectively. The mean follow-up duration (time from the diagnosis up to the start of the study) was (M ± SD): 12.2 ± 7.1 years, range: 1.1–22 years.
Results
Demographic characteristics and clinical manifestations, immunohaematological profile as well as ocular and salivary involvement of elderly onset pSS patients are shown in Table 1, Table 2, Table 3 along with those of patients with adult and young disease onset.
In 21 (6%) patients, disease onset occurred after the age of 65 years. In this group, 20 (95%) were female and one (5%) male. At the time of the study, 15 (71.4%) of these patients reported symptoms of dry mouth and 16 (76.1%) of dry eye.
Discussion
Primary SS is a common autoimmune disorder of adults, especially of the middle-age women. Although pSS commonly appears in the fifth decade of life [4], our study, performed in a large cohort of patients, shows that an elderly onset of the disease (≥ 65 years) is not rare (6% of our patients).
In this study, we confirmed and extended the observations made by other authors [13]: the clinical expression of primary SS in elderly-onset patients is quite similar to the disease in the remaining
Conflict of interest statement
None of the authors has any conflicts of interest to declare.
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