Joint Bone Spine 78 (2011) 171–174 Original article Elderly 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 Costantino Botsios a,∗ , Antonio Furlan a , Pierantonio Ostuni a , Paolo Sfriso a , Marilisa Andretta b , Francesca Ometto a , Bernd Raffeiner a , Silvano Todesco a , Leonardo Punzi a a Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Padova, via Giustiniani 2, 35128 Padova, Italy b Department of Otolaryngology-Head and Neck Surgery, University of Padova, Padova, Italy article info Article history: Accepted 20 May 2010 Keywords: Sjögren’s syndrome Elderly onset Salivary glands Autoimmune diseases Xerostomia Dry eye abstract Objectives: To study and compare the clinical and serological features of patients with elderly versus adult and younger onset of primary Sjögren’s syndrome (pSS). Methods: We analyzed retrospectively 336 consecutive pSS patients followed at our unit. They were subdivided into three groups according to the age at disease onset: elderly (> 65 years), adult (> 40 and ≤ 65 years), and young (≤ 40 years). Clinical and immunological features of the disease, labial salivary glands biopsy, ocular and oral tests were collected at time of diagnosis and then compared among the three groups. Results: In 21 (6%) patients, disease onset occurred after the age of 65 years. At the time of diagnosis, 15 (71.4%) of these patients reported symptoms of dry mouth and 16 (76.1%) of dry eye. The most common extraglandular manifestation were arthralgias in 14 (66.7%), Raynaud’s phenomenon in five (23.8%) and purpura in three (14.2%) cases. Ocular diagnostic tests (Schirmer’s I and Rose-Bengal staining) were positive respectively in 17 (80%) and nine (44.4%) patients. In eight (38%) cases, unstimulated whole salivary flow showed normal values, while 12 patients (57.1%) showed positivity for salivary sialography. A focus score greater or equal to 1 per 4 mm 2 was demonstrated in 11 (53.3%) of the 21 cases. Conclusion: Elderly onset of pSS was associated with similar incidence of the diagnostic tests positivity (parotid sialography, ocular tests, minor salivary gland biopsy) in comparison with adult and younger onset. Moreover, no statistical differences were found among the three groups concerning sex, disease duration, as well as ocular and oral symptoms. © 2010 Société franc ¸ aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved. 1. Introduction Sjögren’s syndrome (SS) is a chronic autoimmune exocrinopa- thy 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 sys- temic process that may involve the musculoskeletal, pulmonary, gastrointestinal, hematologic, vascular, dermatologic, renal, or nervous systems [1–3]. SS can occur alone (primary) or in asso- ciation with another autoimmune rheumatic disease (secondary). Primary SS (pSS) affects 0.3–5% of the population, the propor- tion being determined by the age group studied and by the diagnostic criteria used [4,5]. The disease can occur in patients ∗ Corresponding author. Tel.: +39 049 8212190; fax: +39 049 8212191. E-mail address: constantin.botsios@unipd.it (C. Botsios). 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 mod- ifying 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 immuno- logical characteristics [11,12]. To our knowledge, only few studies have been published so far on the clinical and immunological fea- tures of pSS in the elderly [13,14]. To better determine whether any relation exists between dis- ease manifestations and age at onset, we analyzed, in a series of 336 unselected patients, clinical manifestations and immunoserologi- 1297-319X/$ – see front matter © 2010 Société franc ¸ aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved. doi:10.1016/j.jbspin.2010.05.008