Subgroups of primary Sjogren's syndrome. Sjogren's syndrome in male and paediatric Greek patients

Abstract

Objectives-To describe the clinical and serological findings in male and paediatric Sjögren's syndrome (SS) patients. Patients and Methods-Using the European criteria for the diagnosis of SS 12 male and 13 paediatric patients were identified and compared with those of 30 consecutive unselected adult female SS patients. Results-The mean (SD) age of paediatric patients was 9.4 (2.2) years, ranging from 6 to 14 years. Recurrent parotid gland enlargement was the initial clinical manifestation in the majority of the children with a statistical significance compared with male (p<0.01) and with female patients (p<0.0001). Sicca manifestations were the most common clinical symptoms in male and female patients at disease onset. The systemic manifestations were similar among the three groups except that men showed lower frequency of arthritis (p<0.05) and Raynaud's phenomenon (p<0.05) compared with women. No diVerences were found among the immunological profile of children and female patients, while male patients had a lower frequency of antinuclear antibodies (p<0.025) and antibodies to Ro(SSA) nuclear antigens (p<0.025) compared with women. Conclusion-Primary SS is rare in children and men in Greece. Recurrent parotid gland enlargement is the most common clinical finding at disease onset in children. Male patients seem to have less systemic manifestations and lower frequency of autoantibodies. (Ann Rheum Dis 1997;56:333-335) Primary Sjögren's syndrome (SS) is a chronic autoimmune disorder aVecting the lacrimal and salivary glands, as well as other exocrine glands leading to functional impairment and mucosal dryness. It is often associated with systemic extraglandular manifestations, and laboratory tests show B cell polyclonal hyperactivity. It aVects predominantly women at middle age.

Primary Sjögren syndrome in the paediatric age: a multicentre survey

“…As previously reported, also in the paediatric age are females more commonly affected than males (M:F = 1:7). Recurrent parotid swelling represents the most important clinical manifestation at the onset of disease and can also be present during followup, while oral/ocular involvement is present in a smaller percentage of children, as already reported [2,6,8,9,10,16,20,23]. Moreover, we were able to detect unusual features of the disease such as renal tubular acidosis, central and peripheral nervous system involvement (meningoencephalitis, polyradiculoneuritis and tetraparesis),erythema annulare, Hashimoto thyroiditis, hepatitis, and Raynaud phenomenon.…”

“…SS most commonly affects middle-aged women and has been described very rarely in childhood, especially in its primary form [2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,23,24,25]. Our series is one of the largest published up to now on primary SS in childhood.…”

“…Previous studies have also shown that recurrent parotid gland enlargement is the most common manifestation of SS in children [2,6,8,9,10,13,16,17,20,23,24]. Drosos et al [8] showed recurrent parotid gland enlargement in 77% of the paediatric patients at the onset of the disease whereas sicca manifestations were the most common clinical symptoms in adults.…”

“…The mean age at onset is 40-50 years. The disease is rare in the paediatric age group and clinical manifestations might be different from the adult form [2,8,20]. Accurate information about clinical features and natural history of the disease in childhood are lacking.…”

Sjögrens syndrom

“…Sjögrens syndrom forekommer i alle aldersgrupper men er sjelden hos barn og unge 25,26 . Opplysninger om sykdomsdebut mangler i de større epidemiologiske studiene men er i autoritative verk angitt å opptre hyppigst mellom 40 og 60 år, noe som bekreftes ved gjennomgang av forskjellige kliniske sjögrenmaterialer 3,8,27,28 .…”