Primary and Secondary Sjogren-Jones Syndromes—Historical Evolution

Abstract

©2011 Ethis Communications, Inc. The Ocular Surface ISSN: 1542-0124. Murube J. Primary and secondary SjogrenJones syndromes—historical evolution. 2011;9(1):13-16. ccording to Norman Talal, the history of Sjogren syndrome (SS) can be divided into three phases: clinical (1888-1950), immunologic, and molecular.1 The concept of primary and secondary Sjogren syndrome developed during the transition between the second and third phases. In the last 50 years, since the publications of Jones,2 Bunim,3 and Bloch et al,4 most clinicians and researchers have accepted that SS refers to SS of autoimmune etiopathogenesis, or Sjogren-Jones syndrome. The recognition of the autoimmune basis of the disease opened a new clinical approach.5 The polymorphism of the SSs stimulated several classifications and subclassifications. In 1961, Sjogren6 had already accepted the possible auto-immunologic cause of the sicca syndrome suggested by Jones, Bloch et al, and Bunim,2-4 and he determined that the autoimmunopathies should be divided into collagenoses and adenopathies and that these groups could exist alone or together in various combinations. Clinical differences in SS in the absence or presence of rheumatoid arthritis and of systemic lupus erythematosus had been noted by many clinicians.7-21 The division into primary and secondary SSs had many antecedents, and in 1979 became universally accepted with the publications of Moutsopoulos.22,23 In medical terminology, primary and secondary designations may have several different bases, and have been variously used. In the case of SSs, primary does not mean that it is the origin of the secondary or that it is of more importance than the secondary. In the current terminology of SS, primary and secondary refer to two different etiopathogenic groups or types. De Cremoux et al preferred to say limited rather than primary SS in order to avoid the confusion of the terms.24 Ancochea et al preferred to name these two groups primary and associated SS, the latter being associated with autoimmune diseases and connectivopathies.25 The Triple Classification of Dry Eye proposed describing secondary SS as “SS associated with...” followed by the specific association. This is because sometimes this association is not autoimmunologic, but derives from hormonal, viral, or other causes, or is simply coincident with SS exocrinopathy.26,27 Some authors consider this expression the most appropriate to describe the clinical profile of an SS patient.28