and La/SSB antibodiesÓ tend to restrict the diagnosis, excluding those with sicca symptoms, positive ocular and oral tests, and serological proÞles characterized by the ANA and RF positivity. For similar reasons, the AECG criteria may be too restrictive for use in daily clinical practice, particularly in early disease [40, 41]. The prevalence of Ro/SSA and La/SSB autoantibodies has been estimated to be respectively around 60% and 45% in pSS, these autoantibodies are particularly common in younger patients [42, 43]. Heavy reliance upon the minor salivary gland biopsy also creates potential problems, as the degree of lymphocytic inÞltration may be strongly inßuenced by the age or smoking and the reproducibility of the test is imperfect [44].

In conclusion, although the AECG criteria have been now widely adopted by the scientiÞc community, it is a common belief that some aspects of these criteria should be re-visited. ClassiÞcation criteria for SS remain a work in progress. The NIHsponsored longitudinal observational study known as the SjšgrenÕs International Collaborative Clinical Alliance (SICCA) is likely to provide critical information on SS and future efforts on criteria development [45, 46].

Acknowledgments We thank Miss Wendy Doherty and Miss Luisa Marconcini for their valuable contribution in reviewing the text.

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Chapter 30

Measurement of Chronicity and Activity

in Sjögren’s Syndrome

Claudio Vitali

30.3Assessment of Disease Activity or Damage in Systemic

30.1Introduction

Systemic autoimmune conditions such as Sjögren’s syndrome (SS) are commonly characterized by prolonged episodes of activity, sustained by the underlying immunologic and inflammatory processes [1, 2]. The activity flares are clinically marked by the new appearance or worsening of signs and symptoms that are typical for each one of the different diseases. In some diseases, elevation of acute phase reactants or abnormalities of immunologic markers accompany active periods. If the active phase of the disease does not remit spontaneously or if remission is not achieved by treatment, a chronic phase of the disease may begin and irreversible damage can be produced in the involved organs and systems [1, 2].

Although the concepts of disease activity and damage are easy to formulate in theory, functional definitions have not been established for most autoimmune

C. Vitali

Department of Internal Medicine and Section of Rheumatology,

‘Villamarina’ Hospital, Piombino, Italy