antibodies in patients with high-risk markers can alter the clinical and serological negative prognostic factors. However, large prospective studies are needed to determine whether anti-CD20 treatment in the poor prognosis group of patients has a preventive effect for lymphoma development.

34.4Conclusions

Careful clinical evaluation (parotid gland enlargement, palpable purpura) and routine blood testing (hypocomplementemia, cryoglobulinemia) at the first patient evaluation can identify a subgroup of patients at risk for higher morbidity and lymphoma development. Overall mortality of pSS patients do not differ significantly from ageand sexmatched unaffected individuals. However, pSS patients with lymphoma have higher morbidity and mortality.

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Chapter 35

Primary Sjögren Syndrome in Primary

Health Care

Antoni Sisó-Almirall, Jaume Benavent, Xavier Bosch,

Albert Bové, and Manuel Ramos-Casals

Contents

A.S. Almirall (*) • J. Benavent

Research Group on Primary Care, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), CAP Les Corts, GesClínic, University of Barcelona, Barcelona, Spain

X. Bosch

Department of Internal Medicine, ICMiD, Hospital Clínic, Barcelona, Spain

A. Bové • M. Ramos-Casals

Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Spain

35.1Introduction

Sjogren’s syndrome (SjS) is a slowly progressive autoimmune disease that predominantly affects middle-aged women, with a female to male ratio on the order of 9:1. SS is characterized by lymphocytic infiltration of the exocrine glands, mainly the lacrimal and salivary glands, resulting in reduced secretory function and oral and ocular dryness. The syndrome can present alone as primary SjS (pSjS) or in the context of underlying connective tissue disease as secondary SjS (sSjS). Although the pathogenesis of the disease remains elusive, environmental, genetic and hormonal factors all appear to contribute [1]. The clinical manifestations of pSjS patients are mainly those of an autoimmune exocrinopathy, but almost half of patients develop extraglandular disease, which may be manifested by liver, lung, kidney, skin, or nervous system problems. In addition, the disease spectrum of SjS can also be complicated by the development of lymphoma. Patients with pSjS present a broad spectrum of laboratory (cytopenias, hypergammaglobulinemia, elevated erythrocyte sedimentation rate) and serological features, of which antinuclear antibodies (ANA) are the most frequently detected. Anti-Ro/SS-A antibodies are the most specific autoantibody. Cryoglobulins and hypocomplementemia are the main prognostic markers [2].

35.2General Considerations

Primary care is the first level of access to the health care system for patients and the main entry point for public health services. Primary care, underpinned by the attributes espoused by Alma-Ata (accessibility, continuity, longitudinality, quality and efficiency), includes preventive and curative care, health promotion, and health education [3]. Problems such as oral or ocular dryness present to community-based primary care practitioners very differently than they do to specialists. The prevalence and incidence of illnesses are different from those that occur in hospital settings and serious disease presents less often in general practice because there is no prior selection. Choices about test ordering require a probability-based decisionmaking process that is informed by a knowledge of patients and the community [4]. The positive and negative predictive values of oral or ocular dryness and of immunological tests carry different weights in primary care as opposed to the hospital or specialist settings. Primary care practitioners often must reassure individuals with anxieties about illness after determining that the likelihood of illness is low [5].

The diagnosis of SjS is difficult at early stages of the disease, when patients present with nonspecific complaints and findings such as ocular or oral dryness, conjunctivitis, corneal damage, dental disease, oral ulcerations, angular cheilitis, fatigue or other general symptoms [6]. Thus, important decisions must be undertaken on the basis of limited information in a setting in which the predictive values of clinical examination