Final validation studies of both the ESSDAI and ESSPRI in a large series of real patients are under way. When this ongoing phase of the project is finished, valid and reliable instruments to assess activity in SS will finally be available for clinical trials and practice.

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Chapter 31

Measurement of Quality of Life in Primary

Sjögren’s Syndrome

Simon J. Bowman and Wan-Fai Ng

31.1Introduction

Primary Sjögren’s syndrome (PSS) is an immune-mediated rheumatic disease in which inflammation of secretory (exocrine) glands leads to dry eyes and dry mouth [1]. Dryness of other surfaces such as the skin, vagina, airways, and gastrointestinal tract also occurs. The secretory glands are infiltrated by collections (focal aggregations) of lymphocytes. In the salivary glands, these focal lymphocyte aggregations are typically clustered around the salivary ducts. Patients typically also complain of reduced well-being, fatigue, and arthralgia. Approximately three-quarters of patients have autoantibodies in their blood – anti-Ro and/or anti-La antibodies – and a majority also have elevated total immunoglobulin levels (hypergammaglobulinaemia). Aproportionof patientshaveother systemicorganinvolvementincludingneuropathies,

S.J. Bowman (*)

Rheumatology Department, University Hospital Birmingham (Selly Oak), Birmingham, UK

W.-Fai Ng

Musculoskeletal Research Group, Institute of Cellular Medicine, Newcastle University,

Newcastle Upon Tyne, UK

skin rashes (purpura, vasculitis), interstitial lung disease, renal tubular acidosis, and hematological abnormalities. Finally, patients with PSS are at substantially increased risk for the occurrence of lymphomas, particularly mucosa-associated lymphoid tissue (MALT) B-cell lymphomas. In this chapter, we assess the issue of health-related quality of life (QoL) in PSS.

“Quality of life” is a broad concept that attempts to describe the general wellbeing of individuals and societies [2]. QoL studies often focus particularly on economic well-being. As an example, in terms of QoL within countries, the Economist’s 2005 QoL Index (http://www.economist.com/media/pdf/QUALITY_OF_LIFE.pdf) scores a country’s QoL according to scores on nine domains: health (life expectancy at birth), family life (divorce rate), community life (e.g., church attendance, trade union membership), material well-being (gross domestic product), political stability and security, climate and geography (latitude), job security (unemployment rate), political freedom, and gender equality (ratio of average male:female earnings). The World Health Organization (WHO) defines QoL as “individuals’ perceptions of their position in life in the context of the culture and value systems in which they live and in relation to their goals, expectations, standards and concerns” (http:// www.who.int/mental_health/media/68.pdf). Domains include a person’s physical health, psychological state, level of independence, social relationships, personal beliefs and their relationship to salient features of their environment. The WHOQOL Group has developed questionnaires to measure generic QoL: the initial (WHOQOL-100) [3] and the shorter (26 item) WHOQOL-BREF [4]. The principal domains of the WHOQOL are of physical health, psychological well-being, social relationships, and environmental factors. The questionnaires also include questions pertaining to an individual’s global QoL – “how would you rate your quality of life?” – and also introduce the generic concept of “health-related quality of life (HRQoL)”; for example: “how satisfied are you with your health?”. The WHOQOL questionnaires were designed and piloted in centers around the world and in individuals with various states of health and illness. They are intended to measure QoL independently of cultural constraints.

The line between the concepts of “QoL” and “HRQoL” is often blurred. For example, HRQoL typically focuses on aspects of physical and psychological wellbeing and the consequences of ill-health on social relationships. The environment may have a major impact on individuals and populations quality of life but only indirectly on health status. In the context of evaluating a disease state such as PSS, the term HRQoL is more widely used in most papers but in some the terms HRQoL and QoL are used interchangeably. To some extent, a convention has developed whereby questionnaires such as the WHOQoL measure QoL and other questionnaires (discussed below) measure HRQoL.

QoL/HRQoL measures fall within the broader concept of “patient-reported outcome measures” (PROMs). These are patient-completed questionnaires that encompass symptom questionnaires as well as QoL measures. Some are generic and hence widely applicable, but others are specific to particular conditions or patient groups. They can be used in a number of situations, including clinical trials, economic evaluations, and routine clinical care. Their value is that they complement more