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dryness, pain, and fatigue, but not mental fatigue, were signiÞcantly associated with PtGA. Thus, ESSPRI was redeÞned as the mean of the three scales: dryness, pain, and fatigue.
In summary, to date, the Þrst part of the EULAR project has been completed and the ESSDAI and the ESSPRI have been developed as recently reported in three different papers [54Ð 56]. At the present, in order to adopt the ESSPRI and ESSDAI for assessing the therapy effectiveness in clinical trials, the validation of these scoring systems is ongoing. ESSDAI and ESSPRI still require to be validated in terms of feasibility, face and construct validity, reliability, and sensitivity to change and this phase of the EULAR project has recently begun.
In conclusion, after the achievement of a general consensus on the more recently proposed ÒAmerican and European Consensus Group classiÞcation criteria,Ó nowadays, it is to be hoped that efforts continue. The ultimate goal will be the elaboration of validated Òdisease statusÓ indexes to better understand the natural history, prognosis, functional consequences, and response to treatment of SS. The project is ongoing at the moment; nonetheless, it is likely that in a near future internationally accepted outcome measures will be available for SS as they are for many of the other systemic autoimmune diseases.
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Histopathology and Glandular |
6 |
Biopsies in Sjögren’s Syndrome |
Roland Jonsson, Kathrine Skarstein,
and Malin V. Jonsson
Abstract
The glandular inßammatory lesion in SjšgrenÕs syndrome (SS) is a distinctive but not pathognomonic chronic lymphocytic adenitis best characterized in salivary glands. The glands are readily accessible; decreased function gives rise to prominent clinical symptoms and signs; and the glands are affected in almost all patients. A labial salivary gland biopsy specimen can be very disease speciÞc for SS if it is obtained through normal-appearing mucosa, includes ≥5 separate glands separated from their surrounding connective tissue, is interpreted after lobes or glands showing non-speciÞc changes are excluded, demonstrates focal sialadenitis in all or most of the glands in the specimen, and has a focus score that provides a diagnostic threshold. Such glandular biopsies can also provide tissue diagnosis for conditions that can resemble SS clinically, particularly sarcoidosis and amyloidosis. The characteristic pathologic lesion is part of classiÞcation criteria for SS and probably provides the best single criterion in terms of its disease sensitivity and speciÞcity, convenience, availability, and low risk. More recently, studies have found germinal center reactions in the glandular biopsies indicating a more severe disease phenotype.
Keywords
Acinar ¥ Chronic inßammation ¥ Degeneration ¥ Ductal ¥ Fibrosis ¥ Focus score ¥ Focal sialadenitis ¥ Lymphocytes ¥ Mononuclear cells ¥ Salivary gland
R. Jonsson ( )
Broegelmann Research Laboratory, The Gade Institute, University of Bergen, Bergen, Norway; Department of Rheumatology, Haukeland University Hospital, Bergen, Norway
e-mail: roland.jonsson@gades.uib.no
6.1Introduction
All organs affected by SjšgrenÕs syndrome (SS) display a potentially progressive mononuclear lymphoid cell inÞltration. These circumscribed and most often well-deÞned inÞltrates presumably give rise to functional derangements of
R.I. Fox, C.M. Fox (eds.), Sjögren’s Syndrome, DOI 10.1007/978-1-60327-957-4_6, |
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