Details

Autor(en) / Beteiligte

• Campar, Ana
• Isenberg, David A.
• Hassan, Nada
• Alsanjari, Nazar
• Gendi, Nagui
• Kiely, Patrick D.
• Higton, Alexandra M.
• McNulty, Katrina
• Vlahos, Ioannides
• Grubnic, Sisa
• Edwards, Elizabeth J.
• Chua, Felix
• Wedderburn, Lucy R.
• Varsani, Hemlata
• Charman, Susan C.
• Amato, Anthony A.
• Banwell, Brenda
• Bove, Kevin E.
• Corse, Andrea M.
• Emslie-Smith, Alison
• Jacques, Thomas S.
• Lundberg, Ingrid E.
• Marie, Suely
• Minetti, Carlo
• Nenesmo, Inger
• Rushing, Elisabeth J.
• Sewry, Caroline
• Pilkington, Clarissa A.
• Holton, Janice L.
• Dimitroulas, Theodoros
• Sidiropoulou, Eleni
• Tsavdaridou, Vasiliki
• Settas, Loukas
• Ratnaike, Tilly
• Pugmire, Susan
• Saravanan, Vadivelu
• Kelly, Clive
• Lavelle, Carolyn
• Maguire, Nicola
• McKinstry, Zoe
• Paton, David
• Murray, Eleanor
• Perry, Martin
• Field, Max
• Hadjinicolaou, Andreas V.
• Watson, Philippa A.
• Fang, Brian
• Hall, Frances C.
• Busch, Robert
• Rogers, Matthew
• Lloyd, Mark
• Hughes, Nicholas
• Ho, Timothy

Titel

Sjögren's Syndrome and Other Connective Tissue Disorders [213–222]

Ist Teil von

• Rheumatology (Oxford, England), 2010-04, Vol.49 (suppl-1), p.i115-i119

Ort / Verlag

Oxford University Press

Erscheinungsjahr

2010

Quelle

Oxford Journals 2020 Medicine

Beschreibungen/Notizen

• Background: In an attempt to provide tools to assess patients with primary Sjögren’s Syndrome (pSS) in clinical trials, several outcome measures have been developed. There are significant differences between them. We aimed to compare recent proposals of activity and damage indices to assess outcome in patients with pSS. Methods: We analysed three activity indices—SCAI (Sjögren’s Systemic Clinical Activity Index), SSDAI (SS Disease Activity Index) and ESSDAI (EULAR SS Disease Activity Index)—and two damage indices—SSDDI (SS Disease Damage Index) and SSDI (SS Damage Index)—focusing on the number of patients included, basis for the development and type of index, their validity, reliability and sensitivity to change and comparative strengthens and weaknesses. Proposals for the assessment of perspectives of outcome (PROFAD, Profile of Fatigue and Discomfort; SF-36, Short-Form 36-item questionnaire and ESSPRI, EULAR Sjögren’s Syndrome Patients Reported Index) were considered. A revision of the literature complemented the analysis. Results: The size of cohorts was different between the studies and the patients included had mainly mild stable disease. Most indices were developed on the basis of outcome measures already validated for other autoimmune diseases. SSDAI and ESSDAI are global scores. SCAI is a composite score. ESSDAI measures systemic objective features being complemented by ESSPRI for the assessment of sicca complaints and subjective items. SSDAI and SCAI used single national cohort-based populations with resulting main limitations in validity. ESSDAI demonstrated limitations in reliability and sensitivity to change. Both damage indices demonstrated low or no correlation with the activity scores but both have limitations in content validity. Their methodology was different with respect to observation period and external validation. SSDI differentiates between the damage secondary to pSS and that derived from co-morbidities, but does not stratify each item according to the severity of damaged organ as SSDDI does. Time to complete the forms was not evaluated for any of the indices. Unresolved issues remain including the distinction between activity and damage for some items (e.g. fatigue and lymphoma), whether or not to include the identified prognostic factors for an adverse outcome and an agreed definition of flare in pSS. Conclusions: All indices analysed demonstrated a potentially useful benefit in addition to the panel of exocrine function and subjective outcome measures currently available for patients with pSS. Further studies are needed to assess reliability and sensitivity to change, particularly in response to therapy in order to validate their use in clinical trials for new therapeutic approaches. Improvements in knowledge with respect to the pathophysiology and clinical evolution of Sjögren’s syndrome are important to address some unresolved issues: the most troubling but probably also the most interesting, being the dichotomy of activity vs damage. Disclosure statement: All authors have declared no conflicts of interest.