ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2992

Identification of a New Set of Activity Criteria for Systemic Sclerosis. Preliminary Report

Gabriele Valentini1, Michele Iudici2, Veronika K. Jaeger3, Christopher P. Denton4, Oliver Distler5, Otylia M. Kowal-Bielecka6, Ulf Müller-Ladner7, Gabriela Riemekasten8, Ulrich A. Walker3, Suzana Jordan5, Elise Siegert8, Serena Vettori2 and Yannick Allanore9, 1Department of Clinical and Experimental Medicine, Rheumatology Unit, Rheumatology Unit, Second University of Naples, Naples, Italy, 2Department of Internal and Experimental Medicine, Rheumatology Unit, Second University of Naples, Naples, Italy, 3Department of Rheumatology, University Hospital Basel, Basel, Switzerland, 4Rheumatology and Connective Tissue Diseases, University College London, London, United Kingdom, 5Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 6Department of Rheumatology and Internal Medicine, Medical University in Bialystok, Bialystok, Poland, 7Internal Medicine and Rheumatology, Justus-Liebig-University of Giessen, Kerckhoff-Klinik, Bad Nauheim, Germany, 8Rheumatology and Clinical Immunology, Charité – University Hospital, Berlin, Berlin, Germany, 9Department of Rheumatology, University Paris Descartes and Cochin Hospital, Paris, France

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: ,

Session Information

Date: Tuesday, November 10, 2015

Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's - Clinical Aspects and Therapeutics Poster III

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: The European Scleroderma Study Group (EScSG) Activity Index developed in 2001 has some limitations due to the procedure underlying its development and the inclusion of hypocomplementemia, that was criticized for its poor face validity. The present study is devoted to overcome such limitations.

Methods: Ninety seven clinical charts of patients with SSc, all of whom satisfying 1980 ACR criteria for the disease, were retrieved from a large database. Three  investigators (CD, OKB, YA) external to the enrolling centres assigned a disease  activity score to each chart on a 0-10 visual analogue scale, providing a reliable scoring tool (ICC=0.786; 95%CI=0.716-0.844). The median score served as the disease activity gold standard. Another investigator (GV)  evaluated disease activity as inactive, moderately active, active, and very active. Univariate and multivariate linear regression analyses were used to assess the performance of sets of criteria in predicting the “gold standard” and to identify the  weight of each variable. Pending the final validation in a replication cohort, the resulting preliminary activity index was validated by the jackknife statistical procedure i.e.by leaving out one patient a time. Receiver operating curves served to assess the efficiency of the index in separating active-very active disease from inactive-moderately active disease.                                                                                           

Results: A preliminary weighted 10-point activity index was identified: Δ (ie. patient-assessed worsening during the previous month)-skin= 1.25, Δ- heart-lung= 0.75, modified Rodnan skin score >18=1.25;  digital ulcers=1.75; tendon friction rubs=1.5; erythrocyte sedimentation rate >50 mm/h=1.0; C reactive protein >1 mg/dl= 1.5; Diffusing Lung Capacity for CO <70% of predicted=1.  A cut-off ≥1.75 identified patients with active-very active disease. It resulted to be more sensitive and specific  than the currently used EScSG index in identifying SSc patients with active disease in the 97 SSc patients considered for this part of the study.  

Conclusion: A preliminary revised  activity index has been developed. It is feasible and  has face validity. It is going to be validated in a replication cohort

Disclosure: G. Valentini, None; M. Iudici, None; V. K. Jaeger, None; C. P. Denton, GlaxoSmithKline, 2,Actelion Pharmaceuticals US, 5,GlaxoSmithKline, 5,Serono, 5,Inventiva, 5,CSL Behring, 2,Bayer, 5; O. Distler, Consultancy relationships and/or has received research funding from Actelion, Pfizer, Ergonex, BMS, Sanofi-Aventis, United BioSource Corporation, Medac, Biovitrium, Boehringer Ingelheim, Bayer Pharma AG, Novartis, 4D Science and Active Biotech in the area, 2; O. M. Kowal-Bielecka, None; U. Müller-Ladner, None; G. Riemekasten, None; U. A. Walker, None; S. Jordan, None; E. Siegert, None; S. Vettori, None; Y. Allanore, None.

To cite this abstract in AMA style:

Valentini G, Iudici M, Jaeger VK, Denton CP, Distler O, Kowal-Bielecka OM, Müller-Ladner U, Riemekasten G, Walker UA, Jordan S, Siegert E, Vettori S, Allanore Y. Identification of a New Set of Activity Criteria for Systemic Sclerosis. Preliminary Report [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/identification-of-a-new-set-of-activity-criteria-for-systemic-sclerosis-preliminary-report/. Accessed .

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