ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2390

Proposal of Assessment of  the Activity of Juvenile Localised Scleroderma. Results of the Consensus Meeting in Hamburg, Germany December 2015

Ivan Foeldvari1, Eileen Baildam2, Michael Blakley3, Christina Boros4, Kim Fligelstone5, Antonia Kienast1, Dana Nemkova6, Clare Pain2, Amanda Saracino4, Gabriele Simonini7, Kathryn S. Torok8, Lisa Weibel9 and Nicola Helmus1, 1Hamburg Center for Pediatric and Adolescent Rheumatology, Hamburg, Germany, 2Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, United Kingdom, 3Internal Medicine and Pediatrics, Indiana University School of Medicine and Riley Hospital for Children at IU Health, Indianapolis, IN, 4Pediatric Rheumatology, London, United Kingdom, 5Manchester Academic Health Science Centre, Centre for Musculoskeletal Research, Institute of Inflammation and Repair, The University of Manchester, Manchester, United Kingdom, 6Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic, 7Pediatric Rheumatology, Anna Meyer Children's Hospital, Florence, Italy, 8Pediatric Rheumatology, Univ of Pittsburgh Med Ctr, Pittsburgh, PA, 9Pediatric Dermatology, University Childrenxs Hospital, Zurich, Switzerland

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords:

Session Information

Date: Tuesday, November 15, 2016

Title: Pediatric Rheumatology – Clinical and Therapeutic Aspects - Poster III: Systemic JIA, Autoinflammatory Syndromes, Scleroderma, Vasculitis, Miscellaneous

Session Type: ACR Poster Session C

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

Background/Purpose:  Juvenile Localised Scleroderma (JLS) is an orphan disease which is complicated by difficulties in robust measurement of disease activity.   Several outcome measures to assess disease activity have been described with some recent trials using the mLoSSI (modified localized scleroderma skin severity index which is a validated cutaneous assessment tool in JLS. Whilst such measures are a step forward in assessment of this disease, they do not capture all aspects of the disease that are taken into consideration by clinicians when judging the degree of disease activity and therefore making decisions regarding treatment. In particular extra-cutaneous manifestations are poorly captured. With potential innovative and more effective treatment options emerging, it has become extremely important to define a validated activity index that captures skin, extra-cutaneous disease activity and patient reported outcomes in order to monitor response to treatment.

Methods: Members of the PRES Scleroderma working group and other paediatric rheumatologists and dermatologists interested in JLS met to develop an activity index using the nominal group technique in a consensus meeting in Hamburg, Germany, in December 2015. Eithy percent  agreement was chosen for the selected  domains and items.

Results: Summarises the proposed domains and items to assess the activity of localised scleroderma. Skin:   Change in skin thickening – Modified Rodnan Skin Score (mRSS); Total Skin thickness score of mLoSSi; Research tool: A, durometer B, Ultrasound Degree of change in white waxy appearence since last visit – Marked worsening /some worsening and  some improvemnet/no change/ significant improvement Change in Erythema/violaceousness since last visit – mLoSSI Change in Subcutaneous induration since last visit – Yes or no & elements of mLoSSi Enlargement of the lesion since last visit – Yes or no (captured within mLoSSi) New lesion per anatomical region of mLoSSI – mLoSSI Worsening alopecia (eyelash, beard, body hair) – marked worsening,/some worsening and  some improvement/no change/ significant improvement/no hair in the lesion / decreased Increase in face atrophy – only Parry Romberg patients – 3 D photography Optional item: Change in blood flow in the lesion – Ultrasound with doppler / laser doppler Extracutaneous involvement: Change in Activity of anterior uveitis since last visit – Yes or no Change in active joint count – Number of active joints Worsening of lim discrepancy – Yes or no Active CNS involvement – Yes or no Patient reported outcomes: Change in subcutaneous induration since last visit – marked worsening /some worsening and  some improvemnet/ no change/ significant improvement Uncomfortable feeling in the lesion (not including itch) – VAS score 0-100 Itching in the lesion – VAS score 0-100 Quality of life assessment – PEDsQL, CHQ, CDLQII Functional assessment of daily life – CHAQ/HAQ

Conclusion : 

We propose extended domains to define an activity index for JLS which aim to capture the heterogeneity of disease activity.  This may become an important instrument for evaluating treatment efficacy but requires prospective validation from the PRES and CARRA scleroderma working groups.

Disclosure: I. Foeldvari, None; E. Baildam, None; M. Blakley, None; C. Boros, None; K. Fligelstone, None; A. Kienast, None; D. Nemkova, None; C. Pain, None; A. Saracino, None; G. Simonini, None; K. S. Torok, None; L. Weibel, None; N. Helmus, None.

To cite this abstract in AMA style:

Foeldvari I, Baildam E, Blakley M, Boros C, Fligelstone K, Kienast A, Nemkova D, Pain C, Saracino A, Simonini G, Torok KS, Weibel L, Helmus N. Proposal of Assessment of  the Activity of Juvenile Localised Scleroderma. Results of the Consensus Meeting in Hamburg, Germany December 2015 [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/proposal-of-assessment-of-the-activity-of-juvenile-localised-scleroderma-results-of-the-consensus-meeting-in-hamburg-germany-december-2015/. Accessed .

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