Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
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
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.
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 December 4, 2021.
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