{
  "abstract": "Introduction The assessment of disease activity in Juvenile Localized cutaneous scleroderma (JLS) is challenging due to the lack of standardized outcome measures. Clinical assessment is performed with the Localized Scleroderma Assessment Tool (LOSCAT) (evaluating disease activity (mLOSSI) and damage (LOSDI)) and with Infrared termography (IRT). Recently, also skin high frequency ultrasound (US) in the assessment of disease activity has been proposed. The objective of this work is therefore to compare the ability of LOSCAT, IRT and skin US to assess disease activity in JLS patients.Material and Methods JLS patients followed at the Rheumatology Unit of the Gaslini Hospital were evaluated retro-prospectively. Patients were diagnosed according to 2006 Padua Classification criteria. A minimum follow-up of 6 months was required for inclusion. All patients were evaluated with LOSCAT, IRT (Lesions considered positive when warmer 0.5 °C than surrounding area or contralateral limb) and high frequency US (20 MHz probe, parameters of activity: increased hypodermal echogenicity or loss of dermo-hypodermal borders and increase of dermal and/or hypodermal vascularization, with signs of increased blood arterial or venous flow at color doppler with/without increased velocity > 2 cm/s).Results 7 patients were enrolled (4 male), with a mean age at disease onset of 7.4 years, and at enrolment of 11.95 years. 3 patients presented a circumscribed JLS, while 4 had a mixed type with linear and circumscribed JLS. All patients were off therapy at time of enrolment (in 2 cases therapies discontinued previously). 4 patients had an active disease at enrolment. All patients received an initial steroid course of 2mg/kg and methotrexate (MTX). Disease relapse on MTX therapy was documented in 2 patients, who were switched to mophetil micofenolate. A concordance between mLOSSI, IRT and US was found in 5 cases (71%). In 2 patients (28.6%) IRT and US showed a disease activity, with a negative mLOSSI, thus allowing therapy reintroduction/dose adjustment. In one patient mLOSSi reduced and IRT remained negative while US showed a disease activity. Specifically, the patients with a disease activity presented all an irregularity of the dermo- hypodermal junction and signs of an increased vascularization, with an increased peak velocity.Conclusions There was a good concordance in LOSCAT, IRT and high-frequency US in the assessment of disease activity, even if US and IRT were more efficient than LOSCAT alone in early detection of disease activity. Further studies on larger cohorts of patients are needed to confirm these preliminary findings obtained with three different outcome measures.",
  "authors": [
    {
      "affiliations": [
        "IRCCS Istituto Giannina Gaslini, Rheumatology and Autoinflammatory diseases, Genova, Italy"
      ],
      "name": "Caterina Matucci-Cerinic"
    },
    {
      "affiliations": [
        "IRCCS Istituto Giannina Gaslini, Rheumatology and Autoinflammatory diseases, Genova, Italy"
      ],
      "name": "Saverio La Bella"
    },
    {
      "affiliations": [
        "IRCCS Istituto Giannina Gaslini, Dermatology department, Genova, Italy"
      ],
      "name": "Gianmaria Viglizzo"
    },
    {
      "affiliations": [
        "IRCCS Istituto Giannina Gaslini, Radiology department, Genova, Italy"
      ],
      "name": "Luca Basso"
    },
    {
      "affiliations": [
        "IRCCS Istituto Giannina Gaslini, Rheumatology and Autoinflammatory diseases, Genova, Italy"
      ],
      "name": "Marco Gattorno"
    },
    {
      "affiliations": [
        "IRCCS Istituto Giannina Gaslini, Rheumatology and Autoinflammatory diseases, Genova, Italy"
      ],
      "name": "Clara Malattia"
    }
  ],
  "title": "P.213 Comparison of localized scleroderma assessment tools, teletermography and ultrasound in the evaluation of juvenile localized cutaneous scleroderma: preliminary data of a monocentric study",
  "uid": "89ddac41-bc04-579a-b71f-131ff1500937"
}