{
  "abstract": "Introduction Context: Systemic sclerosis (SSc)-related hand involvement significantly impairs quality of life. The natural history of calcinosis and acro-osteolysis in SSc hands remains poorly characterized, and longitudinal data on risk factors for progression are limited. Ultrasound (US) enables the assessment of macrovascular involvement and finger pulp blood flow (FPBF), offering potential insights into disease mechanisms.Objectives: To identify baseline clinical, ultrasound, and capillaroscopic predictors of radiographic progression of calcinosis and acro-osteolysis in SSc patients undergoing serial hand X-rays as part of routine care.Material and Methods We included SSc patients from a tertiary center who fulfilled ACR/EULAR criteria, underwent baseline power Doppler US, and had at least two hand X-rays. Acro-osteolysis was scored using the Johnstone classification. Calcinosis was assessed with a dedicated scoring system, dividing each hand into 8 regions (score 0–3, from no calcinosis to ensheathing calcinosis). Distal phalanx calcinosis was additionally scored (0–2). This system showed excellent inter- (0.90) and intra-observer (0.98) reproducibility. All X-rays evaluations were assessed by the same reader. All PDUS were performed by the same evaluator, blinded from the result of X-ray evaluations.Results Of 172 patients with baseline X-rays and PDUS from our center, 84 patients (48,8%) with two serial X-rays allowing longitudinal assessment of calcinosis and acro-osteolysis were included. Twenty-nine (N=29/84; 34,5%) showed a worsening of the Johnstone score (new or progressive lesions). In multivariate analysis, the independent baseline predictors of progression were the history of digital ulcers (OR= 8.23 (2.50-27.07), p=0.001), the positivity for anti-centromere antibodies (OR= 4.97 (1.53-16.12), p=0.008). Regarding calcinosis, 32 patients (N=32/84, 38,1%) had worsening of the calcinosis score (new or progressive lesions). In multivariate analysis, the independent baseline predictors of progression of calcinosis were higher disease duration (OR=3.22 (1.08-9.69), p=0.038), presence of telangiectasia (OR=4.38 (1.03-18.76), p=0.046), a higher rodnan skin score (OR=3.41 (1.04-11.21), p=0.043) and the presence of a pathological FPBF (OR=3.18 (1.04 -9.74), p=0.04). Ulnar artery occlusion and abnormal capillaroscopy were associated with the progression of calcinosis in univariate analysis but not in multivariate analysis.Conclusions This longitudinal study demonstrates that markers of severe vasculopathy predict the progression of both acro-osteolysis and calcinosis in SSc. These findings highlight the value of multimodal vascular assessment for identifying patients at risk of progressive hand complications and support the hypothesis that vasculopathy-targeted therapies may help limit the progression of calcinosis and acro-osteolysis. Interventional studies are needed to confirm this hypothesis.",
  "authors": [
    {
      "affiliations": [
        "Department of Radiology, CHU Rennes, Univ Rennes, Rennes, France"
      ],
      "name": "Marine Tas"
    },
    {
      "affiliations": [
        "Department of Radiology, CHU Rennes, Univ Rennes, Rennes, France"
      ],
      "name": "Romain Lecigne"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, CHU Rennes, Univ Rennes, Rennes, France"
      ],
      "name": "Nicolas Belhomme"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, CHU Rennes, Univ Rennes, Rennes, France"
      ],
      "name": "Claire Cazalets"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, CHU Rennes, Univ Rennes, Rennes, France"
      ],
      "name": "Thibaut Louis"
    },
    {
      "affiliations": [
        "Department of Rheumatology CH Saint Brieuc, Saint Brieuc, France"
      ],
      "name": "Marine de Saint Riquier"
    },
    {
      "affiliations": [
        "Department of Rheumatology, CHU Rennes, Univ Rennes, Rennes, France"
      ],
      "name": "François Robin"
    },
    {
      "affiliations": [
        "Department of Rheumatology, CH Dinan, Dinan, France"
      ],
      "name": "Guillaume Coiffier"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, CHU Rennes, Univ Rennes, Rennes, France"
      ],
      "name": "Alain Lescoat"
    }
  ],
  "title": "OC.09 Risk factors of progression of calcinosis and acro-osteolysis in the hands of patients with systemic sclerosis: a longitudinal observational study",
  "uid": "55e5c90f-60ee-52c8-b310-518e6ec77e7f"
}